Disease modifying anti-rheumatic drugs in people with cystic fibrosis-related arthritis.

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.

Objectives: 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.

Search Strategy: 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

Selection Criteria: 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.

Data Collection And Analysis: No relevant studies were identified.

Main Results: No studies were included in this review.

Authors' 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.