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.
From the Department of Internal Medicine IV (Nephrology and Hypertension) (AK, PS, GM); Department of Radiology (RF), Medical University Innsbruck, Innsbruck, Austria; Institute of Immunology (MK), University of Heidelberg, Heidelberg, Germany; 3rd Department of Medicine (AS, DC, ZP), Research Laboratory, Faculty of Medicine, Semmelweis University, Budapest, Hungary; and Department of Nephrology and Dialysis (KL), Academic Teaching Hospital Feldkirch, Feldkirch, Austria
Correspondence: Andreas Kronbichler, MD, Anichstraße 35, 6020 Innsbruck, Austria (e-mail: email@example.com).
Abbreviations: CAPS = catastrophic antiphospholipid syndrome, IAS = immunoadsorption, IgA = immunoglobulin A, IgG = immunoglobulin G, IgM = immunoglobulin M, LDH = lactate dehydrogenase, SLE = systemic lupus erythematosus.
All interventions given were part of normal health care and thus ethical approval was neither obliged, nor sought. However, approval from the patient was obtained to publish the case report.
The authors have no funding and conflicts of interest to disclose.
This is an open access article distributed under the Creative Commons Attribution License 4.0, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0
Received July 22, 2014
Received in revised form September 1, 2014
Accepted September 4, 2014