title: Treatment of hereditary autoinflammatory diseases.
abstract: OBJECTIVE
  The purpose of this review is to summarize recent advances in the treatment of the hereditary autoinflammatory diseases, focusing on Familial Mediterranean fever (FMF), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndromes (CAPS) and mevalonate kinase deficiency (MKD). We discuss recently published studies and their implications for current patient care and future clinical research.
  
  
  RESULTS
  Interleukin (IL)-1 blockade is effective in most autoinflammatory conditions. Younger patients require a higher dose per kg of body weight. In FMF, colchicine remains the treatment of choice. Single daily dosing appears adequate. When colchicine fails, IL-1 blockade is effective. In CAPS, the beneficial effect of IL-1 blockade is sustained, and side-effects are limited. There is no evidence that one IL-1 blocker is superior to the other. In TRAPS and MKD, IL-1 blockade appears effective. Some patients have sufficient suppression of inflammatory symptoms with NSAIDs or corticosteroids.
  
  
  CONCLUSIONS
  Apart from CAPS and FMF, therapy of autoinflammatory diseases is based on small and retrospective studies. IL-1 blockade appears effective, but larger prospective trials are needed, especially in MKD, TRAPS and colchicine-resistant FMF patients.
authors.affiliation: aDepartment of Pediatric Rheumatology bDepartment of General Pediatrics, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Nienke M
authors.initials: NM
authors.lastname: Ter Haar
authors.forename: Joost
authors.initials: J
authors.lastname: Frenkel
mesh_terms: D001688:Biological Products / Q000627:therapeutic use; D003078:Colchicine / Q000627:therapeutic use; D056587:Cryopyrin-Associated Periodic Syndromes / Q000188:drug therapy; D010505:Familial Mediterranean Fever / Q000188:drug therapy; D056660:Hereditary Autoinflammatory Diseases / Q000188:drug therapy*; D006801:Humans; D007375:Interleukin-1 / Q000037:antagonists & inhibitors; D054078:Mevalonate Kinase Deficiency / Q000188:drug therapy; D012720:Severity of Illness Index
chemical_list: D001688:Biological Products; D007375:Interleukin-1; D003078:Colchicine