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joint pain

Dear team,
My joint pain started years ago. Taking ibuprofen the pain usually disappeared after 1-3 days.
This time the joint pain has been around for weeks with short interruptions only. In between it became so bad that I could hardly move, additionally, I had a fever. Due to the joint pain and my lung situation I am having infusions at present. The lung has been much better recently but the pain hasn’t disappeared. It comes and goes – and I cannot see any connections with inflammations in the blood or specific drugs.
My blood was checked regarding rheumatism, everything is fine. Now I am to get on with ibuprofen and maybe cortisone if the pain doesn’t disappear. Is there anything else I could do?
Thank you very much!
Dear questioner,
Many thanks for your question to ECORN. Arthritis in people with cystic fibrosis occurs infrequently, but when it does it can cause considerable problems. There are two distinct types of arthritis that have been described in cystic fibrosis: cystic fibrosis-related arthropathy and hypertrophic osteoarthropathy. As people with CF are living longer, the number of cases of CF related arthritis may be increasing.

CF related arthopathy has not been clearly defined. People can suffer from recurrent episodes of joint pain, swelling, tenderness and limitation of movement which may be disabling. Sometimes the attacks are associated with high swinging fevers and skin rashes. CF related arthropathy can affect one or more joints. Symptoms often develop over approximately 12 to 24 hours and last less than one week and can disappear completely between attacks. In general, the joints affected should be rested, but gentle mobilisation should be continued as possible. The main treatment strategies for CF-related arthropathy involve analgesics such as ibuprofen, and anti-inflammatory therapies. You might also want to discuss a different non-steroidal anti-inflammatory drug with your CF team. Oral prednisolone or intra-articular corticosteroids such as triamcinolone are sometimes needed. When episodic symptoms progress to persistent disease, disease modifying anti-rheumatic drugs (DMARDs) such as methotrexate or azathioprine may be considered. Patients requiring DMARDS should be referred to a rheumatology specialist as there can be considerable side-effects from these drugs. Two Cochrane Reviews have been published on treatment of CF-related arthritis (Thornton and Rangaraj 2008; Thornton and Rangaraj 2009). Both reviews concluded that further well designed studies are required to confirm the efficacy and safety of therapies for the management of arthritis related to CF in adults and children. Clinicians must balance potential benefit against the possible risk of complications in each case and you should take medications as prescribed by your doctor.

This answer has focused on CF related arthropathy, however, there are other problems which your CF clinician may wish to consider, including hypertrophic osteoarthropathy and bone pain secondary to use of intravenous bisphosphonates. Hypertrophic osteoarthropathy is less common and involves clubbing of the fingers and toes, enlargement of the extremities, and painful, swollen joints. It usually starts as a dull bone pain or episodic joint pain and is usually seen in people with CF with more severe lung disease. It also may worsen at times of lung infection. Intravenous bisphosphonates can also cause bone pain. These are used to increase bone mineral density in patients with CF who are at risk of bone fractures. The use of corticosteroids with intravenous bisphosphonates may reduce bone pain, however, further research in this area is required.

Best regards
Lisa Kent
Damian Downey
Stuart Elborn

Elkin S: Arthritis,Vasculitis and Bone Disease. Bush A, Alton EWFW, Davies JC, Griesenbach U, Jaffe A (eds): Cystic Fibrosis in the 21st Century. Prog Respir Res. Basel, Karger, 2006, vol 34, pp 270-277

Thornton J, Rangaraj S. Disease modifying anti-rheumatic drugs in people with cystic fibrosis-related arthritis. Cochrane Database of Systematic Reviews 2009, Issue 1. Art. No.: CD007336. DOI: 10.1002/14651858.CD007336.pub2.

Thornton J, Rangaraj S. Anti-inflammatory drugs and analgesics for managing symptoms in people with cystic fibrosis-related arthritis. Cochrane Database of Systematic Reviews 2008, Issue 1. Art. No.: CD006838. DOI: 10.1002/14651858.CD006838.pub2.