title: Anakinra: an emerging option for refractory idiopathic recurrent pericarditis: a systematic review of published evidence.
abstract: OBJECTIVE
  Accumulating evidence suggests idiopathic recurrent pericarditis as a disease of probable autoinflammatory origin, and thus anakinra could be of benefit. The goal of this systematic review was to assess the efficacy and safety of anakinra in this context.
  
  
  METHODS
  Reports relevant to anakinra administration in patients with idiopathic recurrent pericarditis published up to October 2014 were searched in several databases. All references found, upon initial assessment at title and abstract level for suitability, were consequently retrieved as full reports for further appraisal.
  
  
  RESULTS
  Among 12 citations retrieved, nine reports (four case series and five case reports with 34 patients, 20 men, mean age 26.8 years) were assessed. The mean disease duration was 31 months and the number of recurrences 8.2. Anakinra was generally administered as a daily subcutaneous injection of 100 mg or as a mean dose of 1.1 mg/kg/d in weight-adjusted regimens. The mean full-dose duration was 9.2 months. C-reactive protein normalized within 7.1 days, and steroids were withdrawn within 62 days. Dose tapering was adopted in 64.7% of patients, leading to recurrence in 26% of cases. In a 28.3-month follow-up, eight out of 34 patients (23.5%) were disease free without treatment, after having received anakinra for 10.4 months overall. Anakinra was proved well tolerated, with mild local reaction being reported in 44% of patients.
  
  
  CONCLUSIONS
  Anakinra is a highly effective, rapidly acting, well tolerated and steroid-sparing agent. Recurrences after drug discontinuation are a matter of concern. Randomized trials are required to confirm these findings and address the most effective treatment protocol.
authors.affiliation: aCardiology Department, Maria Vittoria Hospital and University of Torino, Torino, Italy bCardiology Department, University of Athens Medical School, Hippokration General Hospital, Athens, Greece cInternal Medicine, Ospedale Papa Giovanni XXIII, Bergamo, Italy dDepartment of Medicine and Laboratory, Clinical Immunology and Rheumatology Unit, University of Athens Medical School, Hippokration General Hospital, Athens, Greece eGiannina Gaslini Institute; Department of Pediatrics, University of Genoa, Genoa, Italy.
authors.forename: George
authors.initials: G
authors.lastname: Lazaros
authors.forename: Massimo
authors.initials: M
authors.lastname: Imazio
authors.forename: Antonio
authors.initials: A
authors.lastname: Brucato
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Vassilopoulos
authors.forename: Panagiotis
authors.initials: P
authors.lastname: Vasileiou
authors.forename: Marco
authors.initials: M
authors.lastname: Gattorno
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Tousoulis
authors.forename: Alberto
authors.initials: A
authors.lastname: Martini
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000009:adverse effects / Q000627:therapeutic use*; D019317:Evidence-Based Medicine / Q000379:methods; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000009:adverse effects / Q000627:therapeutic use*; D010493:Pericarditis / Q000188:drug therapy*; D012008:Recurrence
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D053590:Interleukin 1 Receptor Antagonist Protein