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

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

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.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Monoclonal, Humanized / administration & dosage*
  • Antiphospholipid Syndrome* / diagnosis
  • Antiphospholipid Syndrome* / drug therapy
  • Antiphospholipid Syndrome* / physiopathology
  • Catastrophic Illness
  • Cholecystectomy / adverse effects
  • Complement Inactivating Agents / administration & dosage
  • Drug Monitoring / methods
  • Humans
  • Male
  • Middle Aged
  • Postoperative Complications* / diagnosis
  • Postoperative Complications* / drug therapy
  • Postoperative Complications* / physiopathology
  • Recurrence
  • Treatment Outcome

Substances

  • Antibodies, Monoclonal, Humanized
  • Complement Inactivating Agents
  • eculizumab