1. Shibu lijack

2. Speaker: Kumar Saurabh

3.  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

4.  Prevalence: 0.73% worldwide  Age group: 20-50 years  Five to 20% of legal blindness  Tedious diagnostic workup  Elusive aetiologies  Permanent structural damage  Low therapeutic index of medications Problem Statement

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

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

7. 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

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

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

10. 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

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

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

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

14. Keratic Precipitates Large keratic precipitates seen in case of granulomatous uveitis

15. 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 0.5 + : 1-5 cells + : 6-15 cells ++ : 16-25 cells +++ : 26-50 cells ++++ : >50 cells Cell * 0 : Nil 0.5 + : 1-5 cells + : 6-15 cells ++ : 16-25 cells +++ : 26-50 cells ++++ : >50 cells *Standardization of Uveitis Nomenclature (SUN). AJO 2005;140: 509-16

16. Anterior Chamber Reaction Cells and flare in uveitic eyes

17. Iris Nodules

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

19. 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

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

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

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

23. 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

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

25. 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

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

27. 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

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

29. Treatment Strategy

30. 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: 40-60 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: 40-60 mg/ day From D3-D7, tapered

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

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

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

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

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

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

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

38. 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: 509-16

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

40. Shibu lijack