Anakinra use during pregnancy in patients with cryopyrin-associated periodic syndromes (CAPS)

Arthritis Rheumatol. 2014 Nov;66(11):3227-32. doi: 10.1002/art.38811.


Objective: To describe the pregnancy course and outcome, and use of anakinra, a recombinant selective IL-1 receptor blocker, during pregnancy in patients with cryopyrin-associated periodic syndromes (CAPS), including familial cold auto-inflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal onset multi-system inflammatory disease (NOMID). Methods: Women currently enrolled in natural history protocols (NCT00059748, and/or NCT00069329 under IND) who have been pregnant were included. Subjects underwent a structured, standardized interview with regards to maternal health, pregnancy and fetal outcomes. Medical records were reviewed. Results: Nine women (four with FCAS, one with MWS and four with NOMID) reported one to four pregnancies, each resulting in a total of fifteen FCAS, three MWS, and six NOMID pregnancies. Six births from FCAS mothers and three births from NOMID mothers occurred while patients were receiving anakinra. If a woman became pregnant while taking anakinra, the pre-pregnancy anakinra dose was continued. Anakinra dose was increased during one twin pregnancy. No preterm births or serious complications of pregnancy were observed. One fetus of the twin pregnancy had renal agenesis and suffered fetal demise. Genetic testing showed the deceased twin carried the same NLRP3 c.785T>C, p.V262A mutation as the mother. The other twin is healthy and mutation negative. Conclusions: Anakinra was continued during pregnancy in women with CAPS and provided significant, persistent CAPS symptom relief while continuing to prevent the long-term sequelae of CAPS. Anakinra was well tolerated. Although a causal relation between anakinra and renal agenesis seems unlikely, further safety data are needed.

MeSH terms

  • Adult
  • Antirheumatic Agents / adverse effects
  • Antirheumatic Agents / therapeutic use*
  • Clinical Protocols
  • Congenital Abnormalities
  • Cryopyrin-Associated Periodic Syndromes / drug therapy*
  • Female
  • Fetal Diseases / chemically induced
  • Humans
  • Interleukin 1 Receptor Antagonist Protein / adverse effects
  • Interleukin 1 Receptor Antagonist Protein / therapeutic use*
  • Interviews as Topic
  • Kidney / abnormalities
  • Kidney Diseases / chemically induced
  • Kidney Diseases / congenital
  • Pregnancy
  • Pregnancy Outcome
  • Young Adult


  • Antirheumatic Agents
  • Interleukin 1 Receptor Antagonist Protein

Supplementary concepts

  • Hereditary renal agenesis

Associated data