Catastrophic antiphospholipid syndrome presenting as ischemic pancreatitis in systemic lupus erythematosus

Antiphospholipid syndrome is often associated with systemic lupus erythematosus. Both syndromes have different clinical manifestations based on organ involvement. Antiphospholipid syndrome commonly causes spontaneous abortions, cerebral vascular occlusion, and deep venous thrombosis. Catastrophic antiphospholipid syndrome occurs when three or more organ systems are affected by thromboses in less than a week.

We report a unique case of a young woman with a history of systemic lupus erythematosus and antiphospholipid syndrome who presented with recurrent ischemic pancreatitis. Pancreatitis was refractory to anticoagulation and low dose steroids. Secondary to recurrence of pancreatitis and other organ involvement, she was treated as a presumed case of catastrophic antiphospholipid syndrome. Aggressive treatment with plasmapheresis, corticosteroids, cyclophosphamide, and anticoagulation eventually led to her recovery.

Awareness of this rare, rapidly fatal medical condition prompts vital, early intervention to improve patients' survival. This case report aims to add to the limited therapeutic data available as well as suggest a possible approach to treating this rare syndrome with very high morbidity and mortality.