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NHGEI Grand Ward Rounds

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					          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(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
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 - 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
                  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

				
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posted:5/7/2011
language:English
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