Table 4

Summary of evidence and regulatory authorisations for IL-1 blockade and TNF-blockade

LEMA approvalFDA approval
 Canakinumab1BApproved for patients with CINCA/NOMID, MWS and ‘severe FCAS’* ≥2 years and ≥7.5 kg body weightApproved for patients with FCAS and MWS ≥4 years
 Rilonacept1BApproved for patients with FCAS and MWS ≥12 years
 Anakinra2AApproved for all patients with CAPS ≥8 months and ≥10 kg body weightApproved for patients with CINCA/NOMID (all ages)
 Canakinumab3Orphan designation
  • L, level of evidence as assessed in a systematic review up to June 2013. 1B, randomised controlled study; 2A, controlled study without randomisation; 2B, quasi-experimental study; 3, descriptive study.9 Approval as published on websites of EMA (European Medicines Agency) and FDA (US Food and Drug Administration) up to June 2015.37 ,38

  • *Severe FCAS is defined by EMA as FCAS presenting with signs and symptoms beyond cold-induced urticarial skin rash.

  • CAPS, cryopyrin-associated periodic syndromes; CINCA, chronic, infantile, neurological, cutaneous and articular syndrome; FCAS, familial cold autoinflammatory syndrome; IL, interleukin; MKD, mevalonate kinase deficiency; MWS, Muckle-Wells syndrome; NOMID, neonatal-onset multisystem inflammatory disease; TNF, tumour necrosis factor; TRAPS, tumour necrosis factor receptor-associated periodic syndrome.