DOI: 10.36347/sajb.2016.v04i03.001

Pages: 185-188

Catastrophic APS (CAPS) is the most severe form of Antiphospholipid antibody syndrome with multiple organ involvement which occurs over a short period of time, usually associated with microthrombosis. We report a case of CAPS in a 39 year old male patient admitted with sudden onset of altered sensorium developing after pain in right lower limb over a period of 5 days. Past history of stroke was present which was not evaluated. Patient was in altered sensorium with bluish discoloration of right thigh and leg. Lower limb arterial Doppler study revealed extensive thrombosis of right Popliteal, femoral, iliac arteries extending in to distal aorta. Computerized Tomography of Brain revealed multiple infarcts in Brain i.e. Right middle cerebral artery, Left posterior Cerebral artery, with a midline shift of 8 mm. Patient also had deranged renal function. ECG, Echocardiography and cardiac enzymes revealed Inferior wall Myocardial Infarction. Patient’s Lupus anticoagulant (LA) was positive with elevated IgG (29.6 U/ml (n-[0.1-15]) aCL antibodies with normal IgM and anti-b-2 glycoprotein-1 IgG/IgM antibodies. Patient expired due to Multi organ dysfunction Syndrome (MODS). Definite CAPS is defined as thromboses in 3 or more organs developing within a week, microthrombosis in at least one organ and persistent aPL positivity. Young patients with thrombosis should be evaluated for the relatively common disease of APS as morality of CAPS is about 50%.