• Thomas A Zikos, Jeremy Sokolove, Neera Ahuja, and Caroline Berube.
• From the Divisions of *General Medical Disciplines and †Immunology and Rheumatology, Department of Medicine, ‡VA Palo Alto Healthcare System, and §Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, CA.
• J Clin Rheumatol. 2015 Sep 1; 21 (6): 311-3.

AbstractCatastrophic antiphospholipid syndrome (CAPS) is fatal in approximately 44% of patients in whom the diagnosis is made, thus demonstrating the inadequacy of current medical therapy. In this report, we discuss a 47-year-old man with a known history of primary antiphospholipid syndrome, who presented with CAPS after undergoing cholecystectomy and a treatment-refractory early relapse after development of colitis. Given the potential therapeutic efficacy of complement inhibition in antiphospholipid syndrome, the patient was administered eculizumab, a terminal complement inhibitor. Progressive clinical improvement and laboratory improvement were observed upon initiation of eculizumab. He has remained in remission for over 16 months of follow-up while on eculizumab. In conclusion, this case represents successful use of eculizumab for the treatment of primary CAPS.

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