The role of eculizumab in catastrophic antiphospholipid syndrome

Julia Fattore and Elizabeth Dalton

Background

Catastrophic antiphospholipid syndrome is a potentially fatal and rare manifestation of antiphospholipid syndrome (APS). It occurs in 1% of APS affected individuals and carries significant mortality. In this report, we discuss the clinical history, investigations and response to treatment in a patient who presented with pancytopenia on a background of APS who developed a florid autoimmune disease with multi-organ failure.

Case presentation

A patient with a 15-year history of APS was admitted for further investigation of pancytopenia with a history of polyarthropathy, fatigue and fevers. He had ongoing fevers during admission with a negative septic screen and on day six developed multi-organ failure including renal, respiratory, vascular and neurological compromise. Over the course of a month he remained in intensive care where he received multiple blood transfusions and routine care for his autoimmune crisis. In addition, he received eculizumab, a humanised monoclonal IgG antibody which is currently a novel agent for autoimmune crisis. Over the following two months he responded favourably with recovery of end organ function.