Catastrophic
antiphospholipid syndrome (CAPS) is a rare but devastating complication in patients with
antiphospholipid syndrome (APS) with a high morbidity and mortality.We describe a case of a 30-year old female patient with
immunoglobulin A (
IgA) deficiency who underwent
splenectomy because of idiopathic thrombocytopenic
thrombocytopenia. Subsequently, an APS and finally
systemic lupus erythematosus was diagnosed. After an uncomplicated pregnancy that was terminated by
cesarean section, the patient developed severe CAPS with cerebral, myocardial, renal, and pulmonary involvement.Because of
IgA deficiency, standard
therapy consisting of
plasmapheresis and
intravenous immunoglobulins in addition to
steroids was not tolerated. After 8 sessions of immunoadsorption (IAS), massive pulmonary
hemorrhage was controlled but relapsed twice whenever IAS was terminated. As other
immunosuppressive agents were considered dangerous because of the risk of
infections in the face of severe
hypogammaglobulinemia, we administered
eculizumab, an inhibitor of the terminal
complement pathway, which led to a persistent control of
her disease. Interestingly,
eculizumab therapy was associated with a further decline of
complement C3 and C4 serum levels. The patient developed a subsequent flare of her
systemic lupus erythematosus, potentially indicating that
complement inhibition by
eculizumab is not effective in preventing lupus flares.Taken together, we describe a unique case of life-threatening and difficult-to-treat CAPS with a good clinical response after
terminal complement complex inhibition with
eculizumab. Further controlled trials are necessary to investigate the value of
eculizumab in patients with CAPS.