Severity of Scleritis

and Episcleritis

Maite Sainz de la Maza, MD, PhD, Nada S. Jabbur, MD,

e. Stephen Foster, MD, FACS

Purpose: Inflammation of the wall of the eyeball may extend to adjacent ocular
tissues with blinding consequences and may be associated with potentially lethal sys-
temic disorders. This study was undertaken to evaluate the ocular complications and
systemic disease associations of the different types of scleritis and episcleritis.
Methods: Ocular complications and specific disease association were evaluated in
266 patients (358 eyes) with different types of scleritis (diffuse, nodular, necrotizing,
scleromalacia perforans, and posterior) and episcleritis (simple and nodular).
Results: In patients with scleritis, decrease in vision occurred in 37%, anterior uveitis
was present in 42%, peripheral ulcerative keratitis developed in 14%, glaucoma occurred
in 13%, cataract formed in 17%, fundus abnormalities appeared in 6%, and specific
disease association was uncovered in 57%. These findings were most commonly as-
sociated with necrotizing scleritis. In patients with episcleritis, decreased vision occurred
in 2%, anterior uveitis was present in 11 %, glaucoma developed in 4%, cataract formed
in 2%, and specific disease association was uncovered in 32%. These findings were
similar in simple and nodular episcleritis.
Conclusions: In a patient with scleritis, examination of visual acuity, anterior uvea,
cornea, lens, intraocular pressure, and fundus must be performed in every follow-up
visit, and a meticulous approach for detection of a specific associated disease must be
undertaken since the first visit. Scleritis is more severe than episcleritis, and necrotizing
scleritis is the most severe type of scleritis. Classification of scleritis and episcleritis
provides valuable prognostic information. Ophthalmology 1994;101:389-396

Inflammation of the wall of the eyeball includes a spec-
trum that ranges from harmless simple episcleritis to
painful, sight-threatening, destructive necrotizing scleritis.
The inflammatory process may extend to the adjacent
tissues causing ocular complications such as uveitis, ker-
atitis, glaucoma, cataract, optic neuritis, macular edema,
subretinal mass, annular ciliochoroidal detachment, se-
rous retinal detachment, or perforation of the globe. In
addition, scleritis may be associated with potentially lethal
systemic disorders. 1- 8
Originally received: March 31,1993.
Revision accepted: August 10, 1993.
From the Ocular Immunology Service and the Hilles Immunology Lab-
oratory, Massachusetts Eye and Ear Infirmary, Harvard Medical School,
Boston.
Supported in part by MEC/Fulbright grant FU 90/37276861 and the
Susan Morse Hilles Fund, Boston, Massachusetts.
Reprint requests to C. Stephen Foster, MD, Immunology Service, Mas-
sachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114.
Some patients experience a single, relatively brief, be-
nign episode of scleritis or episcleritis. Others have recur-
rent and/or prolonged attacks. As with other recurrent
conditions, both physicians and their patients are eager
to know the probabilities for ocular and systemic com-
plications of scleritis and episcleritis. Because of the com-
parative rarity of these diseases, statistics on severity and
prognosis are not so easy to find.
The excellent episcleritis and scleritis survey and trea-
tise performed by Watson and Hayreh 7 at Moorfields Eye
Hospital in London in 1976 provided us with an excep-
tional perspective of the spectrum of these diseases. They
proposed a classification based on the anatomic site and
clinical appearance of the inflammation at presentation.
This classification was useful because the majority of the
patients remained in the same clinical type throughout
the course of their disease. A subsequent study performed
by Tuft and Watson 8 at the same hospital in 1991 on
progression of scleritis confirmed these findings; only 8%
of their patients progressed from one type of scleritis to

389
 Ophthalmology Volume 101, Number 2, February 1994
another. This classification also was found to be useful
because it relates clinically to the severity of the disease.
Different types of scleritis differ in degree of ocular and
systemic complications.
Fifteen years after Watson and Hayreh 7 did their study
on episcleral and scleral inflammation, we performed a
scleritis and episcleritis survey at the Massachusetts Eye
and Ear Infirmary in Boston, and compared patient char-
acteristics, ocular complications, and systemic disease as-
sociations between patients with scleritis and patients with
episcleritis, between patients with different types of scleri-
tis, and between patients with different types of episcleritis.
Our study on scleritis and episcleritis patients is the second
largest to date, after the one performed in England. We
found, as Watson and co-workers did, that classification
of scleral and episcleral inflammation is useful as a ba-
rometer of severity and as an indicator for therapeutic
decisions.
Patients and Methods
We reviewed the records of all patients with scleritis or
episcleritis seen on the Immunology Service of the Mas-
sachusetts Eye and Ear Infirmary during the II-year pe-
riod from May 1980 to May 1991 on whom follow-up
information was adequate. For the purpose of this study,
scleritis was defined by the following characteristics: (1)
edema of the sclera and the episclera which displaces both
the superficial and deep edges of the thin slit-lamp beam
forward as the beam makes an excursion across the surface
of the sclera; (2) associated congestion of the superficial
and the deep episcleral vessels; congestion of the deep
episcleral vessels remains after the application of 10%
phenylephrine drops. Episcleritis was defined by the fol-
lowing characteristics: (1) edema of the episclera which
displaces the superficial edge of the thin slit-lamp beam
forward as the beam makes an excursion across the surface
of the sclera; (2) associated congestion of the superficial
episcleral vessels, which disappears after the use of 10%
phenylephrine drops. Scleritis and episcleritis were clas-
sified after the anatomic types proposed by Watson and
Hayreh.7 Scleritis was divided in anterior and posterior.
Anterior scleritis included diffuse, nodular, necrotizing
with inflammation (necrotizing), and necrotizing without
inflammation (scleromalacia perforans) scleritis. Epis-
cleritis was divided in simple and nodular.
Patient data, including age, sex, scleral disease laterality,
and follow-up period, were recorded. The following con-
ditions were considered ocular or systemic complications:
( 1) decrease in vision, defined as decrease in visual acuity
greater than or equal to two Snellen lines at the end of
the follow-up period, or visual acuity of 20/80 or worse
at presentation (worse of the 2 eyes); (2) anterior uveitis
detected at some point during the course of the disease;
(3) peripheral ulcerative keratitis, defined as corneal
stromal infiltration with epithelial defect adjacent to the
region of the scleral inflammation which may progress
circumferentially and centrally; (4) glaucoma; (5) cataract;
(6) fundus findings, including choroidal folds, subretinal
390
mass, optic nerve swelling, macular edema, annular cili-
ochoroidal detachment, serous retinal detachment, intra-
retinal deposits, and retinal striae; and (7) specific disease
association, ascribed to patients as a result of compatible
historical, review of systems, laboratory, and biopsy data.
Data were analyzed using a customized database soft-
ware and compared between patients with scleritis and
patients with episcleritis, between patients with different
types of scleritis (diffuse anterior, nodular anterior, nec-
rotizing anterior, scleromalacia perforans anterior, and
posterior scleritis), between patients with different types
of episcleritis (simple and nodular), and between patients
with necrotizing scleritis and patients with other types of
scleritis. We chose to report the results in terms of patients
(age, sex, bilaterality, decrease in vision, anterior uveitis,
peripheral ulcerative keratitis, glaucoma, cataract, fundus
findings, and specific disease association) because results
of both eyes of a patient are dependent. To determine the
extent that an observed series of proportions differed from
a theoretic or expected distribution of proportions, sta-
tistical analysis was performed using the chi-square test
(statistically significant: P < 0.05) for all conditions except
age. Patients with scleromalacia perforans were not in-
cluded in the chi-square statistical studies due to low
number (the expected values were sometimes less than
5). The condition "age" was statistically analyzed between
three or more groups with the Kruskal-Wallis nonpara-
metric test and between two groups with the Mann-Whit-
ney U nonparametric test.
Results
Patient Characteristics
A total of 266 patients (358 eyes) constituted the study
population. One hundred seventy-two of the patients had
scleritis and 94 had episcleritis (Table 1). Most of the pa-
tients with scleritis had anterior scleritis (94%), including
77 (45%) with diffuse scleritis, 39 (23%) with nodular
scleritis, 39 (23%) with necrotizing scleritis, and 6 (3%)
with scleromalacia perforans. Seventy-eight ofthe patients
with episcleritis (83%) had simple episcleritis and 16 (17%)
had nodular episcleritis. Episcleritis did not progress to
scleritis in these patients.
The mean age of patients with scleritis was 51.59 years
(range, 11-87 years) (Table 2). Patients with necrotizing
scleritis (mean age, 61.92 years) were older than those
with other types of scleritis (P = 0.0001) (Tables 2 and
3), including patients with nodular scleritis (mean age,
49.80 years), posterior scleritis (mean age, 49.10 years),
and diffuse scleritis (mean age, 46.82 years). Patients with
scleritis were older than patients with episcleritis (mean
age, 43.39 years; range, 14-79 years) (P = 0.0012) (Tables
2 and 4).
Sixty-seven of the patients with scleritis (39%) were
men and 105 (61%) were women (Table 5). Twenty-four
of the patients with episcleritis (26%) were men and 70
(74%) were women. Female predominance did not differ
significantly among patients with different types of scleri-
tis, or among those with different types of episcleritis.
 Sainz de la Maza et al . Severity of Scleritis and Episcleritis

Table 1. Scleritis and Episcleritis Classification

No. of No. of No. of No. of
Diagnosis Patients (%) Eyes Type Patients (%) Eyes
Scleritis 172 (64.7) 231 Diffuse 77 (44.77) 107
Nodular 39 (22.67) 50
Necrotizing 39 (22.67) 48
Scleromalacia 6 (3.49) 11
Posterior 11 (6.40) 15
Episcleritis 94 (35.3) 127 Simple 78 (82.98) 108
Nodular 16 (17.02) 19
Total 266 358

Bilateral inflammation was present in 34% of patients
with scleritis (Table 6); it was more frequent in scleroma-
lacia perforans (83%) as opposed to other types of scleritis,
including diffuse scleritis (39%), posterior scleritis (36%),
nodular scleritis (28%), and necrotizing scleritis (23%).
Bilateral inflammation was present in 35% of the patients
with episcleritis; it was more frequent in simple episcleritis
(40%) than in nodular episcleritis (12%) (P = 0.0375).
The mean follow-up period was 15 months (range, I-
II years) for patients with scleritis and 11 months (range,
1 month-II years) for patients with episcleritis.
Ocular Complications
Decrease in Vision. A decrease in visual acuity greater
than or equal to two Snellen lines at the end of the follow-
up period, or a visual acuity of 20/80 or worse at presen-
tation (worse of the 2 eyes) was recorded in 37% of the
patients affected by scleritis (Table 7). The association
between decrease in vision and type of scleritis was sta-
tistically significant (P = 0.0001): necrotizing scleritis was
the most common type of scleritis associated with a de-
crease in vision (82%) (P = 0.0001) (Tables 3 and 7),
followed by posterior scleritis (45%). The most common
reasons for decrease in vision were anterior uveitis and/
or peripheral ulcerative keratitis in necrotizing scleritis,
and macular edema in posterior scleritis.
A decrease in visual acuity greater than or equal to two
Snellen lines at the end of the follow-up period, or a visual
acuity of 20/80 or worse at presentation (worse of the 2
eyes) was detected in 2% of the patients (3 eyes) with epi-
scleritis (Table 7); this decrease in vision was attributed
to the natural development of cataract in all three eyes.
Decrease in vision was more commonly associated with
scleritis (37%) than with episcleritis (2%) (P = 0.0001)
(Tables 4 and 7).
Anterior Uveitis. Anterior uveitis was present in 42%
of the patients (95 eyes) with scleritis (Table 8). The an-
terior uveitis associated with scleritis ranged from mild
to moderate intensity and most commonly appeared dur-

Table 3. Statistical Associations between

Table 2. Age Distribution in Scleritis Necrotizing Scleritis and Other Conditions
and Episcleritis
Condition p-
Age (yrs) Age 0.0001
Diagnosis/Type Mean Range P Sex NS
Scleritis 51.59 11-87 Bilaterality NS
Diffuse 46.82 11- 78 Disease association 0.0001
Nodular 49.80 18-82 Decrease in visiont 0.0001
Necrotizing 61.92 34-87 0.0001- Anterior uveitis 0.0001
Scleromalacia 61.67 38-75 Peripheral ulcerative keratitis 0.0001
Posterior 49.10 26-67
Episcleritis 43.39 14-79 Glaucoma 0.0287
Simple 42.09 14-79 NSt Cataract 0.0001
Nodular 49.75 20-78
NS = not significant.
Total 48.83 11-87 • Chi-square between necrotizing scleritis and nonnecrotizing scleritis
(diffuse, nodular, scleromalacia, and posterior) for all conditions except
NS = not significant. age (Mann-Whitney U).
- Kruskal-Wallis nonparametric test between the different types of scleritis. t Decrease in visual acuity greater than or equal to two Snellen lines at
t Mann-Whitney U non parametric test between the different types of the end of the follow-up period, or visual acuity of 20/ SO or worse at
episcleritis. presentation; mean follow-up, 15 months (range, 1-132 months).

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 Ophthalmology Volume 101, Number 2, February 1994

Table 4. Statistical Associations between Scleritis Table 6. Bilaterality in Scleritis and Episcleritis
or Episcleritis and Other Conditions
Diagnosis/Type Bilaterality (Ofo) Total Chi-square
Condition P* Scleritis 59 (34.30) 172
Age 0.0012 Diffuse 30 (38.96) 77
Sex 0.0274 Nodular 11 (28.21) 39
Necrotizing 9 (23.08) 39 NS*
BUaterality NS Scleromalaciat 5 (83.33) 6
Disease association 0.0001 Posterior 4 (36.36) 11
Decrease in visiont 0.0001 Episcleritis 33 (35.11) 94
Anterior uveitis 0.0001 Simple 31 (39.74) 78 0.0375
Peripheral ulcerative keratitis 0.0001 Nodular 2 (12.50) 16
Glaucoma O.oz5
NS = not significant.
Cataract 0.0003
• p > 0.05.
NS = not significant. t Not included in the chi-square study due to low number of patients.
• Chi-square between scleritis and episcleritis for all conditions except
age (Mann-Whitney U).
t Decrease in visual acuity greater than or equal to two Snellen lines at
the end of the follow-up period, or visual acuity of 20/80 or worse at scleritis (Table 9). The presence of peripheral ulcerative
presentation; mean follow-up period for scleritis, 15 months (range, 1 keratitis was statistically associated with the type of scleritis
month-II years); mean follow-up period for episcleritis, 11 months (range, (P = 0.0001): peripheral ulcerative keratitis was present
1 month-lO years).
in 41 % of the patients with necrotizing scleritis (P =
0.000 I) (Tables 3 and 9), 6% of the patients with diffuse
scleritis, and 8% of the patients with nodular scleritis.
ing the late course of the scleral inflammation. The pres- None of the patients with episcleritis had peripheral ul-
ence of anterior uveitis was statistically associated with cerative keratitis. There was obviously a statistical asso-
the type of scleritis (P = 0.0012): it was present in 69% ciation between the presence of peripheral ulcerative ker-
of the patients with necrotizing scleritis (P = 0.0001) (Ta- atitis and scleritis (P = 0.000 I) (Tables 4 and 9).
bles 3 and 8) and in 45% of the patients with posterior Glaucoma. Glaucoma was detected in 13% ofthe pa-
scleritis. Six of the seven eyes with posterior scleritis and tients (30 eyes) with scleritis (Table 10); of those, 13 eyes
anterior uveitis also had some degree of anterior scleritis. had keratitis and uveitis, 3 had keratitis without uveitis,
Anterior uveitis was present in II % of the patients (12 and 6 had uveitis without keratitis. The patient with pos-
eyes) with episcleritis (Table 8). The uveitis associated terior scleritis had a secondary angle closure glaucoma
with episcleritis was mild. Anterior uveitis was more due to annular ciliochoroidal detachment. Although the
commonly associated with scleritis (42%) than with epi- presence of glaucoma was not associated with the type of
scleritis (II %) (P = 0.000 I) (Tables 4 and 8).
Peripheral Ulcerative Keratitis. Peripheral ulcerative
keratitis was present in 14% of the patients (34 eyes) with Table 7. Decrease in Vision in Scleritis
and Episcleritis*
Table 5. Sex Distribution in Scleritis Diagnosis/Type Loss of Vision (Ofo) Total Chi-square
and Episcleritis
Scleritis 64 (37.21) 172
No. of No. of Diffuse 20 (25.97) 77
Males Females Chi- Nodular 5 (12.82) 39
Diagnosis/Type (Ofo) (Ofo)* Total square Necrotizing 32 (82.05) 39 0.0001
Scleromalaciat 2 (33.33) 6
Scleritis 67 (38.95) 105 (61.05) 172 Posterior 5 (45.45) 11
Diffuse 33 (42.86) 44 (57.14) 77 Episcleritis 2 (2.13) 94
Nodular 13 (33.33) 26 (66.67) 39 Simple 2 (2.56) 78 NSf
Necrotizing 17 (43.59) 22 (56.41) 39 NS* Nodular 0 16
Scleromalaciat 1 (16.67) 5 (83.33) 6
Posterior 3 (27.27) 8 (72.73) 11 NS = not significant.
Episcleritis 24 (25.53) 70 (74.47) 94 • Decrease in visual acuity greater than or equal to two Snellen lines
Simple 21 (26.92) 57 (73.08) 78 NS* (worse of the 2 eyes) at the end of the follow-up period, or visual acuity
Nodular 3 (18.75) 13 (81.25) 16 of 20/80 or worse at presentation; mean follow-up period for scleritis,
15 months (range, 1 month-ll years); mean follow-up period for epis-
NS = not significant. cleritis, 11 months (range, 1 month-l0 years) .
• p > 0.05. t Not included in the chi-square study due to low number of patients.
t Not included in the chi-square study due to low number of patients. t p > 0.05.

392
 Sainz de la Maza et al Severity of Scleritis and Episcleritis

Table 8. Anterior Uveitis in Scleritis Table 10. Glaucoma in Scleritis and Episcleritis
and Episcleritis
Diagnosis /Type Glaucoma (%) Total Chi-square
Anterior Scleritis 22 (12.79) 172
Diagnosis/Type Uveitis (%) Total Chi-square Diffuse 7 (9.09) 77
Scleritis 73 (42.44) 172 Nodular 4 (10.26) 39
Diffuse 28 (36.36) 77 Necrotizing 9 (23.08) 39
Nodular 11 (28.21) 39 Scleromalaciat 1 (16.67) 6
Necrotizing 27 (69.23) 39 0.0012 Posterior 1 (9.09) 11
Scleromalacia" 2 (33.33) 6 Episcleritis 4 (4.25) 94
Posterior 5 (45.45) 11 Simple 3 (3.85) 78
Episcleritis 10 (10.64) 94 Nodular 1 (6.25) 16
Simple 9 (11.54) 78 NSt
Nodular 1 (6.25) 16 NS = not significant.
• P > 0.05.
NS = not significant. t Not included in the chi-square study due to low number of patients.
• Not included in the chi-square study due to low number of patients.
t P > 0.05.

with posterior scleritis. In our series of 11 patients with

scleritis, it was present in 23% of the patients (13 eyes)
with necrotizing scleritis (P = 0.0287) (Tables 3 and to).
Glaucoma was present in 4% of the patients (5 eyes)
with episcleritis (Table 10); of those, four eyes had chronic
open-angle glaucoma before the onset of episcleritis.
Glaucoma was more commonly associated with scleritis
(13%) than with episcleritis (4%) (P = 0.025) (Tables 4
and to).
Cataract. Cataract was present in 17% of the patients
(38 eyes) with scleritis (Table 11). Sixteen of these eyes
had concomitant anterior uveitis, and most had posterior
subcapsular cataract. The presence of cataract was statis-
tically associated with the type of scleritis (P = 0.0002):
cataract was present in 41 % of the patients with necrotizing
scleritis (P = 0.0001) (Tables 3 and 11),9% of the patients
with diffuse scleritis, and 10% of the patients with nodular
scleritis.
Age-related nuclear sclerotic cataract was present in
2% of the patients (3 eyes) with episcleritis. Cataract was
more commonly associated with scleritis (17%) than with
episcleritis (2%) (P = 0.0003) (Tables 4 and 11).
Fundus Findings. Few patients with diffuse, nodular,
or necrotizing scleritis had macular edema or optic nerve
swelling. Most fundus findings were ascribed to patients
posterior scleritis (15 eyes), nine eyes had choroidal folds,
six had subretinal mass, seven had disc edema, and six
had macular edema. Annular ciliochoroidal detachments
were present in three eyes, two of which had bullous serous
retinal detachments. Intraretinal deposits were found in
three eyes. Retinal striae were detected in two eyes. There
were no episcleritis patients with fundus abnormalities.
Disease Associations
An associated disease was found in 98 patients (57%) with
scleritis (Table 12), including 82 patients (48%) with con-
nective tissue or vasculitic diseases, 12 (7%) with infectious
diseases, and 4 (2%) with miscellaneous diseases (rosacea,
atopy, gout, and a foreign body, 1 patient each). The de-
tection of an associated disease was strongly associated
with the type of scleritis (P = 0.0001): the most common
type of scleritis associated with a specific disease was nec-
rotizing scleritis (95%) (P = 0.0001) (Tables 3 and 12)
followed by scleromalacia perforans (67%), posterior
scleritis (45%), diffuse scleritis (45%), and nodular scleritis
Table 11. Cataract in Scleritis and Episcleritis

Diagnosis/Type Cataract (%) Total Chi-square

Scleritis 29 (16.86) 172
Table 9. Peripheral Ulcerative Keratitis in Scleritis Diffuse 7 (9.09) 77
Nodular 4 (10.26) 39
Diagnosis/Type PUK(%) Total Chi-square Necrotizing 16 (41.02) 39 0.0002
Scleritis 24 (13.95) 172 Scleromalacia· 1 (16.67) 6
Diffuse 5 (6.49) 77 Posterior 1 (9.09) 11
Nodular 3 (7.69) 39 Episcleritis 2 (2.13) 94
Necrotizing 16 (41.03) 39 0.0001 Simple 2 (2.56) 78 NSt
Scleromalacia" 0 6 Nodular 0 16
Posterior 0 11
NS = not significant.
PUK = peripheral ulcerative keratitis. • Not included in the chi-square study due to low number of patients.
• Not included in the chi-square study due to low number of patients. t P > 0.05 .

393
 Ophthalmology Volume 101, Number 2, February 1994
Table 12. Associated Disease in Scleritis
and Episcleritis
Associated
Diagnosis/Type Disease (%) Total Chi-square
Scleritis 98 (56.98) 172
Diffuse 35 (45.45) 77
Nodular 17 (43.59) 39
Necrotizing 37 (94.87) 39 0.0001
Scleromalacia· 4 (66.67) 6
Posterior 5 (45.45) 11
Episcleritis 30 (31.91) 94
Simple 22 (28.21) 78 NSt
Nodular 8 5( 0) 16
NS = not significant.
• Not included in the chi-square study due to low number of patients.
t p < 0.05.
(44%). Within the connective tissue and vasculitic diseases,
rheumatoid arthritis was the most common associated
disease (32 patients), followed by Wegener granulomatosis
(14 patients), relapsing polychondritis (11 patients), ar-
thritis and inflammatory bowel disease (7 patients), and
systemic lupus erythematosus (7 patients); other diseases
included Reiter syndrome (3 patients), psoriatic arthritis
(2 patients), polyarteritis nodosa (2 patients), ankylosing
spondylitis (I patient), Beh<;et disease (1 patient), giant
cell arteritis (I patient), and Cogan syndrome (l patient).
Bacteria (5 patients) were the most frequent microbes iso-
lated in the cases of infectious scleritis, followed by viruses
(4 patients), parasites (2 patients), and fungi (I patient).
Scleritis was the first manifestation of a systemic con-
nective tissue disease or vasculitic disease in 26 patients
(15% of patients with scleritis). Diagnoses were made 1
month to 22 years after the onset of scleritis in these pa-
tients (mean duration, 37 months). Wegener granulo-
matosis (14 patients) and relapsing polychondritis (6 pa-
tients) were the most frequent diseases discovered; other
diagnoses included rheumatoid arthritis (2 patients), Rei-
ter syndrome (I patient), polyarteritis nodosa (1 patient),
systemic lupus erythematosus (1 patient), and Beh<;et dis-
ease (1 patient). Necrotizing scleritis was found to be the
most frequent type in patients whose scleritis appeared as
a first manifestation of a systemic disease (15 patients).
An associated disease was found in 32% of the patients
with episcleritis (Table 12), including 12 patients (13%)
with connective tissue or vasculitic diseases (3 patients
with rheumatoid arthritis, 3 with arthritis and inflam-
matory bowel disease, 2 with psoriatic arthritis, 1 with
systemic lupus erythematosus, 1with Reiter syndrome,
1 with Beh<;et disease, and 1 with Cogan syndrome), 2
(2%) with infectious diseases (1 patient with herpes zoster
infection and 1 with herpes simplex infection), and 16
(17%) with miscellaneous diseases (7 patients with atopy,
7 with rosacea, 1with chemical injury, and 1 with gout).
Episcleritis was the first manifestation of a systemic
connective tissue disease or vasculitic disease in two pa-
394
tients (2% of patients with episcleritis); diagnoses included
systemic lupus erythematosus in one patient and Cogan
syndrome in another patient.
An associated disease was more commonly associated
with scleritis (57%) than with episcleritis (32%) (P
0.0001) (Tables 4 and 12).
Discussion
The results of this analysis provide insights into the se-
verity of scleritis and episcleritis and their respective types.
Objective data on the incidence of episcleritis and scleritis
are very difficult to obtain because these inflammatory
conditions are not extremely common and patient group
characteristics can vary greatly, depending on the insti-
tution at which the analyses are performed. Data from
highly selected patient tertiary referral centers, such as the
Massachusetts Eye and Ear Infirmary, will inevitably be
biased to higher numbers of patients with scleritis, com-
pared with patients with episcleritis, and to higher num-
bers of patients with more destructive types of scleritis,
compared with patients with more benign types of scleritis.
It is interesting to compare scleritis patient character-
istics between tertiary referral centers. The scleritis patient
population from our Immunology Service showed a higher
proportion of patients with necrotizing scleritis and a
lower proportion of patients with nodular scleritis than
those reported in two large retrospective studies performed
by Watson and Hayreh 7 in 1976, and by Tuft and Watson8
in 1991 , both at Moorfields Eye Hospital in London. Our
series of patients with scleritis showed a mean age (51.6
years) and a female-to-male ratio (1.57: 1) comparable with
the figures of51.5 years and 1.27: 1, respectively, reported
by Tuft and Watson. 8 Scleritis was bilateral in 34% of our
patients, which is between the figures of 45% reported by
Watson and Hayreh 7 and 25.5% reported by Tuft and
Watson. 8
Scleritis is a severe, destructive disease that may cause
decrease in vision by leading to one or more ocular com-
plications such as anterior uveitis, keratitis, glaucoma,
secondary cataract, or fundus changes. Visual acuity
should pe evaluated in every follow-up visit of a patient
with scleritis. In our series of patients with scleritis, 37%
had a decrease in vision equal or greater to two Snellen
lines at the end of the follow-up period, or a visual acuity
of 20/80 or worse at presentation; decrease in vision was
least common in nodular scleritis, intermediate in diffuse
scleritis, and most frequent in necrotizing scleritis followed
by posterior scleritis. The most common reasons for de-
crease in vision were anterior uveitis and/or peripheral
ulcerative keratitis. In Tuft and Watson's8 series of patients
with scleritis, decrease in vision (defined as a permanent
drop in Snellen acuity of 2 or more lines) was least com-
mon in diffuse scleritis, intermediate in nodular scleritis,
and most frequent in posterior scleritis followed by nec-
rotizing scleritis.
Anterior uveitis and glaucoma are caused by extension
of the scleral inflammation. The presence of anterior uve-
itis in scleritis, particularly when associated with glau-
 Sainz de la Maza et al . Severity of Scleritis and Episcleritis
coma, should be regarded as a grave sign. Fraunfelder and
Watson,9 in a series of 30 enucleated eyes with a primary
histologic diagnosis of scleritis, found that 68% had signs
of having had uveitis and 46% had signs of having had
glaucoma; scleritis with uveitis and glaucoma was the most
common combination of complications leading to enu-
cleation. Anterior uveal inflammation and intraocular
pressure should be evaluated in every follow-up visit of a
patient with scleritis. In our series of patients with scleritis,
42% had anterior uveitis (41 % of the eyes), the majority
of those with necrotizing scleritis. This is a higher figure
than the one reported by Watson and Hayreh (30% of the
eyes), probably because of our higher proportion of pa-
tients with necrotizing scleritis. Our proportion of scleritis
patients with glaucoma (13%) (13% of the eyes) is similar
to the proportions reported by Watson and Hayreh 7 (12%
of the eyes) and Tuft and Watson 8 (12% of the patients).
Anterior uveitis and glaucoma were present in 8% of our
patients, the majority of those with necrotizing scleritis.
The detection of anterior uveitis and/or glaucoma ac-
companying scleritis requires early and aggressive therapy
to control the scleral inflammation.
Peripheral ulcerative keratitis also is caused by exten-
sion of scleral inflammation. The presence of peripheral
ulcerative keratitis in scleritis also should be regarded as
a grave sign because the layers of the peripheral cornea
progressively are destroyed, leaving the cornea so thin it
can easily perforate. Corneal involvement should be eval-
uated in every follow-up visit of a patient with scleritis.
In our series, 14% of the patients with scleritis had pe-
ripheral ulcerative keratitis, and the majority ofthose had
necrotizing scleritis. The detection ofperipheraI ulcerative
keratitis accompanying scleritis requires early and ag-
gressive therapy to control the scleral inflammation.
Cataract formation in scleritis may be caused either
by longstanding anterior uveitis or by long-term systemic
or local steroid use, and is most common in the necro-
tizing type of scleritis. Cataract formation should be
evaluated in every follow-up visit of a patient with scleri-
tis. A comparison between a group of patients without
scleritis and a group of patients with scleritis, both re-
ceiving long-term systemic or local steroid treatment,
showed an increased risk of development of posterior
subcapsular cataract in the group of patients with scleritis
(36%) as opposed to the group of patients without scleritis
(11.5%). 5 Our proportion of patients with scleritis who
had cataract (17%) (16% of the eyes) is higher than the
proportions reported by Watson and Hayreh 7 (7% of the
eyes) and Tuft and Watson 8 (6% of the patients), probably
because of our higher percentage of patients with nec-
rotizing scleritis.
Fundus findings in scleritis are most often ascribed to
patients with posterior scleritis. Although posterior scleritis
often is associated with anterior scleritis, it also may occur
alone, in which case the absence of anterior scleral in-
volvement makes the diagnosis difficult. 10-12 In addition,
if the concomitant anterior scleritis is severe, posterior
scleritis may be overlooked. Fundus evaluation should be
performed in every follow-up visit of a patient with scleri-
tis. Early diagnosis leads to early therapy and prevention
of complications which could cause permanent loss of
vision.
Scleritis may also be a manifestation ofa potentially
life-threatening systemic autoimmune disease. I- 8 Delay
in diagnosis and initiation of appropriate therapy can re-
sult in vision loss, organ damage, and even death. In our
series of patients with scleritis, 57% had an associated dis-
ease; diffuse scleritis was present in 45%, nodular scleritis
in 44%, necrotizing scleritis in 95%, scleromalacia per-
forans in 67%, and posterior scleritis in 45%. Interestingly,
these proportions are much higher than those found by
Tuft and Watson. 8 In their series of patients with scleritis,
25% had an associated disease; diffuse scleritis was present
in 13%, nodular scleritis in 28%, necrotizing scleritis in
45%, and posterior scleritis in 19%. 8 In both tertiary re-
ferral center studies, rheumatoid arthritis was the most
common disease associated with scleritis, followed by
Wegener granulomatosis, relapsing poiychondritis, ar-
thritis and inflammatory bowel disease, and systemic lu-
pus erythematosus. Medical and family history, meticu-
lous review of systems, and a standard physical exami-
nation including not only the eye but also the ears, nose,
mouth, skin, and joints is essential in patients with scleri-
tis, even at their first episode. Specific systemic manifes-
tations may lead the ophthalmologist to suspect certain
types of autoimmune diseases. Subsequent laboratory, x-
rays, or pathologic studies designed on the basis of infor-
mation obtained from a careful review of systems ("tar-
geted workups") help to confirm the initial diagnostic
impressions. It must be emphasized that because systemic
autoimmune diseases sometimes evolve slowly, one series
of investigations may be insufficient, and sequential di-
agnostic studies may be necessary to reach a diagnosis.
Each of these steps in this approach to patients with scle-
ritis has been reviewed in detail elsewhere. 13
Episcleritis, unlike scleritis, is a benign disorder that
rarely causes decrease in vision, because the associated
complications such as anterior uveitis, keratitis, glaucoma,
or secondary cataract are uncommon and are never se-
vere. 5,7 In our series of patients with episcleritis, only 2%
had decrease in visual acuity equal to or greater than two
Snellen lines at the end of the follow-up period, or a visual
acuity of 20/80 or worse at presentation. This decrease
in vision was attributed to the natural development of
cataract in all of the patients. Mild peripheral corneal
changes such as superficial and midstromal inflammatory
cell infiltration can be observed occasionally in patients
with episcleritis in the area adjacent to the conjunctival
and episcleral edema but these infiltrates never progress
to peripheral ulcerative keratitis. They rarely are perma-
nent unless the attacks are recurrent in the same area.
Intraocular structures are almost never involved. In a few
cases, cells in the anterior chamber and aqueous flare may
appear, but these are never severe. Glaucoma or cataract
are not directly attributed to the episcleral inflammation
unless they are induced by steroid treatment. 5 ,7 Some pa-
tients with episcleritis may have an associated disease but
the majority defies all attempts to discover a cause. 6- 8 A
"targeted workup" designed on the basis of information
obtained from a careful review of systems and from a
395
 Ophthalmology Volume 101, Number 2, February 1994
meticulous examination of the patient is appropriate in
cases of persistent or recurrent episcleritis attacks. 13 Simple
episcleritis does not differ significantly from nodular epi-
scleritis with respect to ocular complications and systemic
disease associations.
Diagnosis and classification of inflammatory diseases
of the wall of the eyeball provide valuable prognostic in-
formation that may be used as a guide for therapeutic
decisions.
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