title: Disease modifying anti-rheumatic drugs in people with cystic fibrosis-related arthritis.
abstract: BACKGROUND
  Arthritis remains a relatively infrequent complication of cystic fibrosis, but is a cause of significant morbidity when it does occur. Two distinct types of arthritis are described in cystic fibrosis: cystic fibrosis-related arthropathy and hypertrophic osteoarthropathy. Management of arthritis in people with cystic fibrosis is uncertain and complex because of the underlying disease and its treatment.
  
  
  OBJECTIVE
  To review the effectiveness and safety of disease-modifying anti-rheumatic drugs (DMARDs) for the management of arthritis related to cystic fibrosis in adults and children.
  
  
  METHODS
  We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database handsearches of relevant journal and abstract books of conference proceedings.Date of most recent search: May 2008
  
  
  METHODS
  Randomised controlled trials which compared the efficacy and safety of DMARDs (e.g. methotrexate, gold, sulfasalazine, penicillamine, leflunomide, hydroxychloroquine and newer agents such as biologic disease modifying agents and monoclonal antibodies) with each other, with no treatment or with placebo for cystic fibrosis-related arthropathy or hypertrophic osteoarthropathy.
  
  
  METHODS
  No relevant studies were identified.
  
  
  RESULTS
  No studies were included in this review.
  
  
  CONCLUSIONS
  Although it is generally recognised that cystic fibrosis-related arthritis can be episodic and resolve spontaneously, treatment with analgesics and anti-inflammatory agents may be needed. But when episodic symptoms progress to persistent disease, DMARDs may be needed to limit the course of the disease. It is disappointing that no randomised controlled trials to rigorously evaluate DMARDs could be found. This systematic review has identified the need for a well-designed adequately powered randomised controlled trial to assess the efficacy and safety of DMARDs for the management of cystic fibrosis-related arthropathy and hypertrophic osteoarthropathy in adults and children with cystic fibrosis. However, given the infrequency of cystic fibrosis-related arthritis and the range of symptoms and severity, randomised controlled trials may not be feasible and well-designed non-randomised observational studies may be more appropriate. Studies should also better define the two conditions.
authors.affiliation: Greater Manchester, Lancashire & South Cumbria Medicines for Children Research Network, 2nd Floor, Colwyn House, Royal Manchester Children's Hospital, Manchester, UK, M27 4HA. judith.thornton@cmmc.nhs.uk
authors.forename: Judith
authors.initials: J
authors.lastname: Thornton
authors.forename: Satyapal
authors.initials: S
authors.lastname: Rangaraj
mesh_terms: D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001168:Arthritis / Q000188:drug therapy* / Q000209:etiology; D003550:Cystic Fibrosis / Q000150:complications*; D006801:Humans
chemical_list: D018501:Antirheumatic Agents