Grand Round Dr Amir H Mani Fellow NUH

History 27 years Indian, F C/o Progressive BOV for 1/12 RE(27 /08/05) Had RE photophobia and pain month back Similar symptom LE one year ago( May 2004 ) Poliosis No family history of eye disease NO Medical history

Ophthalmic examination CVR 6/30 CVL 6/6 AS =>NAD RT RAPD Fundus exam Macular scar ? RE Fundus Photo FFA HVF ERG Lab => Autoimmune work up

Differential diagnosis (? choroiditis ? macular scar) Infective => Histoplasmosis VKH Autoimmune choroiditis APS

Blood Investigation WBC 10.2 ESR =8 URIC ACID 410( ) Anti-cardiolipin IgM Positive Anti Cardiolipin Ig G Negative Lupus Anticoagulant present A PTT > 38.3 RF, ANA SLE Panel => C3,c4 Anti Ds DNA ANCA Negative Syphilis Screen = non reactive

APS(Antiphospholipid Syndrome) Antiphospholipid syndrome (APS) characterized by recurrent venous or arterial thrombosis and/or pregnancy losses associated with typical laboratory abnormalities Age => young to middle aged adult Sex => female predominance Race no predominance Mortality Increase incidence of thrombotic Disease, DVT, CVA pulmonary emboli,Myocardial infarction in young

At least one clinical criterion an one laboratory criterion Clinical Vascular Thrombosis Miscarriage LAB aCL antibodies(IgG or IgM) on 2 or more occasions at least 6 weeks apart Anti–beta-2 glycoprotein I antibodies Activated partial thromboplastin time (aPTT) LA tests such as dilute Russell viper venom time (DRVVT) Serologic test result for syphilis (false positive) CBC count (thrombocytopenia, Coombs-positive hemolytic anemia)

Primary APS Without clinically or serologically evident autoimune disorders Secondary => (35%) SLE Common autoimmune /Rheumatic diseases with + aPL – SLE % – Sjögren syndrome - 42% – Rheumatoid arthritis - 33% – Autoimmune thrombocytopenic purpura - 30% – Autoimmune hemolytic anemia - No figure available – Psoriatic arthritis - 28% – Systemic sclerosis - 25% – Mixed connective-tissue disease - 22% – Polymyalgia rheumatica or giant cell arteritis - 20% – Behçet syndrome - 20% 1-5% in healthy population+ aPL

Ocular manifestation The most common ocular pathology Thrombosis with resultant ischemia(CRVO,CRAO) Vitreous Hemorrhage Choroidal vascular occlusion and RPE changes Anterior ischemic optic neuropathy (AION), Transient ischemic attack (TIA), amaurosis fugax, Isolated retinal hemorrhages Cotton wool spots Retinal neovascularization

Treatment Anticoagulation – Coumadin Moderate to high dose (INR >2) is protective Low dose (INR <1.9) does not prevent thrombosis – Aspirin Protects against pregnancy loss Does not protect against thrombosis LMW Heparin Avoid factors predisposing to thrombosis – Oral Contraceptive use – Immobility Modify atherosclerotic risk factors – Hyperlipidemia – Tobacco abuse – Diabetes Mellitus