Eculizumab Induces Sustained Remission in a Patient With Refractory Primary Catastrophic Antiphospholipid Syndrome. Issue 6 (September 2015)

• Record Type: Journal Article
• Authors: Zikos, Thomas A.; Sokolove, Jeremy; Ahuja, Neera; Berube, Caroline
• Is Part Of: Journal of clinical rheumatology. Volume 21:Issue 6(2015:Sep.)
• Publication Date: 2015-09
• Subjects: antiphospholipid syndrome -- catastrophic antiphospholipid syndrome -- complement cascade -- eculizumab; Rheumatism -- Periodicals; Rheumatology -- Periodicals; Musculoskeletal system -- Diseases -- Periodicals; Musculoskeletal Diseases -- Periodicals; Rheumatic Diseases -- Periodicals; Rhumatisme -- Périodiques; Rhumatologie -- Périodiques; Appareil locomoteur -- Maladies -- Périodiques; Musculoskeletal system -- Diseases; Rheumatism; Rheumatology; Periodicals; 616.723005
• DOI: 10.1097/RHU.0000000000000290
• Languages: English
• ISSNs: 1076-1608

Abstract

Abstract : 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.