Eculizumab Induces Sustained Remission in a Patient With Refractory Primary Catastrophic Antiphospholipid Syndrome.
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.
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.
Thomas A
TA
Zikos
Jeremy
J
Sokolove
Neera
N
Ahuja
Caroline
C
Berube
D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D016736:Antiphospholipid Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D002388:Catastrophic Illness; D002763:Cholecystectomy / Q000009:adverse effects; D051056:Complement Inactivating Agents / Q000008:administration & dosage; D016903:Drug Monitoring / Q000379:methods; D006801:Humans; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D012008:Recurrence; D016896:Treatment Outcome
D061067:Antibodies, Monoclonal, Humanized; D051056:Complement Inactivating Agents; C481642:eculizumab