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Grand Round

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 .

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Grand Round

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  1. Grand Round Dr Amir H Mani Fellow NUH

  2. 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

  3. 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

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

  5. Blood Investigation • WBC 10.2 • ESR =8 • URIC ACID 410(150-370) • 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

  6. 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

  7. 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)

  8. Primary APS Without clinically or serologically evident autoimune disorders Secondary => (35%) SLE Common autoimmune /Rheumatic diseases with + aPL SLE - 25-50% 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

  9. 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

  10. 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

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