Uveitis

Introduction Uveitis : inflammation of uveal tract (iris, ciliay body & choroid)

It is classified anatomically to1. Ant.uveitis (iritis) inflammation of iris accompanied by increased vascular permeability which

allow both protein & WBCs to extravasate into the aqueous. 2 types : granulomatous (Sarcoidosis,Syphilis, Vogt-Koyanagi-Harada

disease Sympathetic ophthalmi Multiple sclerosis Lyme disease Tuberculosis.) nongranulomatous.

circulating White cells could be seen in the aqueous humor of Ant. Chamber using a slit lamp.

Protein which leaked from BV is picked out by its light scattering properties appear as “flare” in the beam of slit lamp.

Anterior Uveitis with hypopyon in a patient with IBD

2. Intermediate uveitis Inflammation of ciliary body (cyclitis),of the

pars plana (pars planitis) and of the vitreous (vitritis)

3. Posterior uveitis Inflammation of posterior uvea May also

involve the choroid(choroiditis) or retina(retinitis)

Panuveitis :its when inflammatory changes involve the anterior chamber,vitreous,retina and/or choroid.

Epidemiology

Incidence 15/100000 (75% ant.uveitis)

8/100000 in US 50% of pts have ass. Systemic dis.

Hx.

pt come to clinic complaining of:o ocular pain (less frequent with post.U) Abrupt onset dull aching eye pain worsen when touch the eye through

eye lid, may refered to temple or periorbital region

o Photophobiao Blurring of vision Redness of the eye

HISTORYo Respiratory symptoms (S.O.B , cough , nature of sputum)

……..TB, Sarcoidosis.o Skin problems acompained by uveitis erythema nodosum (arms +chin) >>>> Sarcoid & Bechet oral & genital ulcers >> Becheto Joint disease AS……….20% ant.uveitis juvenule chronic arthrits Reiter syndrome………..40% ant.uveitiso Bowel dis : IBD

Signs visual acuity may reduced ciliary injection:mostly around limbus Anterior uveitis: Keratitic precipitates inflam. Cells maybe visible as clumps on

endothelial of cornea (mostly Inferior) On slit lamp exam. flare & hypopyon in severe inflam. Hypopyon highly suggestive for HLAB27 related & less

commonly with infectious iritis.

Dilated vessels on iris Posterior synechia……….iris adhere to lens Elevated IOP(inc aqeous protein or due to

occlusion of drainage angle by PAS)

Intermediate and posterior uveitis Cells in the vitreous Retinal or choroidal foci of inflammation Macular edema

Grading of flare(flare refers to liberated protein from the inflamed iris or ciliary body which gives the aqueous a particulate, or smoky, appearance.)

Gading of aqueous cells

trace……………… 1-5 cells +1 …………………. 6-15 +2 …………………. 16-25 +3 ……………………. 26-50 +4 …………………….. >50

0Completely absent

+1Barely present

+2moderate

+3marked

+4Intense (fibrin)

Signs

KP: fibrous deposits on the posterior surface of the cornea, usually associated with uveitis.

White, yellowish greasy precipitates of inflammatory cells Typically distributed in a wedge-shaped region on the inferior corneal endothelium, known as Arlt's triangle

Both the size and distribution of keratic precipitates are helpful in the differential diagnosis.

Signs

Signs

annular s. — adhesion of the whole rim of the iris to the lens.anterior s. — adhesion of the iris to the cornea.peripheral anterior s. — adhesion of the peripheral iris to the cornea.posterior s. — adhesion of the iris to the capsule of the lens or to the surface of the vitreous body.total s. — adhesion of the whole surface of the iris to the lens.

Signs

Signs

Invetigations Aim : determine systemic association Investigation guided by past medical Hx &

review of system:1. Pt with first episode of nongranulomatus iritis with unremarkable

past medical Hx & review of system…so investigations not indicated

2. Recurrent persistant iritis with unusual severity, unresponsive to medical therapy or bilateral……….so here investigation is needed

* At minmum CXR , RPR (rapid plasma reagin) test & fluorescent treponemal antibody absorption (FTA-ABS FTA-ABS) should be ordered

ACE test , lysozyme level & ESR for evaluating sarcoidosis

HLA B27 typing

RF & ANA if we suspect juvenile idiopathic arthritis

Imaging studies

1. CXR to rule out sarcoidosis & TB

2. chest CT done if we have –ve CXR but

sarcoidosis still highly suspected

3. Sacroiliac radiograph , if AS is suspected Procedures

if patient have secluded pupil from extensive post. Synechia , iris bombe with closed angle glucoma ……..here iridotomy maybe needed

Etiology

 Individual forms of uveitis may be distinguished on the basis of location within the eye, onset, symmetry and continuity of inflammation, associated complications, and distribution of cells along the corneal endothelium.

Etiology

Infections Systemic immune-mediated disease (40%) Syndromes confined primarily to the eye Masquerade syndromes 30% of patients don’t fit an well-defined

disease category .

Infections Cytomegalovirus (CMV) in adults is found almost exclusively in the

immunocompromised host, especially patients with HIV infection who have extremely low CD4 counts

Toxoplasmosis is a surprisingly common cause of uveitis in the normal host. In many instances, it is presumed to be a reactivation of a congenitally acquired infection. It is suspected on the basis of a typical chorioretinal lesion; the diagnosis is supported by serology. Most chorioretinal scarring from toxoplasmosis is due to infection during gestation, but scarring is increasingly recognized as a result of recent infection . Patients may complain of hazy vision and floaters.retina is the target choroiditis is secondary.

Syphilis accounts for less than 1 percent of patients with uveitis in most large series. It may present in a variety of forms, including posterior uveitis, such as a chorioretinitis or retinal vasculitis

CMV retinitis(cottage cheese lesion)

Toxoplasmosis

Infections Tuberculosis is an uncommon cause of uveitis in North American

referral centers. It should be considered in the differential diagnosis when the uveitis worsens despite glucocorticoid therapy. Additional factors that raise suspicion for this diagnosis are active tuberculosis elsewhere in the body, cachexia, homelessness, a granulomatous appearance for the ocular inflammation, or immunosuppression. In some geographic areas such as Saudi Arabia, tuberculosis is considered a common cause of uveitis.

Both herpes simplex and herpes zoster can cause a keratouveitis, an inflammation of the cornea along with uveitis that is primarily anterior. The presence of cutaneous vesicles, characteristic corneal changes, reduced corneal sensation, elevated intraocular pressure, and iris atrophy may be clues to the diagnosis. Both herpes simplex and zoster can also cause a retinitis known as acute retinal necrosis. This is a rare but treatable cause of visual loss.

Iris atrophy in a patient with herpes simplex virus–associated anterior uveitis.

Immune-mediated systemic disorders

Spondyloarthritides (SpA), such as ankylosing spondylitis and reactive arthritis, are the most common systemic immune disorders associated with uveitis in North America and Europe

20-40 %

Male >female

typically unilateral, and tends to resolve within three months of its onset. Recurrences are common, and can occur in the contralateral eye.

The prognosis for this form of uveitis is generally excellent

provided that acute attacks are treated early and vigorousely

AS

Fibrin clot and posterior synechiae in a patient with acute, anterior uveitis and ankylosing spondylitis.

Immune-mediated systemic disorders 7% psoriatic arthritis and 2 to 9 % of patients with IBD may develop uveitis.

uveitis associated with IBD or psoriatic arthritis is frequently bilateral, posterior to the lens, insidious in onset, chronic in duration, and more common in females than males .

In 10 of 17 patients in one series, the uveitis preceded signs of IBD

Sarcoidosis accounts for a significant percentage of patients with uveitis in most series from referral centers .

Approximately 20 % develop eye disease as their initial presentation of sarcoid. The eye disease can take on many forms, including uveitis, dry eyes, optic

neuritis, lid inflammation, or orbital disease . Uveitis in patients with sarcoid is frequently associated with retinal vasculitis,

which may be either perivascular or involve retinal vascular changes The eye disease may persist despite the resolution of adenopathy [ (

Immune-mediated systemic disorders as many as 80 percent of patients with Behcet's disease develop uveitis . Uveitis is often the dominant manifestation of this disease, and is typically bilateral. Similar

to HLA-B27-associated iritis, it is frequently episodic, but unlike HLA-B27-associated disease, the uveitis generally does not resolve completely between episodes. Retinal vasculitis is a frequent manifestation of Behcet's disease .

The uveitis typically leads to blindness if the eye inflammation is not treated

Juvenile idiopathic arthritis (JIA) may be associated with uveitis, particularly in the subset of patients with pauciarticular disease and a positive antinuclear antibody. The onset of uveitis is more common in younger children and, in the majority of patients, can be asymptomatic. (white eye but wuth signs of uveitis present ) The uveitis associated with JIA is usually bilateral, insidious in onset, chronic in duration, and anterior. The eye disease is commonly associated with complications such as band keratopathy, posterior synechiae , cataract formation, and glaucoma. The uveitis sometimes lasts for decades, long after the joint disease has disappeared.

Behcet

Male,young,bilateral , hypopyon

Immune-mediated systemic disorders

(SLE) can involve the eye in a variety of forms .Dryness is the most common ocular manifestation; cotton wool spots) occur in about 7 percent of patients and indicate local retinal ischemia .

Anterior uveitis is a rare manifestation.'.) Uveitis is infrequently associated with other

systemic vasculitides, including polyarteritis and granulomatosis with polyangiitis (Wegener’s). More typical vision-threatening manifestations of granulomatosis with polyangiitis (Wegener’s) are scleritis and orbital disease

Vogt-Koyanagi-Harada is the second leading cause of uveitis in Japan after Behçet’s

syndrome Multisystem disorder Bilateral panuveitis with exudative retinal detachment followed by

neurological and cutananeous manifestations (baldness and loss of lash and skin pigment)

F>M 20-50s Prodromal stage(fever,headache,tinnitus and CSF

pleocytosis),weeks later bilateral posterior uveitis and subsequebt datachment.few weeks later the daetachment subsides with pigmentary choriodal changes and depigmentation of the retina.

VKH skin changes (vitiligo of the lashes and eyebrows)

Fuchs heterochromic uveitis

Rare , chronic ,young adults Unknown cause , no systemic association

Mild ant uveitis,no signs of conjunctival inflamation, no ant synechia

KPs diffusely distributed over the cornea

Heterochromic iris due to loss of pigment epithelial cells.

Inflamed vitreous

70% catarct

Steroids arent effective and not prescribed,catarct surgery is done when indicated,and patients usually respond well

Sympathetic ophthalmitis rare, bilateral granulomatous uveitis after either surgical or accidental trauma to one eye. The ocular inflammation in the fellow eye becomes apparent usually within 3 months

after injury. Clinical presentation is an insidious or acute anterior uveitis with mutton-fat keratic

precipitates. The posterior segment manifests moderate to severe vitritis, usually accompanied by

multiple yellowish-white choroidal lesions. Evidence suggests that it represents an autoimmune inflammatory response against choroidal melanocytes mediated by T cells.

Diagnosis is based on clinical findings and a history of previous ocular trauma or surgery. Other causes of granulomatous uveitis, such as Vogt-Koyanagi-Harada disease, sarcoidosis, tuberculosis, and syphilis should be considered.

Treatment of sympathetic ophthalmia consists of systemic anti-inflammatory agents with high dose oral corticosteroid as the drug of choice.

However, if the inflammation cannot be controlled, cyclosporine is then used. Other immunosuppressive agents, such as chlorambucil, cyclophosphamide or azathioprine, may be necessary for the control of inflammation.

The role of enucleation after the diagnosis of sympathetic ophthalmia remains controversial. Visual prognosis is reasonably good with prompt wound repair and appropriate immunomodulatory therapy.

Treatment Aims:

1. relief pain & inflammation

2. prevent ocular structural damage

3. prevent visual loss & retinal or optic

damage

Medication:

cycloplegia

Long acting cycloplegic agents (cyclopentolate , hematropine )used to relief pain& photophobia by medriasis .

Corticosteroids

1. Topical , is the mainstays of therapy used aggressively esp. in initial phases of therapy ,

if no response in 7-10 days we use :

2. subconjunctival injection (celestone) , used if pt poorly complies to topical type or iritis not respond to topical ttt alone

3. oral corticosteroid maybe necessary in severe cases of iritis and post uveitis

Topical aqueous suppressant

in case of elevated IOP

Little or No benefit of using NSAIDS in iritis

Ant.uveitis: dilating pupil to prevent synechia

(homatropine , cyclopentolate or atropine drops)

To break synechia

1. initially …….intensive cyclopentolate ,

phenylphrene or tropicamide drops

2. if resistant synechia …. Subconjuctival

mydriatics

post.Uveitis:

visual loss occur due to destructive process by retinitis or macular edema due to fluid acumulation…………. So here we use antiviral, antibiotics or systemic steroids

some rare severe uveitis may need other immunesuppresive agents (Azathioprine, cyclophosporin)

Follow up: * reexamine pt 2-3 wks later to ensure no

residual inflammation present Complication * recurrent episodes of iritis & the subsequent

therapy may lead to cataract or glucoma Prognosis * Most pt expect to have recurrent iritis. * Visual prognosis is good in absence of either

cataract or glucoma.

Specific conditions ass. with uveitis

DiseaseHxSignsInvestigati-ons

TreatmentPrognosis

ASAnt.uveitisStifness at

restBackache

Typical signs of Ant.uveitis

HLAB27

Sacroili-ac

X-ray

Ocular tttAnti-

inflammatorphysiotherapy

Recuurent attacks

But good for vision

Rieter’sdisease

Male , HLAB27

Urethritis , arthritis,

conjunctivitis

40% withAcute ant uveitis

Juvenile chronic arthritis

Chronicasymptomatic ant. Uveitis

Profound visual defect

White eye 70% shows

bilateralANA

Ocular tttSystemic ttt of arthritis

Fuch’s Heterochromi

c uveitis

Blurred vision & floaters

(not present with typical iritis Hx.)

Mild ant. uveitisHeterochromic iris

Catract 70%Glucoma,KPs,

No post. Synechia

Not respond to steroid

Respond to cataract surgery

Dis.HxSignsinvestigations

Treatment

ToxoplasmosisMostly Congenital(50-

75%)Hazy vision ,

floaters , red & painful eye

Creamy foci of

InflammatoryCells at the

marginAt the margin

of chorioretinal

scar

+ve toxoplasmosisAntibody test is suggestive

Systemic steroid

&clindamycin