Catastrophic Anti-phospholipid Syndrome in the Absence of IgG Anti-cardiolipin Antibodies

Abstract

The Catastrophic Anti-phospholipid Syndrome (CAPS) is a rare acute clinical syndrome associated with serum anti-phospholipid antibodies (aPL). It is rarely preceded by a precipitating event. It may occur as a primary event or be associated with auto-immune diseases. We report a fatal case occuring post-endoscopic retrograde cholangio-pancreatography (ERCP) in a patient with Systemic Lupus Erythematosus (SLE), positive lupus anticoagulant and negative IgG with positive IgM anti-cardiolipin titres. The diagnostic and therapeutic difficulties of such cases is addressed.

Key Words:

• catastrophic anti-phospholipid syndrome
• systemic lupus erythematosus
• precipitating event
• anti-cardiolipin antibodies