The use of drugs to slow or stop progression of arthritis related to cystic fibrosis

Arthritis is not a common complication of cystic fibrosis, but it can cause major problems when it does occur. There are two distinct types of arthritis in cystic fibrosis: cystic fibrosis-related arthropathy and hypertrophic pulmonary osteoarthropathy. The best treatment for these types of arthritis is not clear because of cystic fibrosis and its intense treatment. Some drugs can treat the symptoms of arthritis by reducing inflammation of the joints and relieving pain, but only disease modifying anti-rheumatic drugs can stop or slow progression of the disease. We planned to report evidence from clinical trials which compared different disease-modifying drugs compared with placebo (or dummy treatment), with each other or with no treatment. However, we were disappointed that we could not find any completed randomised controlled trials of these treatments or any evidence from non-randomised controlled trials. We suggest that there should be a randomised controlled trial to look at the effects and the safety of using disease modifying anti-rheumatic drugs to slow or stop the progression of arthritis in people with cystic fibrosis.

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, disease-modifying anti-rheumatic drugs may be needed to limit the course of the disease. It is disappointing that no randomised controlled trials to rigorously evaluate these drugs 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 disease-modifying anti-rheumatic drugs 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.

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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 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 Cystic Fibrosis 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: 10 July 2012.

Selection criteria: 

Randomised controlled trials which compared the efficacy and safety of disease-modifying anti-rheumatic drugs (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.

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