The use of drugs to manage symptoms in people with arthritis related to cystic fibrosis

Arthritis is not a common complication of cystic fibrosis, but it can be a major complication 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 conditions is not clear because of the underlying disease and its intense treatment. We planned to report evidence from clinical studies to evaluate the effectiveness and safety of different anti-inflammatory analgesic drugs compared with placebo, with each other or with no treatment. However, we were disappointed that we could not find any completed randomised controlled studies of these treatments or any evidence from non-randomised controlled studies. One study in cystic fibrosis-related arthropathy has finished and may provide some evidence when published. We suggest that there should be a randomised controlled study to look at the effects and the safety of using anti-inflammatory drugs or painkillers or both to manage the symptoms of cystic fibrosis-related arthritis. This is an update of a previously published review (date of last search 19 January 2016). Since no studies have been included in the review up until January 2016, we will still search for studies every two years, but will not publish an updated version of this review until we can include any new studies.

Authors' conclusions: 

Although it is generally recognised that CFA may be episodic and resolve spontaneously, treatment with analgesics and anti-inflammatory agents may be needed. While this approach may be sufficient to manage symptoms, it is disappointing that no randomised controlled trials to rigorously evaluate these agents were found, nor could the authors identify any quasi-randomised. This systematic review has identified the need for a well-designed adequately-powered randomised controlled trial to assess the efficacy and safety of pharmacological agents for the symptomatic management of cystic fibrosis-related arthritis (CFA and HPO) in adults and children with cystic fibrosis. Studies should also better define the two conditions. A study has recently been conducted in CFA and may help fill this gap when analysed and published.

There are no trials included in the review up to January 2016. We will continue to run searches to identify any potentially relevant studies; however, we do not plan to update other sections of the review until new studies are published.

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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 (CFA) and hypertrophic pulmonary osteoarthropathy (HPO). Management of arthritis in people with cystic fibrosis is uncertain and complex because of the underlying disease and its intense treatment. This is an update of a previously published review.

Objectives: 

To review the effectiveness and safety of pharmacological agents for the symptomatic management of cystic fibrosis-related arthritis in adults and children with cystic fibrosis.

Search strategy: 

We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings.

Date of most recent search: 19 January 2016.

Selection criteria: 

Randomised controlled studies which compared the efficacy and safety of anti-inflammatory and analgesic agents (e.g. non-steroidal anti-inflammatory agents, systemic corticosteroids, intra-articular corticosteroids) with each other, with no treatment or with placebo for CFA and HPO.

Data collection and analysis: 

No relevant studies were identified.

Main results: 

No studies were included in this review.

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