Catastrophic antiphospholipid syndrome: a case series

Antiphospholipid syndrome (APS) is an autoimmune disease with clinical manifestations of arterial and venous thrombosis, obstetric manifestations, and the presence of antiphospholipid antibodies or lupus anticoagulant. Catastrophic APS is a rare variant of APS, defined as acute failure of at least three tissues, organs or systems caused predominantly by small vessel thrombosis confirmed by histopathologic evidence. Catastrophic APS develops rapidly and leads to death in 30% of cases.

We evaluated 11 patients with catastrophic APS -8 of them with a probable diagnosis of catastrophic APS and 3 with a definite diagnosis--admitted to Beilinson hospital during the period 2003-2011.

Overall venous events numbered 18 and overall arterial events 10. The event duration per patient was 2.6 +/- 1.2 weeks (mean +/- SD). Deep vein thrombosis of the legs was quite common (7 events), as was venous intraabdominal thrombosis (10 events). Eight patients had microangiopathic anemia with schizocytes seen in the blood smear. The mean +/- SD hemoglobin level was 10.3 +/- 3.6 g/dl and the mean +/- SD creatinine level 0.98 +/- 0.78 mg/dl. All our patients had high acute-phase reactant and all had lupus anticoagulant positivity, The most common positive antibodies were immunoglobulin G anticardiolipin (8 patients) and IgG beta2-glycoprotein (7 patients). During the events warfarin was stopped and the patients were given intravenous heparin. All the patients received steroids in variable doses. Five patients underwent plasma exchange, two patients received rituximab and two patients intravenous immunoglobulin.

Catastrophic APS, a rare syndrome, is important because of its major morbidity and mortality among young patients.