Catastrophic antiphospholipid syndrome - A case report

Catastrophic antiphospholipid syndrome (CAPS) is a rare, potentially life-threatening condition, acute in onset , characterized by diffuse vascular thrombosis, leading to multiple organ failure in a short period of time in the presence of positive antiphospholipid antibodies (aPL). Lupus anticoagulant and anticardiolipin antibodies are the predominant antibodies associated with CAPS. Treatment options for CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. The high rate of mortality warrants greater awareness among clinicians for early diagnosis and treatment of CAPS. In this case report, 30-year-old post-partum female presented with progressive weakness, shortness of breath of grade IV and swelling of all the four limbs of 15 days duration with an episode of seizure. Investigations revealed MRV - cortical sinus venous thrombosis (CSVT) of transverse and sigmoid sinus, Raised anti-ds DNA anticardiolipin and lupus anticoagulant, 24 hour urinary proteins ? 540 mg/day indicating clinical lupus nephritis. Weakness of all the limbs with areflexia indicated acute inflammatory demyelinating polyneuropathy (AIDP). 2D echocardiography- post partum dilated cardiomyopathy (DCMP).