Shibu lijack

Speaker: Kumar Saurabh

 Group of diseases characterized by severe sight threatening intraocular inflammation primarily involving the uveal tract which extend occasionally to the contiguous structures like vitreous, optic nerve head, cornea and sclera.* * International Uveitis Study Group Definition Uveitis: Definition

 Prevalence: 0.73% worldwide  Age group: years  Five to 20% of legal blindness  Tedious diagnostic workup  Elusive aetiologies  Permanent structural damage  Low therapeutic index of medications Problem Statement

Aetiology of Uveitis  Idiopathic Uveitis: 35.7%  Uveitis Secondary to Systemic Disease  Infectious Uveitis  Lens Induced Uveitis  Masquerade Syndrome

Goals of Uveitis Management  Reduction of visual morbidity  Proper identification of the specific disease  Cost effective utilization of resources

Approach: Ways to achieve that  History  Clinical Assessment: Site, stage and vision  Differential Diagnosis  Tailored Laboratory Tests  Correlation with Ancillary Tests  Definitive Treatment  Control of Adverse Effects

History: Salient Points  Detailed and Suggestive  Geographic History  Family History  Demography: Age, gender, race  Personal History: Pets, diet, drugs, sexual  Systemic Diseases  Ocular History

Ocular History  Onset: Insidious or Sudden  Laterality: Unilateral or Bilateral  Severity: Mild or Severe  Pattern: Single or Multiple Episodes  Duration: Acute or Chronic  Treatment

Clinical Assessment: Ocular  Visual Acuity  External : Lids, lacrimal glands  Conjunctiva & Sclera: Congestion, nodules  Cornea: Keratitis, ulceration, keratopathy  Keratic precipitates  Anterior chamber: Flare and cells  Iris: Surface, nodules, new vessels  Vitreous: Haze, detachment, hemorrhage  Retina: Retinitis, new vessels, oedema  Choroid: Choroiditis, neovascularization

Keratic Precipitates Mutton fat keratic precipitates in a case of granulomatous uveitis

Keratic Precipitates Fine keratic precipitates in a case of non- granulomatous uveitis

Keratic Precipitates Old keratic precipitates seen in the Arlt’s triangle of corneal endothelium

Keratic Precipitates Large keratic precipitates seen in case of granulomatous uveitis

Anterior Chamber Reaction Flare * 0 : Absence + : Barely detectable ++ : Moderate, iris details visible +++ : Marked, iris details not visible ++++: Intense, fixed fribrinous aqueous Flare * 0 : Absence + : Barely detectable ++ : Moderate, iris details visible +++ : Marked, iris details not visible ++++: Intense, fixed fribrinous aqueous Cell * 0 : Nil : 1-5 cells + : 6-15 cells ++ : cells +++ : cells ++++ : >50 cells Cell * 0 : Nil : 1-5 cells + : 6-15 cells ++ : cells +++ : cells ++++ : >50 cells *Standardization of Uveitis Nomenclature (SUN). AJO 2005;140:

Anterior Chamber Reaction Cells and flare in uveitic eyes

Iris Nodules

Vitreous Haze Direct Ophthalmoscope 0 : Clear vitreous + : Few, normal view ++ : Moderate scattered opacities, obscured view +++ : Many opacities, blurring of view ++++ : Dense opacities, no view

Vitreous Haze Indirect Ophthalmoscope ++++ : Optic nerve head obscured +++ : Optic nerve head visible, blurred border ++ : Better view of retinal blood vessels + : Better view of retinal blood vessels & ONH + : Blurring of nerve fiber layer striations 0 : Nerve fiber layer well defined

Retinal Features  Retinitis  Primary vasculitis  Secondary vasculitis  Retinal detachment  Macular oedema  Neovascularization

 Active choroiditis  Inactive choroiditis  Choroidal granulomas  Choroidal neovascularization  Choroidal vasculitis Choroidal Features

Site of Uveitis  Anterior Uveitis : Iritis, iridocyclitis, Anterior cyclitis  Intermediate Uveitis : Posterior cyclitis, Hyalites, Basal Retinochoroiditis  Posterior Uveitis : Chorioretinitis, Retinochoroiditis, Neuroretinitis, Choroiditis

Systemic Examination Mucocutaneous system  Erythema nodosum  Oral ulcerations  Kaposi sarcoma  Kearatoderama  Circinate balanitis  Vitiligo  Sarcoid granulomas Musculoskeletal system  Ankylosing spondylitis  Rheumatoid arthritis  Psoriasis  Behcet’s disease  Reiter’s syndrome

Differential Diagnosis  Working diagnosis  Basis for laboratory investigation  Basis for treatment

Laboratory Investigations  To rule out infective etiology  To alleviate risks of treatment  To find out systemic disease  To find out etiology of masquerade syndrome  To come to specific diagnosis  Academic purposes Aims

Laboratory Investigations  Granulomatous uveitis  Recurrent uveitis (>3 attacks)  Uveitis in a child  Positive leads on examination  Posterior uveitis  Retinal vasculitis  Worsening on steroids Indications

Laboratory Investigations  Complete Blood Count  Rheumatoid factor (RA)  Antinuclear Antibody (ANA)  Anti ds-DNA Antibody  Anti Neutrophil Cytoplasmic Antibody (ANCA)  Angiotensin Converting Enzyme  C- reactive Protein  X-ray Chest and Spine  Toxoplasma, Rubella, Cytomegalovirus, Herpes simplex (TORCH) Test  Mantoux Test  HLA Typing

Ancillary Investigations  Ultrasonography: Media opacity  Fluorescein Angiography: Macular oedema, Chorioretinitis, Disc leakage, Response  Vitreous Biopsy: Lymphoma, Endophthalmitis  Chorioretinal Biopsy:

Treatment Strategy

Specific Therapy Ocular Toxoplasmosis Regimen 1. Pyrimethamine: 75mg, D1 25mg/day, 4-6 weeks PLUS Sulphadiazine: 2 gm, D1 1 gm four times daily, 4-6wks PLUS Prednisolone: mg/ day From D3-D7, tapered PLUS Folinic Acid: 5mg twice weekly Regimen 2. Clindamycin 300mg, 4 times Daily, 4-6 weeks PLUS Sulphadiazine: 2 gm, D1 1 gm four times daily, 4-6wks PLUS Prednisolone: mg/ day From D3-D7, tapered

Specific Therapy  Peripheral Lesion: Follow up  Posterior Pole Involvement: Prednisolone 40mg/day and Thiobendazole 20mg/day. 5-7 days Vitrectomy Ocular Toxocariasis

Nonspecific Therapy: Medical  Steroids: Topical, periocular, systemic Indications: Active Inflammation Prevention and treatment of complications Infiltration of retina, choroid and optic nerve

Nonspecific Therapy: Medical  Nonsteroidal Antinflammatory Drugs Indication: To maintain lower dose of topical steroids

Nonspecific Therapy: Medical  Immunosuppressive Therapy Vision threatening intraocular inflammation Inadequate response to steroids Serious steroid induced side effects Contraindication of steroid therapy

 Immunosuppressive Therapy Absolute Indications: Behcet’s disease Sympathetic Ophthalmia Vogt-Koyanagi-Harada Syndrome Wegener’s Granulomatosis Polyarteritis Nodosa Nonspecific Therapy: Medical

 LASER and Cryotherapy Indications: LASER for choroidal neovascularization Cryotherapy for refractory pars planitis

Nonspecific Therapy: Surgical  Pars plana Vitrectomy Indications: Pars planitis Subretinal neovscular membrane

Treatment Response*  Inactive Uveitis : Rare cell in anterior chamber  Improvement : Two step decrease in cells Reduction in cell count to Grade 0  Worsening : Two step increase in cells Increase in cell count from 3+ to 4+  Steroid resistance : Two weeks  Immunosuppressive resistance : Three months *Standardization of Uveitis Nomenclature (SUN). AJO 2005;140:

Treatment of Complications  Management of cataract  Management of glaucoma  Management of band shaped keratopathy and other corneal complications

Shibu lijack