Grand Round

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Grand Round

• Dr Amir H Mani
• Fellow NUH

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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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Ophthalmic examination

• CVR 6/30 CVL 6/6
• AS gtNAD
• RT RAPD
• Fundus exam Macular scar ? RE
• Fundus Photo
• FFA
• HVF
• ERG
• Lab gt Autoimmune work up

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• Differential diagnosis
• (? choroiditis ? macular scar)
• Infective gt Histoplasmosis
• VKH
• Autoimmune choroiditis
• APS

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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 gt 38.3
• RF, ANA
• SLE Panel gt C3,c4 Anti Ds DNA
• ANCA
  Negative
• Syphilis Screen non reactive

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APS(Antiphospholipid Syndrome)

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

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• 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
• Antibeta-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)

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• Primary APS
• Without clinically or serologically evident
  autoimune disorders
• Secondary gt (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

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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

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Treatment

• Anticoagulation
• Coumadin
• Moderate to high dose (INR gt2) is protective
• Low dose (INR lt1.9) 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