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Uveitis. Introduction. Uveitis : inflammation of uveal tract (iris, ciliay body & choroid) It is classified anatomically to Ant.uveitis (iritis)
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Introduction • Uveitis : inflammation of uveal tract (iris, ciliay body & choroid) • It is classified anatomically to • 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.
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.
Mostly unilateral • Hx. pt come to clinic complaining of: • 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 • Photophobia • Blurring of vision Redness of the eye
HISTORY • Respiratory symptoms (S.O.B , cough , nature of sputum)……..TB, Sarcoidosis. • Skin problems acompained by uveitis erythema nodosum (arms +chin) >>>> Sarcoid & Bechet oral & genital ulcers >> Bechet • Joint disease AS……….20% ant.uveitis juvenule chronic arthrits Reiter syndrome………..40% ant.uveitis • 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
Signs KP: fibrous deposits on the posterior surface of the cornea, usually associated with uveitis. Both the size and distribution of keratic precipitates are helpful in the differential diagnosis. White, yellowish greasy precipitates of inflammatory cells Typically distributed in a wedge-shaped regionontheinferior corneal endothelium, known as Arlt's triangle
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.
Invetigations Aim : determine systemic association Investigation guided by past medical Hx & review of system: Pt with first episode of nongranulomatus iritis with unremarkable past medical Hx & review of system…so investigations not indicated 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
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 spondylitisand reactive arthritis, are the most common systemic immune disorders associated with uveitisin 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 prognosisfor thisform of uveitisisgenerally 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 frequentlyassociated 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.
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.
