title: [Anakinra treatment in Schnitzler syndrome -  results of the first retrospective multicenter study in six patients from the Czech Republic].
abstract: BACKGROUND
  Schnitzler syndrome is a very rare, acquired, autoinflammatory disease of mostly adult onset with characteristic combination of chronic recurrent urticaria and monoclonal immunoglobulin M or G gammopathy predisposing the patients to malignant lymphoproliferation. In this work, we analyzed the results of bio-logical therapy with anakinra on a national level aiming to supply data for effective pharmaco-economic estimates, lay the grounds of nationwide patient registry, raise awareness among professional public and optimize provided health care.
  
  
  METHODS
  The retrospective study (10/ 2006- 9/ 2013) included six males with definite Schnitzler syndrome verified by the new Strasbourg criteria. All patients were pretreated with antihistamines, nonsteroidal antiinflammatory drugs and glucocorticoids. Four patients underwent two or more treatment lines including intravenous bisphosphonates, 2- chlorodeoxyadenosine (cladribine), interferonα, PUVA photochemotherapy, cyclosporine A, thalidomide, bortezomib, chlorambucil, cyclophosphamide, colchicine and methotrexate. Anakinra monotherapy was initiated in standard dosing (100 mg subcutaneously daily).
  
  
  RESULTS
  Complete and partial remissions were achieved in five (83%) and one patients (17%), respectively. Complete remission was characterized by urticaria and pain regression (within hours), normalization of inflammatory markers (with--in days) and bone metabolism improvement assessed by the markers of osteoblastic osteoformation and osteoclastic osteoresorption in one case (within weeks). With normalized inflammatory markers (including interleukin6 and interleukin18), arthralgia and sporadic exacerbations of urticaria and fevers persist in the patient in partial remission with proven Q703K polymorphism in NLRP3 gene. The median treatment followup was 30.5 months (37.2 ± 31.2 (n = 6)). The dosing interval was prolonged in one case of complete remission to 48 hours. No serious adverse reactions occurred during anakinra application.
  
  
  CONCLUSIONS
  In Schnitzler syndrome, anakinra represents an effective, verified and safe medication with potentionally longterm administration not compromising its original efficacy and subjective tolerance. Anakinra, blocking autonomous inflammatory reaction of the organism via interleukin1 pathway, is a generally accepted first line treatment that should be made available in standard dosing for all Schnitzler patients.
authors.forename: P
authors.initials: P
authors.lastname: Szturz
authors.forename: A
authors.initials: A
authors.lastname: Sedivá
authors.forename: M
authors.initials: M
authors.lastname: Zurek
authors.forename: Z
authors.initials: Z
authors.lastname: Adam
authors.forename: J
authors.initials: J
authors.lastname: Stork
authors.forename: Z
authors.initials: Z
authors.lastname: Cermáková
authors.forename: P
authors.initials: P
authors.lastname: Steyerová
authors.forename: A
authors.initials: A
authors.lastname: Vokáčová
authors.forename: J
authors.initials: J
authors.lastname: Hrbek
authors.forename: M
authors.initials: M
authors.lastname: Sýkora
authors.forename: I
authors.initials: I
authors.lastname: Spička
authors.forename: Z
authors.initials: Z
authors.lastname: Mechl
authors.forename: J
authors.initials: J
authors.lastname: Mayer
mesh_terms: D018501:Antirheumatic Agents / Q000627:therapeutic use*; D018153:Czech Republic; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D012074:Remission Induction; D012189:Retrospective Studies; D019873:Schnitzler Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein