Professional Documents
Culture Documents
Regions
The uvea lies between the corneosclera (outermost layer of the eye) and the
retina (innermost layer/in the back of the eye). It is traditionally divided into 3
or 4 regions, the iris, ciliary body, pars plana and choroid. These
distinctions are based on their different structures as seen under light
microscopy, and continued use of these terms is appropriate in anatomical
studies. For clinical use, the terms anterior uvea (ie, iris and ciliary body) and
posterior uvea (ie, choroid) are now in common use, since diseases often
spread beyond a single anatomical region of the uvea.
Histology
1
What is uveitis
Uveitis is a general term denoting intraocular inflammation that either primarily
or secondarily involves one (or more) components of the uveal tract. Although
components of the uveal tract are comprised of the iris, ciliary body, and
choroid, all layers of the eye may be involved by inflammation. Because the
uveal tract is supplied by the systemic circulation, systemic diseases and local
diseases that affect blood vessels can involve the uvea as well as contiguous
ocular tissues and structures.
CLASSIFICATION
Importance Of Classification
The most widely used classification of uveitis was devised by the International
Uveitis Study Group (IUSG) in 1987 based on the anatomical location of the
inflammation.
Classification parameters
1. Patient demographics
2. Location of the inflammatory process (anatomical)
3. Duration, onset, and course of inflammation
2
4. Clinicopathological classification
5. Etiology of the inflammation
The intellectual exercise of naming each parameter allows the clinician to focus
upon those diagnoses most likely attributed to each patient's disease. Thus, a
specifically tailored, cost-effective uveitis evaluation may be obtained to further
process the differential for a given constellation of parameters.
1. Patient demographic
Age
The age of the patient often is helpful in focusing the differential diagnosis.
Sex
Race
The race of the patient may focus the differential even further.
Sexual habits
Sexual orientation may play a role in diagnosis because young, sexually active
males are more likely to contract HIV and other AIDS-related infections, such as
cytomegalovirus (CMV) retinitis, Kaposi sarcoma, or herpes zoster
ophthalmicus. Sexually active individuals are also at increased risk of
contracting a sexually transmitted disease, such as syphilis or gonorrhea, which
may cause a primary uveitis, or chlamydia, which itself may trigger a HLA-B27-
related uveitis.
Geographic history
Family history
Social habits
Travel history
Travel and immigration are important components of the uveitis history. Past
exposure to tuberculosis or dysentery may be directly related to a recent or
recurrent ocular inflammatory episode. Exposure to malaria, yellow fever,
typhoid fever, hepatitis A, B, and C, as well as trachoma, leprosy, or
onchocerciasis must be considered in rare cases. The local immigration and
health departments may provide critical information regarding endemic
diseases in specific travel and immigration destinations.
Occupational exposure
Occupational and hobby exposure may provide useful clues to the determined
diagnostician, particularly in the farming or animal husbandry environment.
Brucellosis may occur in livestock workers and slaughterhouse workers, while
some cases of brucellosis are caused by consumption of unpasteurized milk
products. Leptospirosis previously was seen in miners or sewer workers;
however, most cases today follow exposure to water contaminated by domestic
animals carrying the bacteria. Chlamydia psittaci infection results from parrot
exposure. Cat scratch disease caused by Bartonella henselae infection and
toxoplasmosis can follow feline exposure.
Anterior uveitis
Intermediate uveitis
5
Middle uveitis, cyclitis, intermediate uveitis, pars planitis, peripheral uveitis, or
chronic cyclitis often indicates a more severe form of ocular inflammation than
isolated anterior disease.
Posterior uveitis
Panuveitis (diffuse)
6
3. Clinicopathologic classification
I) Suppurative
II) Exudative
III) Granulomatous
8
4. Etiology of the inflammatory process
When diagnostic testing has been performed, the specific etiology of the
underlying process may be identified. Sometimes, only the category, but not
the actual cause of inflammation may be known. This knowledge alone may be
sufficient to initiate appropriate therapy. For instance, when history rules out
trauma, age rules out ischemia, and comprehensive serologies rule out
infection in the presence of arthritis, systemic anti-inflammatory therapy may
begin despite the absence of a specific diagnostic syndrome.
9
Clinical Picture and complication Different Types Of
Uveitis:
The most commonly used parameter of classification is the anatomical
classification.
Thus each part of the uveal tract ( iris, ciliary bdy, choroid ) may be
inflamed.
So there is iritis, cyclitis, and choroiditis.
Each may be ( exogenous, secondary to eye lesion, or endogenous
according to the source of inflammation)
And may be ( suppuritive, exudative or granulomatous ) according to the
pathology of disease.
And may be ( localized or diffuse ) according to the clinical state of the
disease
Symptoms
Signs
2. ciliary injection
10
5. Iris :muddy iris
Lost iris pattern ( as iris furrows and crypts are ffilled with fibrinous or purulent
exudates
6. Koeppe nodules are found at the pupillary border. Busacca nodules are located
on the surface of the iris
7. Hypopyon { in severe cases}
10.Pupillary Miosis
Prognosis
Complication
3. Iris Atrophy
11
5. Complicated Cataract : complicated anterior cortical catarct in relation
to synechia or complicated posterior cortical catarct
Cyclitis
o It is rarely acute but usually chronic insidious
Symptoms
Signs
12
4. A.C : appear deep when retinal detachment occur as acomplication
Complication
Iridocyclitis
Intermediate uveitis
Pars planitis
o Bilateral ocular inflammatory disease
Symptoms
Signs
Complications
14
3. Retinal Neovascularization { as a result of ischemia } causing
vitreous hemorrhages and vitreous fibrosis can cause traction on the
peripheral retina and lead to retinal detachment
granulomatous
Syphilis
Behçet disease
Histoplasmosis
Toxocara
Symptoms. Patients are free of pain, although they report blurred vision and
floaters.
15
Signs: Ophthalmoscopy reveals isolated or multiple choroiditis foci. In
acute disease they appear as ill-defined white dots. Once scarring has
occurred the foci are sharply demarcated with a yellowish-brown color.
Occasionally the major choroidal vessels will be visible through the
atrophic scars. No cells will be found in the vitreous body in a primary
choroidal process.
However, inflammation proceeding from the retina (retinochoroiditis) will
exhibit cellular infiltration of the vitreous body.
Complication.
Prognosis.The inflammatory foci will heal within 2–6 weeks and form
chorioretinal scars. The scars will result in localized scotomas that will
reduce visual acuity if the macula is affected.
Now we are going to discuss choroiditis according to its pathological picture
( suppuritive, exudative and granulomatous)
Suppuritive choroiditis
Exudative choroiditis
16
1. Diffuse involving a large area of choroid. It is usually due to syphilis or
tuberculosis. The lesion is greyor yellow, and the overlying retina is
edematous. The lesion heal with a white scar. Pigment hypertrophy
within the lesion may be extensive and give apicture similar to retinitis
pigmentosa but pigmentations is irregular and in clumps. Ring scotoma
and night blindness are characteristic in both lesions.
Granulomatous choroiditis
o Caseaton may occur ending with destruction of the eye and phthisis
bulbi
Diffuse uveitis
Endophthalmitis
Definition: This refers to acute or chronic intraocular inflammation of Microbial
or immunologic causes .In the strict sense, any intraocular inflammation is
endophthalmitis. However, in clinical usage endophthalmitis refer to diffuse
suppuritive choroiditis with a secondary abcess in the vitrous, and suppuritive
retinitis.
Symptoms.
Signs
1. Amauritic cat's eye reflex
Complications
1. Panophthalmitis
2. Pseudo glioma
3. Atrophia bulbi
18
4. Act as aseptic focus to the other eye.
Exogenous Endogenous
Panophthalmitis
Definition it is diffuse suppuritive inflammation of all coats of the eye with
Tenon's capsule.it is dramatic acute inflammation that usually ends with
destruction of the eye.
Etiology :
a) Exogenous : after operation penetrating injuries, corneal ulcers, or an
infected trephining operation. Rarely an iris inclusion
b) Endogenous ( metastatic ) : some cases of panophthalmitis are
metastatic infections specialy in diabetes or during fevers such as
pneumonia, puerperal fever, or meningitis
c) Secondary ( as acomplication of ) to cavernous sinus thrombosis or
orbital cellulitis.
Symptoms
There are severe pain in the eye, fever, general malaise, headache, swelling of
both lids bulging of eye, congestion of the conjunctiva and loss of vision.
Signs
1. Proptosis and limitation of eye movement (D.D from endophthalmitis).
2. The lids are edematous and tender
3. Conj and ciliary injection with chemosis of conjunctiva which may bulge
as chemotic folds through the palpebral fissure.
4. The cornea, in exogenous cases shows a suppurating wound or a
perforated ulcer and in the endogenous group shows a ring abcess.
Finally, in both groups the cornea slouphs out with complete necrosis.
5. Total hypopyon masks a purulent iritis
6. Vision is lost
Complication
20
5. Metastatic pyamic complication
d. Complicated cataract.
Clinical picture. Floating spots and loss of accommodation are among the
earliest symptoms. The disease may progress to severe iridocyclitis with pain
and photophobia. Commonly the eye remains relatively painless while the
inflammatory disease spreads through the uvea. The retina, however, usually
remains uninvolved, although perivascular cuffing of the retinal vessels with
inflammatory cells may occur. Papilledema, secondary glaucoma, vitiligo, and
poliosis of the eyelashes may accompany.
Complications :-
1. iridocyclitis
2. Papilledema.
3. secondary glaucoma.
4. vitiligo, and poliosis
5. Optic nerve injury
Differential diagnosis. The disorder should be distinguished from
iridocyclitis and choroiditis from other causes.
1. Acute serous detachment of the retina is the hallmark of the VKH syndrome (Fig. 8.41)
and may be accompanied by multiple areas of yellowish or yellowish-white edema of the
retinal pigment epithelium.
2. retinal vasculitis
3. swelling of the optic disc.
4. Associated anterior iritis may be severe and may be either granulomatous or
nongranulomatous in appearance.
22
5. Some patients will experience associated headache and stiffness of the neck or other
neurologic symptoms such as tinnitus.
6. With time, many patients will manifest associated dermatologic changes including poliosis
(whitening of the lashes) and vitiligo (patchy depigmentation of the skin). Neither the
vitiligo nor poliosis is generally present at the outset of the disease.
Pathophysiology :-
-The etiologic and pathogenic factors in VKH syndrome remain unclear. The clinical course of
VKH syndrome with an influenza like episode suggests a viral or post-infectious origin. Some
studies invoke a possible role of Epstein-Barr virus reactivation in this disease. Although a viral
cause has been proposed, no virus has been isolated or cultured from patients with VKH syndrome.
Morris and Schlaegel found virus like inclusion bodies in the sub-retinal fluid of a patient with
VKH syndrome.
-Clinical and experimental data continue to support an immunologic etiology. An autoimmune
reaction seems to be directed against an antigenic component shared by uveal, dermal, and
meningeal melanocytes.
BEHECET'S DISEASE
Definition Behecet's disease is a form of diffuse bilateral uveitis of unknown cause that is
associated with significant dermatologic and vascular features. It is found in young adults, and
although
rare in the United States, it is the most common form of uveitis in Japan. The ophthalmologist may
be the first to recognize the condition because of the unusual presentation and clinical findings.
Clinical Features
o Major features
Recurrent aphthous ulceration of the oral mucous membrane
Skin lesions - Erythema nodosum–like lesions, subcutaneous
thrombophlebitis, folliculitis (acnelike lesions), cutaneous hypersensitivity
Eye lesions - Iridocyclitis, chorioretinitis, retinouveitis, definite history of
chorioretinitis or retinouveitis
Genital ulcers
o Minor features
Arthritis without deformity and ankylosis
Gastrointestinal lesions characterized by ileocecal ulcers
Epididymitis
Vascular lesions
Central nervous system symptoms
o Diagnosis
Complete - Four major features
Incomplete - (1) 3 major features, (2) 2 major and 2 minor features, or (3)
typical ocular symptom and 1 major or 2 minor features
Possible - (1) 2 major features or (2) 1 major and 2 minor features
Systemic diseases has a broad wide effect on the eye and the uvea because the uvea is supplied by
the systemic diseases. Systemic diseases mostly cause diffuse uveitis, with the consideration that
organisms show variation in the preferable site.
A. Bacterial
B. Parasitic
C. viral
Non infective
I) Sarcoidosis
24
A. Bacterial Systemic
I) Tuberculosis
Tuberculosis is a chronic disease caused by Mycobacterium tuberculosis. Any organ system,
including the eye, can be affected. Ocular tuberculosis is rare, although it is becoming more
common with the advent of AIDS and the use of immunosuppressive agents in the treatment of
other diseases.
Clinical Features
o A posterior granulomatous choroiditis is the most common finding in ocular tuberculosis
(Fig. 8.47).
o Retinal vasculitis in the form of sheathing of the veins and arteritis is common.
o Blurred vision is usually the presenting symptom and is generally related to vitritis.
o Chronic iridocyclitis may also occur, although it may be solely immunologic in nature,
without active organisms.
o In overwhelming systemic infections, tuberculous endophthalmitis has been reported.
As with many of these rare conditions, a high index of suspicion is necessary for the initial
diagnosis. Skin testing and a chest radiograph are indicated as part of the ophthalmologist's workup,
but an internal medical consultation also is indicated before determining the diagnosis and
treatment.
II) Syphilis
Ocular syphilis may occur as both an acquired and a congenital form of disease, and can confuse the
uveitis diagnosis. In acquired syphilis, eye involvement can occur in the secondary, latent, and
tertiary phases of the disease. Untreated acquired syphilis and congenital syphilis are associated
with multisystem abnormalities.
Clinical Features
a) Acquired syphilis
o may be acute or chronic in nature, and are often
o granulomatous in character.
o Any portion of the ocular system may be involved.
o Some characteristic findings of acute ocular infection are
1. anterior uveitis,
2. diffuse and focal chorioretinitis,
3. retinal vasculitis
4. optic neuritis (Fig. 8.45).
5. Late findings include interstitial keratitis, chorioretinal scars, (salt-and-pepper)
retinal pigment epithelial scarring, and optic atrophy.
6. The Argyll-Robertson pupil, which fails to react to light but preserves
accommodation, classically has been associated with syphilis. .
b) Congenital syphlis
o Ocular findings associated with congenital syphilis may include
1. anterior uveitis
2. chorioretinitis : is usually seen in the healed stage and pigmentation may take one or
more of the following pictures ::
25
a) salt and pepper fundus where pigmented spots lie on ayellowish bachground
and usually the lesion is peripheral.
b) Peripheral isolated pigmented spots.
c) Coarsely pigmented peripheral areas
d) Atrophic white patchs
3. interstitial keratitis
4. optic atrophy.
III)Gonorrreal uveitis
IV)Brucellosis
V) leprosy
B. Parasitic systemic
diseases
I) Toxoplasmosis
Causative organism : Toxoplasma gondii
Signs :
2. focal retinitis with overlying vitrous haze ( active lesion ) severe vitritis
causes " headlight in the fog " appearance , where the inflammatory
focus is difficultly seen through the hazy vitrous
3. 3-Papillitis
Man is the definitive host and simulium fly is the intermediate host.
Ocular lesions
4. Interstitial keratitis
6. A grey brown mass of dead larva in te angle of the anterior chamber with iris atrophy
9. Posterior synechia
III)Loasiasis
Loa loa (African eye worm), rarely affects the eye. The adult worm can move under the skin or the
conjunctiva, in the anterior chamber, in the vitrous or protruding through a macular hole
IV)Toxocariasis
Causative organism : Toxocara canis .
Forms :
27
1. Chronic endophthalmitis
3. Peripheral granuloma
V)cysticercosis
Tainea solium rarely causes intraocular cysts. These cysts are commonly subretinal with detachment
2-signs
• Corneal sensation may be reduced and nummular keratits may be
present.
29
5- Treatment with topical steroids needs to be continued for several months
and then tapered very gradually. Some patients may require very low dose
treatment indfinititely in order to maintain long term control of inflammation.
I) Sarcoidosis
Characters of the disease
o Sarcoidosis is a multisystem systemic disease of unknown cause in which lymphoid tissue
develops noncaseating, granulomatous inflammation.
o Usually, sarcoidosis is a disease that affects 20- to 40-year-old individuals, with persons of
African descent and women more predisposed than other patient subgroups.
o The lungs are the most frequently involved organs, but the skin, liver, spleen, joints, and
heart may also be involved.
o Ocular involvement occurs in 17% of cases of systemic involvement. Ocular sarcoidosis can
take many forms, but it is often the presenting sign of the systemic disease.
Clinical Features
1. Acute iridocyclitis may be the earliest form of sarcoid uveitis.
2. Patients complain of ocular pain, photophobia, and redness,
3. flare and cells are seen in the anterior chamber.
4. The uveitis may be granulomatous or nongranulomatous.
5. Fine, gray KPs initially may be seen on the posterior surface of the cornea.
6. The condition often becomes chronic, and mutton-fat KPs develop as the disease process
continues.
7. The granulomatous nature of sarcoidosis is responsible for some of the ocular features,
including conjunctival granulomas, mutton-fat KPs, Busacca nodules within the iris
stroma, and Koeppe nodules along the pupil margin (Fig. 8.33).
8. Posterior segment findings in ocular sarcoidosis may include vitritis, pars plana
snowbanking, cyclitis, choroiditis, optic neuritis, cystoid macular edema, retinal arteritis,
retinal phlebitis, and optic nerve granulomas (Figs. 8.34; 8.35A, B). All or none of these
may be found. White, fluffy opacities may be seen in the inferior vitreous, and yellow-white
exudates, called candle-wax drippings, and sheathing of the peripheral retinal veins may be
seen (Fig. 8.36).
The characteristic granulomatous anterior, posterior, or diffuse uveitis should alert the clinician to
search for systemic involvement. The workup should include a chest x-ray, serum angiotensin-
converting enzyme assay, and if necessary, a gallium scan limited to the head, neck, and
mediastinum. Biopsy of ocular tissues such as conjunctival granulomas (Fig. 8.37) or lacrimal gland
granulomas may be helpful in confirming the diagnosis
Gouty (uratic) iritis is usually preceded by episcleritis or scleritis. The attack is usually
asscociated with acute rise of tension. The un complicated attack of iridocyclitis usually
30
resolves in a week's time with only pigment dispersion on the anterior lens capsule. Urates are
not deposited in the uveal tract and inflammation is recurrent.
2. Diabetes mellitus
Non inflammatory changes in uveal tract are more common in diabetes mellitus. The
pigment epithelium of the iris shows edema, glycogen infliteration and finally ruputure.
The muscles of the iris are infiltrated with glycogen and the papillary reaction are
affected. Rubeosis iridis, with recurrent hyphema and haemorrhagic glaucoma are seen
in advanced cases.
True diabetic iritis is rare. There are albuminous exudates that fill the pupil,
neovascularization around the pupil that disappear with subsidence of the attak, and
pigmented posterior synechiae. The papillary response to mydriatic is poor.
III)Uveitis in spondyloarthropathies
A. Ankylosing spondylitis
Ankylosing spondylitis (AS) primarily involves the sacroiliac joints and axial
skeleton. About 90% of patients are positive for HLA-B27 and some have
associated inflammatory bowel disease (enteropathic arthritis)
Acute anterior uveitis occur in 30% of patients with AS, conversely 30% of
males with acute iritis will have AS. Although both eyes are rarely involved
simultaneously either eye frequently affected at different times. In severe
uveitis there may be fibrinous anterior chamber exudates. There is no
correlation between the severity and activity of eye and joint involvement.
Despite the high rate of recurrence the long-term visual prognosis is good and
vision-threatening complications rare. In few patients with many recurrent the
inflammation may eventually become chronic.
B. Retire syndrome
Retire syndrome is defined as an episode of peripheral arthritis of more than 1
month's duration occurring in association with urethritis or cervicitis or both.
About 70% of patients are positive for HLA-B27 and 60% have associated
sacroiliitis.
31
7-10 days and does not require treatment. Cultures for bacteria are
usually negative.
Clinical features
The uveitis is chronic non granulomatous, and bilateral in 70% of cases. It is
unusual for unilateral uveitis to become bilateral after more than a year.
When bilateral, the severity of inflammation is usually symmetrical.
2. Signs
• Uninjected eye even in the presence of severe uveitis.
32
• During acute exacerbations the entire endothelium shows dusting
but hypyon does not develop.
3. Prognosis
• In about 10% of cases the uveitis is mild with never more than +1
aqueous cells, and persists for less than 12 months.
• About 15% of patients have one attack , which lasts less than 4
months, the severity of inflammation varying from +2 to +4
aqueous cells.
• In 25% of cases the uveitis is very severe, lasts for several years
and responds poorly to treatment. In this subgroup band
keratopathy (Fig 10.23) occurs in 40$ of patients, cataract in 30%
(Fig 10.24) and secondary inflammatory glaucoma in 15%.
MANAGEMENT
Lines of treatment:-
A-Accurate diagnosis
B-Non specific treatment
C-specific treatment
D-treatment of complication
A-Accurate diagnosis: by both etiological and clinical methods this needs
the following:
a- Proper examination of the eye to exclude other causes for red eye
33
- Eye: - we do paracentesis of anterior chamber to take a specimen of
aqueous to be examined by histological, biochemical, biological,
bacteriological and immunological methods.
- Systemic investigations by :-
i- Skin test :
Anti-streptococcal tests
I- Local treatment
I-Local treatment
Advantage:
34
2-Dark glasses
3-Hot fomentations
II-General treatment
4-Anti-bacterial drugs
6-Anti-histaminics
4- Iridocyclitis:
36
5- fuch's Heterochromic cyclitis: Atropine and cortisone are of no value.
Glaucoma is treated medically if this fails, an externally fistulizing operation is
done.
7-Endophthalmitis:
- Conservative treatment is tried first.
- We give general anti-bacterial drugs.
- Locally we give atropine and anti bacterial drugs by every possible route such
as sub-conjunctival or intra-vitreal injections of penicillin or vancomycin
-If medical treatment failed and the eye is irritade we do enucleation of the
eyeball
8-Panophthalmitis:
- Medical: as endophthalmitis but usually without effect
- Surgical: we do evisceration to evacuate the intra-ocular purulent
content. Under general anaesthesia we remove the cornea. Then curette
all the intra-ocular contents with a scoop. The inside of sclera is washed
37
Treatment of this immunologic condition includes the use of systemic
corticosteroids and sometimes includes immunosuppressive agents such as
chlorambucil or methotrexate. Cyclosporine may also be used in the treatment
of this condition. The final outcome depends on the control of inflammation and
prevention of scar formation. Visual function may remain quite good, although
the course may be long term and indolent (Fig. 8.42).
D- Treatment of complication:
1- Secondary glaucoma: continue atropine. Give diamox and osmotherapy.
Do paracentesis if necessary.
2- Occlusio and seclusio pupillae: after the eye becomes quite and
iridectomy is necessary both for secondary glaucoma and for vision.
References
Classification
• e.medicine classification
39
• DR. ABDEL-BASSET ELNAGGAR. Diseases of uveal tract in his book (THE
EYE AND ITS DISEASES). Second edition 1976. TANTA UNIVERSITY.
• Bloch-Michel E, Nussenblatt RB: International Uveitis Study Group
recommendations for the evaluation of intraocular inflammatory disease.
Am J Ophthalmol 1987 Feb 15; 103(2): 234-5[Medline].
• Gritz DC, Wong IG: Incidence and prevalence of uveitis in Northern
California; the Northern California Epidemiology of Uveitis Study.
Ophthalmology 2004 Mar; 111(3): 491-500; discussion 500[Medline].
• Jabs DA, Nussenblatt RB, Rosenbaum JT: Standardization of uveitis
nomenclature for reporting clinical data. Results of the First International
Workshop. Am J Ophthalmol 2005 Sep; 140(3): 509-16[Medline].
• Karcioglu ZA, Brear R: Conjunctival biopsy in sarcoidosis. Am J Ophthalmol
1985 Jan 15; 99(1): 68-73[Medline].
• Sheppard JD: Posterior uveitis. Ophthalmol Clin North Am 1993; 6: 1.
• Sheppard JD, Garovoy MR: The major histocompatibility complex in
ophthalmology. In: Friedlander MH, ed. Duane's Basic Ophthalmologic
Science. Vol. 1. 1991.
• Sheppard JD, Nozik RA: Practical diagnostic approach to uveitis. In: Duane
JA, Jaeger EW, ed. Duane's Clinical Ophthalmology. Vol. 4. 1989.
• Smith RE, Nozik RA: Uveitis: A Clinical Approach to Diagnosis and
Management. 2nd ed. Baltimore: Williams & Wilkins; 1988.
Management
40