Catastrophic anti-phospholipid syndrome in the absence of IgG anti-cardiolipin antibodies.
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 occurring 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.
Kennedy
M
M
Department of Histopathology, St. James's Hospital, Dublin, Ireland.
Jackson
J
J
Khan
I
I
Keeling
P W
PW
Gaffney
E
E
D000328:Adult; D017153:Antibodies, Anticardiolipin / Q000097:blood*; D016736:Antiphospholipid Syndrome / Q000209:etiology / Q000276:immunology* / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008180:Lupus Erythematosus, Systemic / Q000150:complications*
D017153:Antibodies, Anticardiolipin; D007074:Immunoglobulin G; D016682:Lupus Coagulation Inhibitor