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J Clin Rheumatol. 2015 Sep;21(6):311-3. doi: 10.1097/RHU.0000000000000290.

Eculizumab Induces Sustained Remission in a Patient With Refractory Primary Catastrophic Antiphospholipid Syndrome.

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1
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.

Abstract

Catastrophic 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.

PMID:
26267719
DOI:
10.1097/RHU.0000000000000290
[Indexed for MEDLINE]

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