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Brit. J. Ophthal. (I 976) 6o, I 63
Scleritis and episcleritis

PETER G. WATSON* AND SOHAN SINGH HAYREHt
From Moorfields Eye Hospital, City Road, London*, and Addenbrooke's Hospital, Cambridge*,
and the Department of Ophthalmology, University of Iowa Hospitals and Clinics, Iowa City, USAt
From the earliest available descriptions of episcleritis Leigh, I965), subconjunctival phlegma or simple
(Slade, 1838; Rognetta, i844; Desmarres, 1847) or phlegmatous conjunctivitis by Mackenzie (I830),
a confusion has existed between scleritis and and episcleritis periodica fugax by Fuchs (I895).
episcleritis. This has led to contradictory views Duke-Elder and Leigh (I965) distinguished be-
on the relative severity and prognosis of these tween nodular episcleritis and rheumatic episcleritis,
conditions, and the erroneous belief that episcleritis but such a differentiation appears to be unjustified
is accompanied by serious complications (Viswaling- as rheumatoid arthritis can be associated with
ham, 1936; Wood, 1936; Mann and Markson, I950; either condition. We have classified episcleritis
Clavel and Teulieres, 1958). While Slade (I838), into two types:
Rognetta (i844), and Desmarres (I847), thought i. Simple episcleritis
that episcleritis was both common and severe, 2. Nodular episcleritis.
Mackenzie (i 830) considered it to be neither and gave
at the same time a vivid and accurate description SCLERITIS
of scleritis. This confusion still persists today.
In an attempt to clarify the position we have Many classifications of scleral disease have been
been studying patients with scleritis and episcleritis proposed (Holthouse, I893; van der Hoeve,
in a special clinic at Moorfields Eye Hospital 1934; Franceschetti and Bischler, I950; Duke-
during the past io years. Of these, 207 patients Elder and Leigh, I965; Sevel, I967) which have
(30I eyes) with episcleritis and 159 patients (2I7 been based on a mixture of clinical and pathological
eyes) with scleritis were analysed in depth.§ The observations. Although we tried to adopt these,
results reported below have led us to conclude from the clinical point of view they have all been
that scleritis and episcleritis are clinically distinct, unsatisfactory. We eventually adopted the following
with a different symptomatology and prognosis classification because, although there is some slight
and requiring different management. overlap between the groups, clinically we have
The incidence of these conditions is not known, observed several distinct patterns. This classifica-
but Williamson (1974) suggested that it was about tion has proved to be satisfactory and has enabled
o-o8 per cent of hospital new patient referrals. Our us to assign almost all the patients to a particular
series is from highly selected patients, all being category at the initial examination.
referred from elsewhere, often where there was (a) Anterior scleritis
difficulty in diagnosis, and with unusual problems. i. Diffuse scleritis
This is reflected in the fact that go per cent of the 2. Nodular scleritis
patients who had rheumatoid arthritis were seen 3. Necrotizing scleritis
within a year of the start of the special clinic. a. with inflammation
b. without inflammation (scleromalacia per-
forans)
Classification (b) Posterior scleritis.
EPISCLERITIS
Episcleritis has been recognized for over a century, Material
although-,called by many different names, such as Using the above classification, patients were
subconj4ctivitis by von Graefe (Duke-Elder and distributed as in Table I.
§Mr B. Augier and Mrs B. Phillips of the Institute of Ophthal-
mology. London, used the University of London computer to
analyse the findings Normal anatomy
Address for reprints: 22 Parkside, Cambridge, England*, or
Before considering the criteria by which the
University of Iowa, Iowa City, Iowa 52242, USAt differential diagnosis is reached it is necessary to.
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164 British Journal of Ophthalmology
understand the normal vascular anatomy of the 3. Scleral (deep episcleral) plexus (Fig. I)
outer coats of the eye. The blood vessels of the This plexus consists of a rete (criss-cross) of
episclera are not easily seen in the uninflamed eye, vessels lying within the visceral layer of Tenon's
but as soon as the eye becomes congested three capsule, closely applied to the sclera. At the limbus
quite separate vascular plexuses become readily the superficial and deep episcleral plexuses merge
visible. into one another and terminate in the superficial
marginal plexuses of the cornea. When congested
i. Bulbar conjunctival plexus (Fig. i) this layer looks bluish-red in colour and is immobile.
This is the most superficial plexus of fine hair-like
interlacing vessels freely moveable over the under- Clinical examination
lying structures. Overlying the episclera the Careful examination will determine the depth of
conjunctival arteries are derived from two sources the inflammation, so that it is almost always possible
the anterior ciliary arteries at the limbus, and the to decide when the patient is first seen whether
palpebral branches of the ophthalmic and lacrimal scleritis or episcleritis is present. This depends on
arteries. When they are inflamed the colour is accurate observation of the relationship of one
bright red. layer of vessels to the other and to the underlying
sclera.
2. Episcleral plexus (Fig. i)
HISTORY
The vessels, which are straight and radially A detailed clinical history is taken of the patient's
arranged, lie in the superficial episclera (parietal ocular and systemic disorders, including family
layer of Tenon's capsule) at a depth of about one- history. Any contact with chemical irritants and
quarter to one-third of the distance between the solvents is noted, as is any history of allergic
surface of the conjunctiva and sclera (Graves, conditions, rheumatic, connective-tissue or skin
1937). The visible vessels are mainly veins, accept- disease, gout, venereal disease, tuberculosis, or
ing the aqueous veins at intervals around the globe. sarcoidosis. The site, distribution, and nature of
These vessels are moveable over the deep layers, any ocular pain and disturbances of vision are
although not so easily as the conjunctival vessels. important in deciding the type of inflammation
In the anterior episcleral plexus (anterior to the
equator and over the muscle) the vessels belong to present.
the anterior ciliary system; while in the posterior EXAMINATION OF THE EYE
episcleral arterial plexus (posterior to the equator)
they are derived from the arteries of the oblique After the visual acuity has been recorded, the
muscles, the posterior ciliary arteries, and the vessels following routine of examination is used:
of the optic nerve sheaths (Hayreh and Baines,
1972). When inflamed, these radially arranged vessels I. External examination of the eye in daylight
can easily be seen, giving the eye a salmon pink It is essential to examine a case of episcleritis in
colour. daylight as this is often the only way of distin-
FIG. i Normal vascular networks of

episclera. There are three layers-
the-deepest is almost invisible in the
uninflamed eye
Scleritis and episcleritis I65
guishing episcleritis from the much more serious dary retinal detachment, and optic neuritis. Prop-
scleral disease. In episcleritis the colour is salmon tosis and limitation of movement of the extraocular
pink; in deep scleral disease the colour has a muscles are also looked for as evidence of an
much deeper purple hue. These colour changes involvement of posterior sclera.
are difficult to distinguish in tungsten or fluorescent
light. After recurrent attacks of scleral inflammation SYSTEMIC EXAMINATION
the sclera may become more transparent and is
sometimes thinner, and as a consequence the grey As scleritis is frequently a manifestation, and
choroid may be seen through the sclera. Some- sometimes the first manifestation, of systemic
times these areas are black and well defined, but disease, a thorough physical examination is impera-
more often they give a faint grey tinge to the sclera. tive, special attention being paid to the joints and
This is a particularly important observation in the cardiovascular system to eliminate a vasculitis.*
scleromalacia perforans, in which there is no sur-
rounding inflammation. Time and again it is Special investigations
possible to see areas of scleral thinning in daylight ROUTINE STUDIES CARRIED OUT IN ALL PATIENTS
which were invisible with the slit-lamp or by
illumination with tungsten or fluorescent light. i. Full blood count
This included haemoglobin estimation, white blood
2. Slit-lamp microscopic examination cell count and differential white cell count, and
erythrocyte sedimentation rate.
Of the greatest assistance in distinguishing epi-
scleritis from scleritis is the relationship of one 2. Rheumatoid arthritis latex agglutination test
vascular network with another and the changes
within them. The object of this examination is to The Rose-Waaler sheep-cell agglutination test was
confirm the macroscopic findings. In scleritis, the used as a confirmatory test for rheumatoid factor.
examination is directed to detecting scleral oedema
and the involvement of scleral vessels, ignoring 3. Serum uric acid estimation
the fact that the overlying episclera will be con-
gested and oedematous. In episcleritis there will 4. Serological tests for syphilis
be no oedema of the sclera. In addition avascular
patches must be diligently searched for, as these A patient who had a positive Wassermann reaction
indicate a vasculitis and carry a poor prognosis. also had the fluorescent treponemal antibody
absorption (FTA (ABS)) test, Reiter's comple-
Diffuse illumination confirms the macroscopic ment-fixation test, the Venereal Disease Refer-
impression that only episcleral tissue is involved, ence Laboratory (VDRL) test, and the Treponema
and in addition reveals any corneal changes. pallidum immobilization (TPI) test to confirm the
diagnosis of syphilis. It was recognized that some
Slit-lamp examination detects the depth of maxi- cases of syphilis might have been missed as only the
mum vascular involvement, infiltration, and oedema Wassermann and Kahn tests were performed as a
of the episclera. Any corneal changes can be routine. In the case of any abnormal result the
categorized and the anterior segment and vitreous test was repeated.
scrutinized for evidence of uveitis.
Red-free light examination should never be omitted; 5. X-rays of the chest and sacro-iliac joints
it is the ideal method of examining the vascular These were taken whether the patient had any
changes because the vessels become more easily relevant complaints or not.
visible. Infiltration of the episclera also shows
up as a yellow patch. Adrenaline i: IOOO or io per
cent phenylephrine can be instilled into the con- ADDITIONAL STUDIES CARRIED OUT IN CERTAIN
junctival sac. This constricts the conjunctival and PATIENTS
superficial episcleral vessels more than the deep i. A full immunological survey for tissue anti-
scleral plexus, thus revealing any underlying bodies, soluble immune complexes, complement,
scleral disease. lupus erythematosus cells, and antinuclear factor
(ANF) were undertaken.
3. General eye examination 2. Mantoux test.
3. Electroretinograms and electro-oculograms were
It is essential to exclude complications such as *r his was carried out in our clinic by the rheumatologists Dr
glaucoma, uveitis, pars planitis, choroiditis, secon- Fawler, Dr Hazleman, and Dr Lachmann
I66 British Journal of Ophthalmology
performed initially in all patients but later in the Within one year of the onset of this condition 92
series these were confined to patients with severe per cent had sought advice.
disease and to all those with posterior scleritis.
AGE AND SEX DISTRIBUTION
Results Scleritis was more than I times as common in
PATIENTS women as in men. This contrasted with episcleritis,
which was equally common in both sexes.
The data were derived from 207 patients (30I eyes)
with scleritis and 159 patients (2I7 eyes) with
episcleritis (Table I). i. Scieritis
Analysis of the age and sex distribution for the
FAMILY HISTORY
different types of scleritis revealed distinct pat-
terns for each type of scleritis. The diffuse and
A family history was elicited in 14 patients (four nodular varieties were most prevalent. In men
of these patients had simple episcleritis, three the peak incidence occurred in the fourth decade
nodular episcleritis, six nodular scleritis, and (P= < o05); women showed two separate peak
one diffuse anterior scleritis). In the case of the incidences, one in the third decade and another
three patients with nodular scleritis the condition in the sixth decade (P= < o0o5).
was very similar to that seen in close relatives, but
in all others the history was indefinite. This Scleritis-diffuse anterior scleritis
indicates that neither episcleritis nor scleritis is a
familial condition. In women the disease was commonest in the
fourth to seventh decades, with no predilection
for any age group. In men it was more prevalent
TIME BETWEEN ONSET AND FIRST ATTENDANCE in the third to sixth decades, with a distinct peak
In contrast to episcleritis, patients with scleritis during the fourth. The condition was rare in the
attended much sooner after the onset of the disease, young and the old.
but even so (presumably because the onset was
often insidious) many patients did not seek advice Scleritis-nodular anterior scleritis
until the pain had become severe, which might The nodular disease in both sexes was most pre-
not have been for several weeks. Of the patients valent from the fourth to sixth decades, but in
with episcleritis I3 per cent reported in the first women there was a very marked peak during the
month and 64 per cent within two months. Of sixth decade.
those with scleritis the results were very similar,
37 per cent being seen within the first month and
68 per cent before the end of the second month. Scleritis-necrotizing type with inflammation
Of the patients who presented with this severe
variety of scleritis the majority were women, but
Table I Patients with episcleritis and scleritis whose of those who presented during the fourth decade
records were analysed by computer all but one were men.
Further
Diagnosis Patients Eyes subdivisions Eyes Scleritis-necrotizing type without inflammation
(scleromalacia perforans)
Episcleritis I 59 217 Simple 170 All these patients were women. The age range
Nodular 47 was 35 to 75 years, most cases occurring in the
Scleritis 207 30I Diffuse anterior II9 fifth decade.
Nodular anterior I34
*Necrotizing
with Scleritis-posterior scleritis
inflammation 29 Posterior scleritis was usually associated with a
without 42 diffuse anterior scleritis, occurring most frequently
inflammation I3 J in women in their sixth decade, but one woman of
Posterior 6
35 years was seen with this condition.
*The incidence of necrotizing anterior scleritis is open to question
because, although sometimes obvious when the patient first presents,
the disease more often starts as a diffuse or nodular anterior scleritis. 2. Episcleritis
Active treatment has certainly prevented some patients from de-
veloping necrotic lesions. Only those patients whose diagnosis was Altogether 88 men and 71 women suffered from
absolutely certain have been included in this category episcleritis. There was a significant variation in the
Scleritis and episcleritis i67
sex distribution at different ages. The condition symptoms; in nodular episcleritis, scleritis, and
was not seen in childhood. Episcleritis in men diffuse anterior scleritis the onset was much more
occurred maximally from the third to the sixth gradual, the intensity of the inflammation increasing
decade, with a peak in the fourth decade. In over a period of several days. The redness was
women, however, although there was a peak sectorial, ranging in intensity from the barely
incidence in the fourth and fifth decades, the distri- noticeable to the fiery red; the degree of inflam-
bution did not show a predilection for any parti- mation bearing no relationship with the seriousness
cular age group. The difference between the of the condition. Indeed, in scleromalacia perforans
peak incidence in the different series was highly (necrotizing scleritis without surrounding inflam-
significant (P= < o005). There was no significant mation) there might be no redness of the eye at all.
difference in the sexes between the time of onset of
simple and nodular episcleritis, but nodular epi- LACRIMATION, PHOTOPHOBIA, AND CONJUNCTIVAL
scleritis had a peak incidence in the fifth decade DISCHARGE
whereas simple episcleritis was most common in
the fourth decade (P= <o0o5). Conjunctival discharge was not a feature of scleritis
or episcleritis. Indeed, if the discharge is anything
INVOLVEMENT OF ONE OR BOTH EYES
but watery in consistency an incorrect diagnosis has
probably been made. Photophobia and lacrimation
I. Scleritis did not universally accompany episcleritis or
In 45 per cent of the patients the disease was scleritis, but where these symptoms were severe
bilateral, but in cases in which the disease involved they often indicated the presence of a necrotizing
only one eye, one side was not involved more than process.
the other. When each type of scleritis was further In scleritis these complaints were not universal;
analysed it showed that diffuse anterior scleritis of the patients with diffuse anterior and nodular
was more often bilateral (P = < o oo5) and that scleritis 27 per cent complained of some degree of
nodular anterior scleritis was more often unilateral watering. However, 70 per cent of the patients
(P= < 0o005). In necrotizing scleritis with inflam- with necrotizing scleritis complained of photo-
mation both eyes were more often affected than in phobia and in 50 per cent the eye watered. Photo-
patients with scleromalacia perforans. These dif- phobia was unusual in posterior scleritis but
ferences were not, however, significant. about half the patients complained of some lacri-
Posterior scleritis was usually unilateral. mation. No correlation with the presence or type
In 35 per cent of those patients in whom the of keratitis could be demonstrated. The incidence
condition became bilateral, the other eye was of keratitis in each type of scleritis was much less
affected between one and three months after the than suggested by the symptoms of photophobia or
onset of the condition in the first eye. In the rest lacrimation. In episcleritis when these symptoms
the disease did not occur in the other eye in less occurred they were rarely, if ever, severe. Photo-
than six years. phobia occurred in io per cent of the patients and
lacrimation was complained of in 25 per cent.
Patients with nodular disease did not have more
2. Episcleritis severe symptoms than those with simple episcleritis.
Of patients with episcleritis, 36-5 per cent developed Photophobia and lacrimation showed some cor-
the condition in both eyes. When only one eye relation with corneal changes, but these symptoms
was affected there was no significant difference in were not always due to changes within the cornea.
the distribution between right and left eyes.
Nodular disease occurred more commonly uni- PAIN
laterally than bilaterally. The fellow eye was affected
either between one and three months or after six Scleritis
years. There were no exceptions to this in this This is one of the very few severely painful eye
series. conditions and severe pain was complained of in
6o per cent of these patients (Table II). The pain,
Symptoms which was boring in character, was either localized
to the eye or generalized, in which case it radiated
REDNESS
from the orbital margin to the temple or the jaw
Almost all patients naturally presented with in the distribution of the trigeminal nerve. Some
redness of the eyes. In simple episcleritis the patients had been erroneously diagnosed as having
onset was often extremely rapid, the eye becoming migraine or sinusitis, and one patient was thought
flushed within a few minutes of the onset of to have a cerebral tumour. Many other patients
Table II Types of pain experienced in different types of scleritis (percentage)

Localized Generalized Both localized Total no.
Type of scleritis only only and generalized of eyes
Diffuse anterior 14 I5 30 II9
Nodular anterior 25 I6 20 134
Necrotizing anterior
With inflammation 0 I5 46 29
Without inflarnmation
(scleromalacia perforans) 0 13 I9 I3
Posterior 0 5 33 6
suffered mild discomfort, but as this did not the various types from their typical clinical appear-
constitute one of their complaints these patients ances although, as already stated, while necrotizing
are not included in the table. Pain is rarely com- scleral disease sometimes started as a nodular
plained of in scleromalacia perforans, in which scleritis, its presence could usually be suspected
there is no surrounding inflammation; this is in because of the severity of the symptoms.
marked contrast to the pain of necrotizing scleritis, The essential diagnostic feature in scleritis was
which is the severest of all, often preventing sleep the involvement of scleral tissue in the inflam-
and causing severe loss of weight, and described by matory process. This usually took the form of
Mackenzie (I830) more than I40 years ago scleral oedema which could be detected by observ-
['the pain . . . is pulsative and deep seated, the chief ing the outward displacement of the deep vascular
pain, however, is not so much in the eyeball, as round network of the episclera (Fig. 2).
the orbit, under the eyebrow, and in the temple, cheek Because the episclera was always swollen and
and side of the nose, and is severely aggravated from congested in scleritis, scleral oedema was the sign
sunset to sunrise']. which had to be looked for. The deep episcleral
The pain could indeed be so severe that the eye vascular plexus was more congested than the
had to be removed. superficial network, giving rise to the typical
bluish-red colour. The conjunctival vessels were
Episcleritis rarely involved.
Severe pain is not a feature of episcleral involve-
ment. Discomfort, rarely described as pain, oc- SCLERITIS-NODULAR ANTERIOR SCLERITIS (Fig. 3)
curred in 51 per cent of patients and was almost Although these patients resembled those with
equally common in simple and nodular types. The nodular episcleritis on cursory examination, de-
sensation in the eye was described as hot, sharp, tailed examination revealed marked differences
a slight ache, or irritable. Equally the pain around and rarely presented any difficulty in diagnosis.
the eye was described as an ache and, although it The nodule was deep red in colour, totally immo-
sometimes radiated to the temple or jaw, it was bile and quite separate from the overlying con-
never described as boring. However, very occa- gested episcleral tissues. All the vessels were lifted
sionally a patient will present with severe swelling by the nodule. There was, of course, some secon-
of the lids, extreme discomfort, and even miosis dary overlying episcleritis and conjunctival con-
and temporary myopia. gestion which involved the whole anterior episclera
The pain or discomfort was localized to the eye in 20 per cent (Fig. 3). Multiple nodules were seen
in 25 per cent of those patients with simple epi- in 42 per cent, sometimes reaching an enormous
scleritis and 33 per cent of those with nodular size; in 50 per cent they were tender.
episcleritis, and was felt around the eye but not
in the eye itself in only 9 per cent of those with SCLERITIS-DIFFUSE ANTERIOR SCLERITIS (Fig. 4)
simple episcleritis and 14 per cent with nodular
episcleritis. Both localized and generalized pain This was the most benign form of scleritis and it
was experienced in i9 per cent in simple episcleritis was associated with the least severe of the associated
and in io per cent in the nodular variety. general conditions. The patients might present
with an eye in which the sclera was obscured by
a diffuse redness and swollen episclera, which
Physical signs might be accompanied in severe cases by conjunc-
SCLERITIS tival oedema so severe as to obscure the cornea
In the classification of scleritis which we have and protrude from between the eyelids. In these
adopted it has been possible to distinguish each of patients adrenaline i :iooo had to be applied to
Scieritis and episcleritis 169
FIG. zSlit-lamp appearances in scleritis.

Sclera is swollen so that deep plexus of vessels is
displaced forwards. Accompanying episcleritis
causes slight separation of superficial networks
~. complications other than scieral thinning and 40

per cent suffered a deterioration of visual acuity.
P ~~~~~~~~~Those
patients who were firstseen at the onset of
the disease presented with a localized patch of
scleritis which was associated with acute congestion,
sometimes so severe as to merit the term 'brawny'
scleritis, but there was nothing in the physical
-~ appearances, the aetiology or prognosis, to justify
including them as a separate category.
Another physical sign which must alert one to
the presence of severe necrotizing scleral disease
is the avascularity of a patch of episcleral tissue
overlying or adjacent to an area of scleral oedema
FIG. 3 Nodular anterior scleritis. A discrete scleral (Fig. 6). This sign was found in 27-5 per cent of
nodule, displacement of deep vessels over nodule and
surrounding inflammation overlying unaffected sclera
are characteristic of this condition
the conjunctival sac so that the deep scleral 4

network and the scleral tissue itself could be
visualized, and any degree of scleral oedema deter-
mined. In the less severe cases the area of redness
might be confined to a small area of the globe
with very little overlying congestion. The inflam-
mation was more widespread than in the nodular
disease, and the extent of the involvement varied
from a small area (61 per cent) to the whole anterior
segment (39 per cent). The vascular pattern was I
occasionally grossly distorted and the normal radial

pattern lost, with replacement of these vessels
with abnormal tortuous new channels (Fig. 5).
The eye was tender in 54 per cent of patients.
SCLERITIS-NECROTIZING SCLERITIS WITH SIGNS OF
ADJACENT INFLAMMATION (Fig. 6) ~FIG. 4 Diffuse anterior scleritis. Underlying scleral
This was found to be the most destructive formn of oedema could be detected only by first blanching
the disease. Of these patients 6o per cent developed swollen congested episclera with i :1000o adrenaline
FIG Abnormal, tortuous vascular

N'
i
i U. ~~~~~~~~~~~~~~channels
which develop after recurrent
attacks of diffuse anterior scieritis
had become involved (Fig.
mation subsided, or was suppressed by treatment,

transparent sclera remained. Complications did not
-
- ~~~~~~~~supervene until the inflammatory process had pro-
gressed through almOst 360 degrees. Rarely the
whole anterior segment became inflamed at one and
the same time, and in some of these eyes areas of
necrosis occurred which, if they had not been
l illtreatedurgently, would have become sequestrated,
exposing the underlying uvea. It was not, however,
our experience that staphyloma (bulging of the
FIG. 6 Necrotizingr anterior scleritis. Superficially this-

patient appears to be similar to those shown in Figs
4 and 5, but there is an avascular area adjacent to
the limbus. If this is not treated, ulceration rapidly
occurs similar to that in Fig. 7
patients with necrotizing scleritis with inflammation v

and in 23 per cent of those without inflammation
(scleromalacia perforans).
Several types of response were noted. In some K
eyes the inflammation remained localized to one
small area, arid, if not checked by treatment, FIG. 7 Necrotizing anterior scleritis. Late stage of
resulted in almost total loss
ls of scleral tissue from necrotizing scleritis. Inflammation is progressing around
resultedrei
that area. More toequental onfsleram tionssu tedf
aMostfrequently the inflammation started
the globe, leaving an area of avascular necrotic sclera
behind. Almost always these areas are crossed, as here,
in one area and spread in both directions around by a large vein which drains the limbal area on a thin
the globe until the whole of the anterior segment bridge of normal sclera
Scleritis and episcleritis I7I
uvea into the scleral defect) occurred unless the circular crack, and the area inside this would take
intraocular pressure became raised above 30 mmHg on a yellowish or greyish appearance and after a
and had remained high for a prolonged period. variable period would separate as a sequestrum
Avascularity of a patch of overlying sclera oc- together with the overlying episciera. Large
curred in 27-5 per cent of the patients. These patches abnormal vessels surrounded and crossed the area,
often occurred very early in the disease, and the and where they crossed it near the limbus they
condition must be treated vigorously if further left an area of about o 5 mm on either side of a
necrosis is to be prevented. The eye was tender in relatively normal sclera. The resulting defect was
40 per cent of these patients. covered by a very thin layer of connective tissue
which appeared to be derived from the conjunctiva.
SCLERITIS-NECROTIZING SCLERITIS WITHOUT
Unless the intraocular pressure rose, no staphy-
ADJACENT INFLAMMATION (SCLEROMALACIA
loma was seen. In no case was the eve tender to
the touch.
PERFORANS) (Fig. 8)
This condition was associated with long-standing SCLERITIS-POSTERIOR SCLERITIS
rheumatoid arthritis in 46 per cent of patients.
They presented without any subjective symptoms, We were certain of the diagnosis in six cases
the doctor or the patient having noticed either a (2z9 per cent), but it was suspected in several
greyish or yellowish patch on the sclera or, in cases, and it was likely that others escaped our
more severe cases, areas of complete loss of tissue. notice. Since there are no apparent external signs
Any inflammatory change was minimal. There of posterior scleritis unless there is also an asso-
was no reactive oedema because it appeared that ciated anterior scleritis, the presence of proptosis,
the episcleral tissue overlying the defect had disap- exudative detachment and other fundus changes,
peared, and elsewhere it was so thin as to be almost including papilloedema, may mislead the ophthal-
invisible. The number of vessels in the remaining mologist to such an extent that the correct diag-
episcleral tissue also appeared to be much dimin- nosis is never made. In view of this it is possible
ished, giving a porcelain-like appearance to the that the incidence of posterior scleritis may not be
white of the eye when viewed from a distance. as low as indicated by the present series, as our
Altogether 40 per cent of the patients had more patients were all referred from elsewhere. The con-
than one necrotic patch. We had the opportunity dition was unilateral in four of the six cases.
of watching some of these develop and the pattern
seemed to be the same. The conjunctiva and PRESENTING FEATURES IN POSTERIOR SCLERITIS
remaining episclera might become suffused over a
wide area or might remain unaltered. A demar- Symptoms. In all the patients there was extreme
cation line would develop in the sclera, like a pain and tenderness.
FIG. 8 Necrotizing scleritis without

inflammation (scleromalacia
perforans). Almost complete
dissolution of scleral collagen occurring
with little if any surrounding
inflammation in woman of 6o years
with long-standing rheumatoid
arthritis
FIG. 9 Appearance of fundus after

resolution of extensive serous
detachment in patient with posterior
scleritis who presented with
diminution of vision, anterior
scleritis, slight proptosis, and a
diffuse leak early in dye transit on
fluorescein angiography
Visual acuity. All eyes except one showed some In the early stages there was a diffuse leak early
reduction in visual acuity. in the dye transit on fluorescein angiography.
Fundus changes. In four of these patients there Proptosis. Two patients presented with proptosis,
was an exudative retinal detachment associated a diffuse anterior scleritis, limitation of ocular
with swelling of the optic disc and an anterior movements, especially upwards, and retraction of
diffuse scleritis. The anterior scleral involvement the lower lid (Fig. io) when attempting to look
was minimal and had to be looked for carefully, upwards. This physical sign seemed to be specific
but the retinal changes were typical. Areas of for this condition and was presumably caused by
exudation occurred beneath the retina, giving the infiltration of both the tendons and the muscle
appearance of pale greyish-white spots with a cone in the region of the posterior scleritis. Diplopia
surrounding dark greyish line that could be seen was also a presenting feature of posterior scleritis
through the overlying serous detachment (Fig. 9). because of involvement of the muscles and tissues
FIG. IO Posterior scleritis. Retraction

of left lower lid in patient with
posterior scleritis who also had severe
_ limitation of ocular movements. Ocular
' movements became normal with
- treatment but the lid sign remained
Scleritis and episcleritis 173
within the muscle cone by the inflammatory

process. All these features returned to normal
after treatment. The exudative detachment in one
patient disappeared within five hours of starting
treatment. The site of inflammation remained pale
and white after the inflammation had disappeared,
leaving some areas of pigment migration at the
edges, giving the appearance of a high-water mark.
EPISCLERITIS
Simple and nodular episcleritis differed in their
clinical signs, but in both, the oedema or infiltration
were entirely within the episcleral tissue; the sclera
itself was not involved. The episcleral vascular
network was maximally congested, with some FIG. 1 2 Nodular episcleritis. Swelling and oedema
congestion of the conjunctival vessels and minimal confined to episcleral tissue and vessels retain their
normal architecture
congestion of the scleral vessels (Fig. iI).
EPISCLERITIS-NODULAR EPISCLERITIS
EPISCLERITIS-SIMPLE EPISCLERITIS In contrast with simple episcleritis the oedema and
The redness of the episclera was often very intense infiltration of nodular episcleritis were localized to
and was localized to one sector in 69 per cent of one part of the globe, except in seven eyes in
patients and generalized in 3I per cent. The epi- which the redness was widespread. A nodule from
scleral vessels, although engorged, retained their within the episcleral tissue, which was surrounded
normal radial position and architecture. The by some congestion, was mobile on the underlying
colour varied from fiery red to a mild red flush sclera and there was no involvement of the sclera
but did not have the bluish hue so typically seen in in the oedema. The scleral plexus of vessels could
scleritis. be distinguished deep to the nodule lying flat on the
In simple episcleritis there was a diffuse oedema sclera, which retained its normal contour (Figs iI
of the episcleral tissue which was sometimes and I2). The nodules were usually single but were
infiltrated by greyish deposits which appeared sometimes multiple, reaching the size of a large pea.
yellowish in red-free light. The depth of the The eye was tender to the touch in 40 per cent.
oedema and its effect on the vascular plexus was
best recognized by slit-lamp examination, using a Vascular changes in the episclera
narrow beam. The eye was tender to the touch in Although vascular changes were most unusual in
33 per cent of cases. episcleritis, vessels that appeared to be abnormal
FIG. i iEpiscleritis. Although deep vascular

network is congested there is no scleral swelling,
oedema and infiltration being confined to episclera
either in course or type were noted in i 9 eyes Table IV Fall of visual acuity in scleritis due
with simple episcleritis and iO eyes with nodular to keratitis
episcieritis. All these patients had had recurrent
attacks at the same site for many years. One patient No. of eyes with fall in visual acuity
showed avascularity of the episcleral nodule which Keratitis during follow-up
was thought to be due to the intensity of the oedema, Superficial 2
as the vessels returned to normal as soon as treat- Midstromal 3
ment was given. Deep o
Guttering 3
Complications Others 5
Complications of some sort were found in only

I5 per cent of patients with episcleritis but ocular Keratitis
complications, other than simple increased scleral SCLERITIS
transparency or thinning, occurred in 57 per cent
of all patients with scleritis. The complications Corneal changes of a type characteristically seen
looked for and analysed were: in scleritis were observed in 29 per cent of patients.
i. Fall in visual acuity This keratitis can be serious, leading to permanent
2. Keratitis changes in the cornea; these are so characteristic
3. Cataract that when certain types of sclerosing keratitis and
4. Uveitis lipid changes are seen in an eye which is otherwise
5. Glaucoma normal, it can be inferred that the patient has had
6. Scleral thinning (and scleral defects) scleritis at some time in the past.
Characteristic corneal changes were seen in
each type of anterior scleritis and could be classified
Fall in visual acuity as follows:
SCLERITIS
A total of 42 eyes (14 per cent) in patients with Nodular scleritis
scleritis lost a significant amount of vision after i. Localized stromal keratitis
the disease had been present for one year. This fall ii. Localized sclerosing keratitis
in visual acuity was attributed to cataract (five eyes),
anterior uveitis (two eyes), and posterior uveitis
(two eyes). The commonest reason for the fall in Diffuse scleritis
visual acuity was the association with keratitis i. Acute stromal keratitis
(Table IV), particularly if this was accompanied ii. Sclerosing keratitis
by a uveitis. iii. Corneal guttering
EPISCLERITIS Necrotizing scleritis

No patient with episcleritis had a fall in visual i. Sclerosing keratitis
acuity during the period of follow-up. ii. Keratolysis
The incidence of keratitis in different types of
Table III Fall of visual acuity in scleritis scleritis is given in Table V.
Fall in visual acuity
Total 2 lines or more during Acute stromal keratitis (Fig. I3)
Type of scleritis no. of period offollow-up
eyes (per cent) In the severe necrotizing scleritis of acute onset
the cornea became oedematous and dense white
Diffuse anterior II9 15 infiltrates appeared within the stroma (Fig. I3).
Nodular anterior I34 7 These were sometimes central and sometimes
Necrotizing anterior peripheral and might coalesce as the disease pro-
With inflammation 29 27 gressed. If, in addition, there was anterior chamber
Without inflammation activity, keratic precipitates adhered to the pos-
(scleromalacia terior corneal surface. The opacity was sometimes
perforans) 1I3 31 surrounded by a 'precipitin' ring. If the scleritis
Posterior 6 50
was treated vigorously with full doses of steroids
Scleritis and episcleritis 175
Table V Keratitis and vascularization of the cornea in scleritis

Keratitis Corneal vascularization
Total
Type of scleritis no. of Super- Mid- Gutter- Super- Mid-
eyes ficial stromal Deep ing Other ficial stromal Deep Other
Diffuse anterior iI9 8 I0 2 4 4 3 2 3 2
Nodular anterior 134 6 I4 4 5 6 5 0 I 0
Necrotizing anterior
With inflammation 29 4 4 2 4 3 2 I 3 0
Without inflammation
(scleromalacia perforans) I3 0 3 I 3 0 I 3 2 I
Posterior 6 0 0 I 0 0 0 0 0 0
Total 30I i8 3I Io i6 13 II 6 9 3
these opacities could disappear without trace. More of the keratitis which might be complete or, more
usually, however, linear opacities within the usually, the lamellae of the cornea were altered to
stroma remained and, if central, these impaired leave thin linear opacities which looked exactly
vision. like the sugar crystals of 'candy floss' (Fig. I4).
Crystalline (candy-floss) opacities were seen in
Sclerosing keratitis (Fig. 14) 23 patients with diffuse and another 23 patients
with nodular scleritis, and five patients with necro-
The corneal opacities associated with scleral disease tizing scleral disease. This type of corneal change
usually appeared less dramatically; the cornea could not be correlated with vascularization or any
adjacent to the swollen sclera became oedematous particular aetiological agent. Keratolysis was ob-
and vessels grew into this oedematous area. Vascu- served in four patients, all of whom had severe
larization never seemed to precede the oedema. necrotizing anterior scleritis. These opacities, once
Although these opacities usually occurred close to formed, never disappear. In very severe cases the
the limbus they could appear in the superficial and opacification of the cornea progressed circum-
midstroma centrally in the cornea, having no ferentially until only a central clear area in the
obvious connexion with the site of the scleral cornea remained, and even this area might disap-
disease. pear, resulting in a 'scleralized cornea'. Corneat
Whatever the type or site of the corneal lesion, grafting was very successful in restoring the visual
treatment of the scleritis brought about resolution acuity in these patients.
'FIG. 13 Acute stromal keratitis in

scleritis. Dene white infiltration in
cornea in same patient as in Fig. 9.
Immune rings similar to those seen in
Fig. 14 appeared round these lesions.
AAll corneal signs, etc. disappeared
after treatment
FIG. 14 Sclerosing keratitis in

scleritis. A 66-year-old woman with a
I3-year history of recurrent bilateral
diffuse sclero-keratitis with crystalline
(candy-floss)deposits in the deep
stroma and a 'precipitin ring' around
the infiltration at 12.0 o'clock. The
vascularization is passive. Vessels
enter the cornea only at site of
advancing edge of corneal change and
at late stage in disease. Acute
stromal keratitis will progress to
this if left untreated
Limbal guttering these metabolites to the limbus and it was not a

Guttering of the peripheral cornea was not infre- product of the corneal inflammation.
quently seen, particularly in patients who had
severe long-standing rheumatoid arthritis. These Posterior corneal changes
gutters never extend more than 2 mm from the
Although we have not analysed this series for it, we
limbus, but may become circumferential and very have recently come to recognize another pattern
thin, requiring grafting. Erosion of the deep stroma of corneal change in scleritis (Holt-Wilson and
resembling Terrien's disease may also occur, and Watson, I974), which presented as an interstitial
in one case this led to an expansion of the eye to i8 keratitis indistinguishable from that seen in con-
dioptres of astigmatism in that axis, necessitating genital syphilis but which occurred in patients
corneo-scleral grafting over the affected region who had a mild scleritis and in whom the serological
(Fig. I5). investigations were entirely negative (Fig. 17).
Keratitis could be considered the sole reason for a
Keratolysis severe fall in visual acuity in I 3 eyes (4 per cent).
The most severe corneal disease of all is keratolysis.
This was seen in only four patients, all of whom EPISCLERITIS
had very severe necrotizing scleritis, usually, but
not invariably, accompanied by loss of scleral Minimal corneal changes were seen in i5 per cent
tissue. In this terrifying situation the whole of the patients with simple episcleritis and in 15
stroma of the cornea disappeared with alarming per cent of those with nodular disease, but these
rapidity (Fig. i6). The progress could have been were never severe. The oedema of the cornea
stopped by full steroid therapy but only after very occurred when the episcleral inflammation was
prolonged treatment would any corneal tissue have close to the limbus and seemed to be a consequence
been re-formed. It was usual therefore to graft of this adjacent inflammation. Keratitis was most
these eyes when the scleritis had become well common in the superficial and midstromal layers
controlled or if rupture appeared imminent. of the cornea in both varieties. Some localized
Lipid deposition occurred in areas where the corneal guttering occurred in six eyes after recur-
cornea had been damaged and at the inner edge of rent attacks in the same area for many months.
a long-standing corneal gutter. This was presum- Vascularization of the cornea occurred in six eyes,
ably because of interference with the usual flow of the vessels being deep in three, midstromal in one,
..........
FIG. 15 Limbal guttering in

scleritis. A 24-year-old man with a
\10\Vio-yearhistory of unilateral scleritis

localized to area of sclera between
12.0 and 2.0 o'clock. Cornea in area
has become guttered and produced a
c..>f.'Terrien'-like change. This area
required grafting (Fig. I9) because

X.
of rapidly increasing expansion of
globe in this axis
worse during the course of the disease. All these

patients were between the ages of 55 and 8o years
and all had serious scleral disease. In only three
patients (five eyes) with an associated long-
standing anterior uveitis was it felt certain that the
sclero-uveitis was responsible for the cataracts.
In two of these patients it was particularly notice-
able that the cataract had developed rapidly once
the scleritis had involved the sclera through 360°.
Three of these cataracts have required extraction.
The sclero-corneal wound has healed normally
after cataract extraction in spite of continuing
active scleritis in the area of the wound. One
FIG. I6 Keratolysis. Severe necrotizing changes in eye developed a steroid-induced cataract after
both cornea and sclera which occurred in a period of
five weeks in a 74-year-old woman. Systemic steroids
suppressed inflammation and both cornea and sclera
were grafted successfullv Table VI Keratitis in episcleritis
Type of keratitis
and superficial in two. No serious complications Episcleritis
were found in any of the patients studied. Mid-
Superficial stromal Deep Guttering
Cataract Simple 7 14 3 5
SCLERITIS Nodular 2 4 I I
Cataracts were detected in only 22 eyes, 7 per cent. Comeal
These were bilateral and senile in type in 15 eyes. vascularization 2 I 3 o
In 12 eyes the cataract was noted to have become NB Some eyes showed more than one type of keratitis
FIG. 17 Deep stromal corneal

changes as seen in group of young
patients with diffuse anterior
scleritis. When vessels enter cornea
they do so adjacent to Descemet's
membrane, giving the appearance of
a syphilitic interstitial keratitis
prolonged local steroid treatment. Five eyes in an anterior uveitis did occur it tended to be long-
which cataract developed also showed a severe standing and intractable.
long-standing anterior uveitis. The distribution was evenly divided between
unilaterally and bilaterally affected eyes. Only two
EPISCLERITIS
patients developed a uveitis in the other, apparently
unaffected, eye.
Eight patients, six of whom were over 6o years old,
were found to have cataracts. However, in none of Posterior uveitis
these patients did the cataract progress during the
period of follow-up, nor were the cataracts worse in All the patients with posterior scleritis had posterior
the eyes with episcleritis. One patient had developed uveitis, but posterior uveitis was also found in those
a typical steroid cataract from prolonged local steroid who had an anterior scleritis (Table VIII).
treatment before we saw him. No correlation was In two of the patients with nodular anterior
found between episcleritis, uveitis, or cataract. scleritis, and in three of those with necrotizing
disease, discrete areas of subretinal exudation
Uveitis
Table VII Anterior uveitis in scleritis
SCLERITIS
Anterior uveitis Anterior uveitis

Type of scleritis Eyes (per cent)
There was evidence of anterior uveitis in 30 per cent
of all the eyes with scleritis. It was found univer- Anterior
sally in those patients with scleromalacia perforans Diffuse II9 35
(Table VII). However, it did not always occur in Nodular '34 17
those with necrotizing scleritis, nor was there any Necrotizing 29 37
particular correlation between the presence of an Scleromalacia perforans 13 100
Posterior 6 66
anterior uveitis with any particular disease. When
Table VIII Posterior uveitis in scleritis uveitis with secondary glaucoma, required other
medication for control of the intraocular pressure.
Posterior uveitis None of these patients has yet required surgery.
Type of scleritis Eyes (per cent) No patient with posterior scleritis had glaucoma.
One patient developed a steroid glaucoma which
Anterior has required continuous medication for its control.
Diffuse II9 13
Nodular I34 3
Necrotizing 29 17 EPISCLERITIS
Scleromalacia perforans 13 23 Glaucoma was detected in nine eyes (4 per cent).
Posterior 6 100 Two patients were known to have chronic open-
angle glaucoma before the onset of the episcleritis.
Five eyes had developed a steroid glaucoma from
could be seen at the periphery of the fundus with a prolonged administration of local steroids. In no
'high-water mark' of retinal and subretinal oedema instance could the glaucoma be attributed to the
around the patches of exudation. One patient episcleral inflammation, although there was corneal
developed an annular choroidal detachment at involvement in one eye.
the height of the scleritis.
Scleral thinning
EPISCLERITIS When the sclera looked blue in the daylight when
No severe uveitis occurred in this series. Mild contrasted with surrounding white sclera this was
uveitis, detectable on the slit-lamp by an increase regarded in our analysis as scleral thinning. The
in the number of cells in the anterior chamber and sclera in many of these patients had become
a mild aqueous flare, was found in I2 eyes. In
transparent rather than thin; however, the blue
two unilateral cases, flare and cells were noted in patch appeared only in cases in which there had
the unaffected eye. No correlation between comeal been active disease. The change in transparency
was usually not apparent during the active stage of
involvement and uveitis was detected.
the disease but it was observed at the site of recur-
rent simple or nodular episcleritis in I 9 eyes
Glaucoma (8 per cent), and in 36-5 per cent of eyes affected
with scleritis, during the period of follow-up.
SCLERITIS
After repeated attacks of episcleritis, particularly
Glaucoma was present in 35 eyes (iI 6 per cent), if these occurred at the same site, the sclera might
of which 20 per cent showed associated keratitis. become transparent but not thin. Similarly in
Of the patients with secondary glaucoma nine diffuse and nodular scleritis the sclera might
had no abnormality other than scleritis, seven also become transparent with the blue choroid showing
had keratitis, and six uveitis. through. This was easily seen in daylight and
Secondary glaucoma often accompanies severe could be used as an indication of previous scleral
anterior scleritis, the cause apparently being due inflammation. Ectasia and destruction of scleral
to obstruction of the outflow channels by oedema tissue occurred only in necrotizing scleritis. This
because the intraocular pressure falls and the out- could take the form of an avascular area appearing
flow of aqueous rises as soon as the scleritis comes in the centre of an area of scleritis, which then
under control with systemic treatment. Six patients, broke down to form a slough and eventually to
who had anterior synechiae and a long-standing leave an area of thinning.
Table IX Glaucoma in scleritis

Total with Primary Primary Steroid-
Type of scleritis Total glaucoma open-angle closed-angle Secondary induced
Diffuse anterior II9 12 6 0 6 0
Nodular anterior I34 II 6 0 5 0
Necrotizing anterior 29 8 2 0 5 I
Scleromalacia perforans 13 4 0 0 4 0
Posterior 6 0 0 0 0 0
30I 35 14 0 20 I
I8o British _ournal of Ophthalmology
Table X Scleral thinning or transparency in scleritis

Eyes with thinning
Type of inflammation Total no. Total incidence
of eyes Localized Generalized (per cent)
Episcleritis
Simple 170 I2 3 9
Nodular 47 4 0 9
Scleritis
Diffuse anterior II9 23 15 32
Nodular anterior 134 35 2 28
Necrotizing anterior 29 II I0 69
Scleromalacia perforans 13 7 6 I00
Posterior 6 I 0 I6
In the type of necrotizing scleritis which pro- necrotizing disease was associated with inflamma-
gressed around the globe, the area behind the tion, the episclera became avascular, overlying the
advancing edge could become extremely thin, area which would eventually become sequestrated.
and sometimes melted away altogether. In the Treatment at this stage would prevent sequestration.
variety of necrotizing scleritis without inflammation In scleromalacia perforans sequestration might take
(scleromalacia perforans), the episclera was usually place without any warning. The loss of tissue in
extremely thin (Fig. 8), and the underlying sclera either condition might involve only a part of the
would become white, develop a demarcated margin, scleral thickness.
and then become sequestrated. The conjunctiva and Sequestration occurred in 22 eyes (7 per cent).
thin episclera overlying this process might be Loss of tissue took place in 41 per cent of eyes with
slightly congested but this was by no means always necrotizing disease associated with inflammation,
the case. A combination of A- and B-scan ultrasound and in 77 per cent of those without inflammation.
could be used to distinguish between thinning and The rest of these eyes developed severe thinning
increased transparency-A-scan being used to esti- without sequestration. Of eyes with scleral defects
mate the thickness and B to locate the lesion. 59 per cent also had keratitis, although this was
accompanied by a loss of corneal tissue (keratolysis)
Scleral defects (Fig. i8) in only three eyes (Fig. i6). No scleral defects
developed in patients who had episcleritis or any of
Scleral defects, as opposed to scleral thinning or the less severe forms of scleritis.
increased scleral transparency, were seen only in
the severest forms of necrotizing disease. When the Associated systemic diseases
The incidence of the various systemic conditions
detected in this series is summarized in Table XI.
In eyes where scleritis and episcleritis co-
existed, the area of severe damage occurred where
the sclera was involved, and not where the episclera
was inflamed without deep involvement.
We have observed, however, that the patients
with herpes zoster ophthalmicus developed an
episcleritis at the time of the vesicular eruption.
The episcleritis disappeared as the skin condition
subsided. Three patients developed a severe
nodular scleritis two or three months later at the
site of the original lesion. This does not imply
that the episcleritis progressed to the scleritis,
because there was a distinct interval between the
FIG. I8 Scleral defect. Scleral defect occurring in two episodes during which the eye was apparently
patient with severe necrotizing scleritis. Ciliary body is normal. Since making this observation we have
exposed but no staphyloma occurs unless intraocular studied a further 20 patients who were not included
pressure rises above 40 mmHg in this series presenting with herpes zoster ophthal-
Scleritis and episcleritis I8r
Table XI Incidence of associated systemic diseases in episcleritis and scleritis

Episcleritis Scleritis
Disease Diffuse Nodular Necrotizing Scleromalacia
Simple Nodular anterior anterior anterior perforans Posterior
Rheumatoid arthritis 5 2 8 4 3 6 0
Ankylosing spondylitis o o I 0 0 0 0
Psoriatic arthritis o o 0 3 0 0 0
Systemic lupus
erythematosus o 0 0 I 2 0 0
Periarteritis nodosa o 0 0 0 2 0 0
Systemic vasculitis o 0 I 0 I 0 0
Wegener's granulomatosis o 0 0 0 I 0 0
Relapsing polychondritis o o 0 0 2 0 0
Rheumatic heart disease 2 I I I 0 0 0
Palindromic rheumatoid o o 0 0 2 0 0
Associated with hypersensitivity

disorders
Erythema nodosum 0 0 0 2 0 0 0
Severe asthma or hay fever 0 0 2 0 0 0 0
Erythema multiforme I 0 0 0 0 0 0
Henoch-Schonlein purpura 0 0 0 0 0 0
Penicillin sensitivity 2 0 0 0 0 0 0
Contact dermatitis 2 I 0 0 0 0 0
After desensitization
vaccination 2 0 0 0 0 0 0
Rosacea I 2 0 2 0 0 0
Associated with granulomatous

conditions
Tuberculosis
Active I 0 I 0 3 0 0
Inactive I 2 0 0 0 0 0
Syphilis O I I 3 2 0 0
Associated with virus infection

Herpes zoster 2 I 2 14 0 0 0
Herpes simplex o 0 0 2 0 0 0
Associated with metabolic

disorders
Gout 6 5 2 3 0 0 0
Other associated conditions* I 0 IO 7 I 0 0
*In simple episcleritis one patient had regional ileitis

In diffuse anterior scleritis Io patients had other diseases-orbital granuloma (I), essential hypertension (3), postirradiation to other eye (I),
fragilitas osseum (I), thyrotoxicosis (I), degenerative osteoarthritis (2), ankylosing spondylitis (I)
In nodular anterior scleritis seven patients had other diseases-for example, Cogan's syndrome (3), thyrotoxicosis (2), ophthalmia nodosa (I),.
hypertension (I)
One patient with necrotizing scleritis had hypertension as the only other systemic disease. There was no other evidence of a generalized
systemic vasculitis
micus and episcleral disease. This phenomenon of In the latter part of the series plasma protein
late appearance of scleral disease was observed in estimations and electrophoresis were performed
seven of the 20 patients (35 per cent). routinely in all patients with scleritis, and an im-
munological survey performed by the Immunology
Laboratory investigations Department of the University of Glasgow. Although
these results apply only to those patients who were
The various laboratory investigations carried out in seen during the last nine months of the analysis,
these patients are mentioned on page i65. they are shown in Table XIII because they showed
Table XII Laboratory investigations: abnormal applied. Other examinations were carried out if
findings in patients indicated. Of patients in this series 42 per cent
were intensively investigated by Fowler (1970),
Episcleritis Scleritis who found sacro-iliac abnormalities in seven, of
Test whom three had definite ankylosing spondylitis.
No. (percent) No. (percent) Only one patient had symptoms which would
Erythrocyte sedimen-
have led to this diagnosis without specific examina-
tation rate 22 I4 76 37 tion.
White blood count 7 4 I4 7
Latex test for Electrodiagnostic investigations
rheumatoid factor 12 7 28 I3 5
Wassermann reaction I o-6 6 3 Electro-oculograms (EOG) were performed initially
Uric acid I8 II.3 14 7 in all patients; later in the series they were per-
formed if the patient had severe disease or posterior
scleritis. In 71 consecutive patients with episcleritis
that a more sophisticated survey would reveal and scleritis presenting in the scleritis clinic, I3
evidence of immunological abnormality not re- had subnormal EOGs in the affected eye. All but
vealed by routine laboratory testing. one of these patients had scleritis but this was not
Of the 48 patients with scleritis, 27 (56 per cent) severe in every case. The other patients had re-
had some positive result in the immunological test. current episcleritis and glaucoma.
Eight patients had more than one positive result.
These are presented with a control series of 30 Tissue biopsy
patients matched for age and sex, with eye disease
but with no scleral or uveal involvement. Biopsy of scleral tissue is not recommended unless
Of the I2 patients with a positive result in the there is great difficulty in diagnosing the eye
rheumatoid arthritis latex agglutination test, only condition or the underlying systemic disease.
two had clinical rheumatoid arthritis. Episcleral biopsies were performed on three
patients to try to elucidate the underlying disease.
Two showed infiltration of the tissue with chronic
Skin tests inflammatory cells including occasional plasma
Intradermal or prick tests-inhalant, mould, fabric, cells, but no changes specific to any other disease.
dusts, and pollens-were performed in 30 patients One revealed a lvmphomatous infiltration of the
with episcleritis, particularly those who gave some episclera.
history of allergy. Mild reactions were detected
to several agents in eight patients, but no overall
pattern of allergic response as judged by this
Treatment (see pages i87 to I89)
response could be detected. Two patients gave a The various medical treatments given for scleritis
history of a flare-up of episcleritis when in contact and episcleritis are summarized in Tables XIV
with printer's ink. The results of intradermal, patch and XV. Recently oxyphenbutazone (Tanderil)
and prick tests were negative, but vapour exposure ophthalmic ointment has been used in episcleritis
produced flushing of the episclera. and has been the subject of a double-blind controlled
trial (Watson, McKay, Clemmett, and Wilkinson,
X-ray examination 1973), but it was not used in the patients in this
series.
X-ray examination of the chest and sacro-iliac joints
was carried out routinely in all patients with
scleritis unless there was some contraindication. Surgical treatment
The seven-day rule with regard to ovulation was Three cataracts have required extraction in the
Table XIII Tissue antibody responses

Rheumatoid
Antinuclear factor Thyroid Tanned Smooth Gastric Raised
Patients factor (latex) R3 antibodv RBC muscle parietal y-globulin
Scleritis (48) 3 1I2 2 5 2 7 6 4
Control (30) 0 0 0 2 0 I 2 0
presence of active scleritis. No complications Enucleation

followed these operations. Scleritis is a serious condition and even if fully and
Four patients have had corneo-scleral grafts for carefully treated can lead to the loss of the eye, as
descemetocele formation, expansion of the globe, indeed happened in three of the patients in this
increasing corneal guttering, and threatened per- series. Only one of these patients was seen early in
foration (Fig. I9). All have done well. the disease and she was not able to tolerate steroid
Two patients were given scleral grafts to cover treatment; the eye was removed for intractable
thin sclera. The graft remained in place in one; pain after the necrotizing disease had spread
in the other patient the graft became involved by inexorably around the globe. The vision was pre-
scleritis and eroded away, leaving the choroid served until the last area of undamaged sclera
covered by a thin layer of conjunctiva. The course became involved, but then fell rapidly from 6/i8 to
of the scleral disease was not influenced by the hand movements within a week. Another eye was
grafting. removed because it was blind and extremely painful.
The third patient had severe keratolysis and
Table XIV Treatments given in episcleritis necrotizing scleritis with loss of corneal and scleral
tissue when first seen. Attempts at covering these
Simple Nodular defects by grafting failed and the eye was eventually
Treatment episcleritis episcleritis removed.
Not known 2 3 Follow-up
Placebo 6 0
Local prednisolone drops 8o 17 Of the 159 patients with episcleritis and the 207
Systemic prednisolone 2 I patients with scleritis, 146 (9i per cent) and i86
*Systemic sintisone 3 2 (go per cent), respectively, have been followed-up
Systemic other steroid 0 0
during a period of between one and eight years
Systemic antibiotics 0 0
(mean 5-8 years). All the patients who had not
Systemic oxyphenbutazone 22 2
Systemic indomethacin 2 2 attended the clinic for six months and had had the
Other systemic treatment disease for at least one year were sent a question-
for example, aspirin 4 naire in an attempt to determine the recurrence
Local steroid with rate. Altogether 332 patients, of 366 who were
systemic steroid I0 4 circularized (9I per cent), replied to the question-
Local steroid with systemic naire or returned to the clinic for follow-up.
oxyphenbutazone 17 9 In cases in which scleritis was seen in the initial
Local steroid with systemic stages and fully treated the recurrence rate was
indomethacin 5 0
Other antibiotics 17 6 only 20 per cent. The recurrence rate was higher in
those patients with anterior diffuse scleritis than
Table XV Treatments given in scleritis

Anterior
Treatment Posterior
Diffuse Nodular Necrotizing Scleromalacia
perforans
Not known 2 7 0 0 0
Placebo 0 0 0 0 0
Local steroid 22 34 0 0
Systemic prednisolone I0 7 2 0
Systemic sintisone 7 12 0 0 0
Systemic antibiotics 0 0 0 0
Systemic oxyphenbutazone 7 5I 2 2 0
Systemic indomethacin 4 0 0
Other systemic treatment I I 0 0 0
Local steroid with systemic steroid '9 I4 7 5 2
Other systemic steroid 0 0 0 0 0
Local steroid with systemic oxyphenbutazone '7 21 5 0 2
Local steroid with systemic indomethacin 4 4 0 0 0

Other combinations 26 27 '4 3 I
FIG. I9 Overlay corneo-scleral

lamellar graft on same patient as in
Fig. I5 whose astigmatism was
reduced from I5D to 6D by this
procedure. Grafting only adds
supporting tissue and does not affect
underlying pathology
with any other variety. The recurrences were also patients, six (43 per cent) had had necrotizing
more severe in scleritis than episcleritis, 6o per scleral disease, one diffuse anterior scieritis, and
cent of them requiring further hospital treatment. five nodular scleritis. None of the patients with
It is important to follow up those patients with scleromalacia perforans was known to have died
necrotizing disease because the disease can continue during this period, although many of these patients
to progress when they are symptom-free, and they
may require continuous treatment even when the
disease is quiescent. Table XVI Duration of disease
In episcleritis, however, each attack is self- Months Years
limiting and usually clears without treatment. This Disease
has been confirmed in a double-blind controlled 6 i8 3 6 10 15 20 30
trial (Watson and others, 1973) in which each
attack varied from a few hours to several days, the Episcleritis 6 35 24 I7 I I 29 0 4
average being about seven days. Episcleritis was Scleritis 10 34 24 I9 6 I 0 6
most common in the interpalpebral region, often
changing from one side to the other with each attack.
However, some patients always developed the Table XVII Percentage of recurrences
inflammation at the same site although recurrences
of both episcleritis and scleritis could occur up to Both eyes
30 years after the onset of the disease (Table XVII).
Two-thirds of the patients had recurrences up to Recurrence Total Same Other Simul- At different
six years after the onset of the disease, but after eye eye taneously times
this the recurrence rate fell. We found that there Episcleritis 64 46 5 27 22
was often a considerable interval between the onset Scleritis 69 48 4 31 15
of the disease and the first attendance of the
patient, who often came only because of apprehen-
sion caused by the recurrent attacks rather than Table XVIII Severity of recurrences (per cent)
the severity of the symptoms.
Treatment needed
Deaths (Table XIX) Disease Not At By own By self-
Of the I4 patients who were known to have died severe hospital doctor medication
during the period of follow-up, only two had Episcleritis 3 I
episcleritis. One died as a result of a road accident, 38 26 4
Scleritis 27 6o 13 I
and the other committed suicide. Of the remaining
Table XIX Cause of death in the I4 patients who died during the period of follow-up
Patient Sex Type of scleritis General conditions Cause of death
I1 Female Necrotizing Rheumatoid arthritis Coronary thrombosis
Cardiovascular disease
2 Female Necrotizing Rheumatoid arthritis Bronchopneumonia
3 Male Necrotizing Diffuse connective-tissue disease Cerebral haemorrhage
Chronic nephritis
Hypertension
4 Female Necrotizing Hypertension Cerebral haemorrhage
5 Female Necrotizing Bronchitis Congestive heart failure
Emphysema
6 Male Necrotizing Pulmonary tuberculosis Bronchopneumonia
Emphysema
Bronchiectasis
7 Male Diffuse anterior Polyarteritis nodosa Gastrointestinal haemorrhage
8 Male Nodular Rheumatoid arthritis Coronary thrombosis
9 Male Nodular Myocardial infarction
I0 Male Nodular Old herpes zoster ophthalmicus Myocardial infarction
II Male Nodular Old herpes zoster ophthalmriicus Myocardial infarction
I2 Male Nodular Stabbed to death
'3 Male Nodular Suicide
I4 Male Simple episcleritis Road accident
had long-standing rheumatoid arthritis and were associated systemic disease was 42 per cent, of
generally unwell at the time of their initial exami- which I5 per cent consisted of connective-tissue
nation. Unfortunately three of these patients diseases.
were not found for follow-up and might well have It has been suggested in the literature (Duke-
died in the interim. Of those who died of natural Elder and Leigh, I965) that rheumatoid arthritis
causes, 54 per cent had necrotizing disease, and is the commonest single associated condition, but it
27 per cent of the patients with severe necrotizing was found in only io per cent of our patients.
scleritis were dead within eight years of the onset Scleritis also occurs in association with other
of the scleritis. connective-tissue diseases-systemic lupus erythe-
Five of the patients who died were known to matosus, polyarteritis nodosa, relapsing polychon-
have had diffuse connective-tissue disease; 2I per dritis, Wegener's granulomatosis-thus reflecting
cent of all those with connective-tissue disease in the collagenous nature of the tissue of which the
this series. Another had hypertension and, it was sclera is composed. Altogether 31 patients (15 per
thought, could have had a systemic vasculitis. As no cent) were found to have manifest connective-tissue
necropsy was performed this could not be confirmed. disease; in seven of these the scleritis was the
presenting feature. In I9 other patients there were
Discussion conditions thought to have been immunologically
induced, including two who had erythema nodosum.
While scleritis is an uncommon disease, accounting A total of 40 had positive latex agglutination tests
for only o-o8 per cent of new referrals to eye for rheumatoid arthritis and a positive antinuclear
departments (Williamson, I974), of those with the factor, and if all the immunological tests are in-
necrotizing disease 21 per cent died within cluded, 72 patients (35 per cent) in the series tested
eight years of the onset. The presence of scleritis had some abnormality of this type.
may therefore portend serious systemic disease. It seems that necrotizing scleral disease repre-
Early diagnosis may lead to early treatment of the sents a different phase of scleritis in certain patients.
general condition, prolonging and probably im- In some patients who have had recurrent scleral
proving the quality of life for these patients. disease for many years the scleritis has changed in
In our present series 46 per cent of the patients type, coinciding with a change in the character of
with scleritis had a known associated systemic the systemic disease-for example, the onset of
condition. Of these, I5 per cent had definite vasculitis in rheumatoid arthritis and renal disease
connective-tissue disease, confirmed by a rheuma- in systemic lupus erythematosus. In other
tologist (Table XI). This incidence has recently patients the onset of disease is 'malignant' wit
been confirmed in an analysis of a further 88 progressive and inexorable destruction of scler..
patients with scleritis, in whom the incidence of tissues if treatment is not adequate. The mortality
i86 British Journal of Ophthalmology
in this group is very high. Finally, there appears to found to have severe active syphilis with a positive
be yet another type of scleral disease, scleromalacia cerebrospinal fluid serological result. The scleritis
perforans, which is almost completely confined to disappeared when the syphilis was fully treated.
women, half of whom have long-standing rheu- Altogether 14 patients had a positive Wassermann
matoid arthritis. In these sequestration of the reaction in this series, seven presented with simple
sclera occurs without warning and little, if any, episcleritis and the others with a diffuse anterior
inflammation. This type of scleral reaction is neither and nodular scleritis. The scleritis associated with
the same as that found in other forms of scleritis syphilis and tuberculosis was in no way different
nor does it necessarily have the same pathogenesis. from the other forms.
A biopsy is contraindicated in scleral disease The aetiology of episcleritis is even more un-
because the sclera does not heal easily-a fact well certain. However, two patterns are apparent. Most
known to surgeons performing detachment opera- patients have intermittent bouts of moderate or
tions a few years ago when scleral surgery was the severe inflammation at intervals of one to three
rule. Large scleral nodules often contain pultaceous months, lasting seven to ten days and occurring
material which, if released, exposes the underlying much more commonly during the spring and
choroid, but they may heal with fibrosis if left alone autumn than in the summer or winter. Only rarely
(Watson, I974). Much of our knowledge of the can any certain precipitating factor be found, the
underlying processes has therefore to be derived attacks usually being related to family upsets and
from the few biopsy specimens which have become worries at work. Occasionally, however, a clear
available, and from those eyes which have been history of exogenous sensitization is given-for
enucleated for severe disease (Sevel, I967; Verhoeff example, contact with printer's inks. In one instance
and King, 1958; Anderson and Margolis, 1952; the patient had only to be inside the printing works
Fransois, 1970; Henkind, I974). This unfortunately for his eyes to flare up; exposure to the vapours
means that we can find out little about the disease in of the ink also produced an attack under hospital
most of the patients other than what can be deduced conditions, but skin tests gave negative results. i i per
from their clinical examination and serological in- cent of patients with episcleritis had recurrently high
vestigations. Biopsy of the episcleral lesions showed serum uric acid values (more than 6 5 mg per cent on
oedema, separation of the episcleral fibres, infiltration three readings), and 7 per cent had clinical gout.
with lymphocytes, and some cuffing of the vessels. 12 per cent also had a history of asthma and hay
Henkind and Gold (I973) observed a rheumatoid fever; only one patient related the attacks to
nodule in the episcleral tissue but we have not seen menstruation. Two patients developed episcleritis
this. It is certain, however, that the changes typical after a desensitization vaccination.
of rheumatoid nodules occur in the sclera in certain The second group of patients gave no history of
patients with necrotizing scleritis and rheumatoid periodicity but rather of mild, prolonged attacks
arthritis. More typical, however, is a granulomatous of inflammation. Into this category came almost all
reaction in the sclera, similar to that found else- those patients who had some other intercurrent
where in the body in connective-tissue disease, the disease. Seven patients had a positive Wassermann
predominant feature being fibrinoid necrosis. reaction (one with active syphilis), four had mild
Similar lesions can be produced in animals with the rheumatoid arthritis with a positive rheumatoid
Schwartzmann reaction. It was probable therefore factor but only a few joint symptoms, and two had
that in many cases the altered immunity in the erythema multiforme; the others had active tuber-
patient was the underlying cause of this condition. culosis, Henoch-Schonlein purpura, or rosacea.
The sclera is also involved in gout, in which the Herpes simplex virus has been grown from one
response is due to inflammation caused by pro- lesion. The appearances of the episcleritis did
teolytic enzymes released from polymorphs which not differ in these two groups, and in neither
have ingested uric acid crystals. did the episcleritis progress to a scleritis except
The chronic granulomatous diseases of bacterial in those patients with herpes zoster who have
origin have also been quoted as a cause of scleritis already been discussed. General physical examin-
(Duke-Elder and Leigh, I965). We saw only two ation and chest x-ray failed to reveal any evidence
patients with active pulmonary tuberculosis and of focal sepsis.
scleritis, although ancient photographs and histo- Herpes zoster involves both the cornea and
logical sections make it clear that this was a much sclera. As already discussed, an episcleritis may
commoner condition when tuberculosis was severe occur in the early vesicular stage of the disease,
and less easily treatable. We have not knowingly disappearing completely and then presenting as a
seen scleritis in association with sarcoidosis. deep localized scleritis at the site of the original
Syphilis, however, can present as a scleritis. lesion some two to three months later. Alternatively
Four patients have presented in this way with the the sclera may be involved early in the disease, in
eye changes as their only symptom. They were which case a nodular scleritis is the rule.
Clinical gout was found in five patients who be from the tissue involved in order that
presented with episcleral and scleral disease, and complexes may be formed elsewhere. In
32 had raised levels of uric acid on more than one general, one would expect the antigen to
occasion. The serum uric acid is a notoriously un- be found at the site of the lesion, for instance
reliable test and a single positive recording should the thyroid antigen in autoimmune thyroiditis.
not be taken as indicative of underlying gout. It was It should be possible to:
observed that those patients with clinical gout and 2. Extract the antigen.
scleral and episcleral disease had deposits of uric 3. Reproduce the disease in some experimental
acid beneath the conjunctiva around the anterior animal.
ciliary vessels at the plica. It was hoped that this 4. Demonstrate specific antibodies to the antigen
could be used as a diagnostic sign, but deposits or specifically sensitize cells reacting with
which look almost identical are frequently seen in antigen.
patients whose serum uric acid is normal. We were not able to fulfil these postulates partly
It could be suggested that this group of patients because of the undesirability of obtaining biopsy
with episcleritis may represent the earlier stages of tissue in our patients, and because of uncertainty that
a benign form of scleritis. If this was so, one would the disease produced in animals was of the same
expect a significant number of cases to progress nature. However, the circumstantial evidence from
from episcleritis to scleritis, but during the eight- the well-proven association with known connective-
year period of follow-up we did not see this. We tissue disease is strongly suggestive that this is a
did see three patients who had episcleritis in one manifestation of immune complex disease in a
part and scleritis in another part of the same large number of cases. In herpes zoster ophthal-
eye. However, it is an interesting and definite micus and gout the precipitating cause is known,
observation that the episcleritis did not progress but until more is known about the precipitating
to scleritis even during a prolonged follow-up. factors of the immune-complex disease we are not
We feel that this suggests that episcleritis and justified in making any further conclusions.
scleritis are two different types of reaction to a Whatever the pathogenesis might be, we found
similar antigen within the different tissues. Thus the that by careful differential diagnosis we were able
fact that the episclera is involved in the course of to treat the disease appropriately and adequately
the disease does not mean that the underlying so as to prevent a serious destructive change
sclera would become involved, even after a long taking place in the eye. The differential diagnosis
period of time. We have, as yet, no definite evidence of scleritis and episcleritis is summarized in
that scleritis is an autoimmune disease, and the Table XX.
evidence presented in this paper is only circum-
stantial. Equivalents of Koch's postulates which TREATMENT
need to be satisfied for auto-immune disease are
as follows (Glynn, 1974): Apart from the very few patients in whom a
i. There must be a specific antigen. It may not specific bacterial or viral agent could be found to
Table XX Cardinal features in the differentiation of scleritis from episcleritis

Episcleritis Scleritis
Anterior
Posterior
Scleromalacia
Simple Nodular Nodular Diffuse Necrotizing perforans
Onset Sudden Usually Gradual Gradual Gradual Insidious Gradual
sudden
Redness Bright red Red Dusky red Bluish red Bluish red Very slight Variable
Pain Slight Slight Moderate, Moderate, Severe, Slight, if any Severe,
localized localized localized and localized and localized and localized and
referred referred referred referred
Photophobia and Occasionally Occasionally Occasionally Occasionally Common None Unusual
lacrimation
Vsacular changes Superficial Superficial Superficial and Superficial and Superficial and Avascular area Superficial
episcleral episcleral deep episcleral deep episcleral deep episcleral and deep
and occasionally episcleral
avascular patch
Chemosis Occasionally Rare Rare Common Variable Absent Common
Scleral thinning or Rare Rare Common Common Common Always Rare
transparency
Visual acuity Normal Normal Normal Occasional loss Common loss Common loss Very common
loss
i88 British Journal of Ophthalmology
have caused episcleritis or scleritis, the treatment multiple recurrences, we recommend the following
is suppression of the inflammation in order to routine:
relieve the discomfort and prevent the destructive Topical medication: Any one of the following
changes which may accompany the inflammation. medications may be used:
Treatment, which must be continued until the i. Prednisolone eye drops 0o5 per cent hourly
disease has followed its natural course, has proved until 24 hours after the inflammation has
to be effective and many eyes are being saved or subsided, then reduced to three times a day
prevented from developing severe complications. for a further four days.
Only three eyes (o.6 per cent) were lost-one that 2. Betamethasone (Betnesol) eye ointment i per
of a patient who could not tolerate the treatment cent four times a day during the attack, and for
rather than because of the severity of the eye disease. a further week after the attack has subsided.
3. Oxyphenbutazone (Tanderil) eye ointment
EPISCLERITIS io per cent four times a day during the
As the condition is self-limiting, harmless, and attack, and for a further week after the attack
frequently symptomless, treatment may not be has subsided.
necessary. Double-blind controlled studies have Systemic therapy: Occasionally patients will not res-
been conducted to determine first whether any pond adequately to local therapy, in which case
treatment was necessary and secondly which systemic oxyphenbutazone, indomethacin (Indocid),
drugs were most effective in suppressing the or corticosteroid may be needed.
inflammation. i. Oxyphenbutazone: 6oo mg daily in divided
In this trial some patients received placebo, doses is given until there is a response. The
others local steroid ointment, and the remainder dose is then reduced to 400 mg until the eye is
oxyphenbutazone ointment. All the patients re- quiet and then it is stopped. This treatment
covered within I0 days. Both oxyphenbutazone is given in addition to local treatment.
eye ointment and betamethasone ointment ap- 2. Indomethacin: If the patient fails to make a
peared to hasten the recovery by about one day, rapid recovery the drug may be changed to
but the differences were not significant (Watson indomethacin I00 mg daily in divided doses,
and others, 1973) (Fig. 20). and then reduced to 75 mg as soon as there is
If it is felt necessary to treat the patient, either a response. A double-blind controlled study
because of the symptomatology or because of just completed indicates that oxyphenbuta-
zone and indomethacin are equally effective
100. as anti-inflammatory agents, although some
patients respond better to one drug than the
90 other.
80 3. Corticosteroids: In the rare case which does
.t ..'* not respond to local treatment, or systemic
70 oxyphenbutazone or indomethacin, it is
E
justifiable to give a short, intensive course of
prednisolone or its equivalent. A suppressive
t; 50 dose for this condition is 6o mg prednisolone
c0
E 40
daily which, as soon as the inflammation starts
to regress, can be reduced rapidly with a view
$ 0,
30 Betamethasone to withdrawing the drug entirely within one
.........Oxyphenbutazone month.
20
Placebo A double-blind controlled study was carried out
10 in these patients (Watson, Lobascher, Sabiston,
Lewis-Faning, Fowler, and Jones, I966) which
0 revealed that both oxyphenbutazone and predni-
0 7 14 21 solone were effective, but that patients receiving
Days
systemic prednisolone had a rebound of their
FIG. 20 Improvement of conjunctival and episcleral symptoms, while those having oxyphenbutazone
injection in patients with episcleritis treated with remained symptom-free.
either placebo, oxyphenbutazone ointment or
betamethasone per cent ointment. Although those
i
treated with active substances improved slightly faster, SCLERITIS

8o per cent of those receiving placebo had recovered by Because of the depth and severity of the inflam-
2I days mation local steroid treatment is ineffective. Drops
or ointment are soothing and reduce the inflam- after which time they rapidly regress. As soon as
mation but are not sufficient to control the scleritis. the condition is brought under control the dosage
As a result of our experience we have come to can be dropped within a week to a maintenance
the conclusion that the following regimen of level which is usually between io and 20 mg
treatment is the most likely to be effective: prednisolone daily. Cautious reduction from this
As soon as scleritis is diagnosed: level usually enables the patient to be taken off the
Systemic oxyphenbutazone 6oo mg daily in drug or to be maintained with another anti-inflam-
divided doses for four days, followed by 400 mg matory agent within three to four weeks. In patients
daily until the condition is suppressed. This dosage who have been unable to tolerate, or have failed to
is continued for one week after all symptoms have respond to, prednisolone, occasionally a response
subsided. Or can be obtained with Sintisone (prednisolone
Systemic indomethacin I00 mg daily in divided 2I-stearoylglycolate), eight tablets (each tablet
doses, reduced to 75 mg daily in divided doses as soon containing o-63 mg) daily in divided doses (Hayreh
as there is a response. Treatment is continued until and Watson, I970). Why this preparation is so
the condition has been quiescent for seven days. effective is uncertain, but we have several patients
These two drugs appear to be more effective who have to be maintained on it rather than on the
than any of the other anti-inflammatory agents in conventional steroids.
general use-for example aspirin, ibuprofen (Bru-
fen), etc. Surgery
Surgery is very rarely necessary in scleral disease.
Local steroids Provided the condition is seen early and treated
Usually prednisolone or betamethasone can be vigorously, scleral necrosis can be largely prevented.
given in addition. Comeal grafting has had to be performed in four
patients because of threatened perforation, an
Subconjunctival steroids are not recommended in expanding globe, for peripheral corneal guttering,
scleritis and for descemetocele. The corneal grafts have
taken well and in all cases the scleritis has become
We have seen two patients in whom extreme less severe after this was done.
thinning has occurred at the site of a subconjunc- Many reports appear in the literature of scleral
tival injection of steroid (Watson, I974; Fraun- grafting for scleromalacia perforans (Holthouse,
felder and Watson, 1976). It is not certain whether it I893; Ashton and Hobbs, 1952; Torchia, Dunn,
is the vehicle in which the steroid is contained or Pease, I968; Vancea, Vancea, Jalobceastii, Colev,
the steroid itself which is the irritating factor. We and Calin, 1971; Sevel and Abramson, 1972; and
have from time to time given intraorbital steroid others). This has been necessary in only two patients
injections in patients unable to take steroid syste- in this series-neither of whom could tolerate
mically. The effect is transient and the injections steroids. In both patients the graft took well.
have to be repeated weekly to be effective. Most In one the whole necrotic area was removed and
patients will not tolerate this regimen and so it is the residual area of scleral inflammation could be
not satisfactory for routine use. controlled with local steroids and oxyphenbutazone
systemically. In the other the necrotizing process
Systemic corticosteroids continued from the edge of the scleral overlap and
the eye was eventually lost, no other medical
If the scleritis is very severe, and does not respond treatment being tolerated.
to the oxyphenbutazone or indomethacin within a We consider scleritis to be a medically treatable
week, or if any avascular areas appear in the condition, and provided the dosages of the drugs
episclera, systemic steroids must be given in heavy are adequate to suppress the inflammatory change,
suppressive doses. The sclera is avascular and high the condition will be controlled. In this we disagree
concentrations are required to produce a thera- with Sevel and Abramson (1972) and Aronson and
peutic effect. Elliott (I974). We feel our results justify our claim.
Systemic prednisolone Summary

8o mg a day in divided doses is usually sufficient The data from I59 patients (217 eyes) with epis-
as an initial dose. If it is effective the response is scleritis and 207 patients (30I eyes) with scleritis
dramatic; although there is little change in 24 have been investigated in detail and the results
hours or even 48 hours, the pain disappears. The analysed with the help of a computer. Of these
eye signs may not improve for a further 48 hours, patients, 9I per cent were followed-up during a
o90 British Journal of Ophthalmology
period of one to eight years. A new classification 21 per cent of these patients were among those that
is presented which is as follows: died. It is probable that necrotizing scleritis
Episcleritis (217 eyes) represents the eye changes of the malignant phase of
Simple episcleritis (170 eyes) systemic connective-tissue disease.
Nodular episcleritis (47 eyes) Treatment is unnecessary in episcleritis but in
Scleritis (30I eyes) scleritis systemic treatment with anti-inflammatory
Diffuse anterior scleritis (i I9 eyes) agents or corticosteroids is obligatory. Using these
Nodular anterior scleritis (134 eyes) routines of treatment only three patients (o-6
Necrotizing scleritis (42 eyes). Of these, 13 were per cent) have lost an eye.
regarded as scleromalacia perforans. Apart from ourselves, the following have worked in
Posterior scleritis (6 eyes) the Scleritis Clinic during the past io years: A. Atkinson,
The diagnosis is based on an exact clinical P. Awdry, P. E. Cleary, R. S. Clemett, P. Fowler,
A. Freedman, B. Hazleman, P. Henderson, A. D.
examination which is fully described. Holt-Wilson, D. Jones, S. Lachmann, D. Lloyd-Jones,
Episcleritis has been shown to be a benign D. Lobascher, A. J. Lyne, and D. A. R. Mackay, and
recurring condition, a mild keratitis being the only we would like to thank them all for their help and
occasional complication. Episcleritis does not ideas. We are sure many of these ideas are incorporated
progress to scleritis, except in the case of herpes in this article but it is impossible to acknowledge them
zoster which sometimes starts as an episcleritis properly because we have forgotten where they origi-
with the vesicular stage of the eruption, to reappear nated. We should also like to thank all those surgeons
three months later as a scleritis in the same site. who have referred patients to us: without them there
No clear conclusions could be drawn as to the would be no clinic.
The computer analysis was completed at the Institute
aetiology of episcleritis. of Ophthalmology and many of the illustrations were
Scleritis is always accompanied by episcleral reproduced there; the rest were done in the Medical
inflammation which must be ignored in making Illustration Department of Addenbrooke's Hospital,
the diagnosis. Scleritis is a much more severe disease Cambridge.
than episcleritis, leading to loss of visual acuity Figs 6 and I4 were reproduced from the Transactions
from corneal changes, uveitis, cataract, or retinal of the Ophthalmological Society of the United Kingdom;
detachment if not treated. Necrotizing scleritis with Fig. i6 from Connective-tissue disease in the eye, in
inflammation is the severest form of the disease Recent Advances in Ophthalmology, ed. P. Trevor-Roper,
and 2I per cent of the patients with this condition published by Blackwell; and Fig. 20 from the British
died within the eight-year-period of follow-up. Journal of Ophthalmology.
Last but not least we should like to thank all those
Connective-tissue disease was the commonest who have at various times typed part or all of this
associated general condition; the incidence of manuscript, in particular Mrs J. Field and Mrs J.
necrotizing scleritis was higher in this group and McCombie.
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Scleritis and episcleritis.
P. G. Watson and S. S. Hayreh
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Brit. J. Ophthal. (I 976) 6o, I 63

Scleritis and episcleritis

164 British Journal of Ophthalmology

FIG. i Normal vascular networks of

Scleritis and episcleritis I65

I66 British Journal of Ophthalmology

Scleritis and episcleritis i67

I68 British Journal of Ophthalmology

Table II Types of pain experienced in different types of scleritis (percentage)

Scieritis and episcleritis 169

FIG. zSlit-lamp appearances in scleritis.

~. complications other than scieral thinning and 40

the conjunctival sac so that the deep scleral 4

occasionally grossly distorted and the normal radial

170 British Journal of Ophthalmology

FIG Abnormal, tortuous vascular

had become involved (Fig.

mation subsided, or was suppressed by treatment,

FIG. 6 Necrotizingr anterior scleritis. Superficially this-

patients with necrotizing scleritis with inflammation v

Scleritis and episcleritis I7I

FIG. 8 Necrotizing scleritis without

172 British Journal of Ophthalmology

FIG. 9 Appearance of fundus after

FIG. IO Posterior scleritis. Retraction

Scleritis and episcleritis 173

within the muscle cone by the inflammatory

FIG. i iEpiscleritis. Although deep vascular

174 British Journal of Ophthalmology

Complications of some sort were found in only

EPISCLERITIS Necrotizing scleritis

Scleritis and episcleritis 175

Table V Keratitis and vascularization of the cornea in scleritis

'FIG. 13 Acute stromal keratitis in

176 British Journal of Ophthalmology

FIG. 14 Sclerosing keratitis in

Limbal guttering these metabolites to the limbus and it was not a

Scleritis and episcleritis I77

FIG. 15 Limbal guttering in

\10\Vio-yearhistory of unilateral scleritis

c..>f.'Terrien'-like change. This area

required grafting (Fig. I9) because

worse during the course of the disease. All these

I78 British Journal of Ophthalmology

FIG. 17 Deep stromal corneal

Anterior uveitis Anterior uveitis

Scleritis and episcleritis I79

Table IX Glaucoma in scleritis

I8o British _ournal of Ophthalmology

Table X Scleral thinning or transparency in scleritis

Scleritis and episcleritis I8r

Table XI Incidence of associated systemic diseases in episcleritis and scleritis

Associated with hypersensitivity

Associated with granulomatous

Associated with virus infection

Associated with metabolic

*In simple episcleritis one patient had regional ileitis

I82 British Journal of Ophthalmology

Table XIII Tissue antibody responses

Scleritis and episcleritis I83

presence of active scleritis. No complications Enucleation

Table XV Treatments given in scleritis