Background: Musculoskeletal problems in CF are reported in the literature, including an inflammatory arthritis. However, defining and delineating the diagnoses underlying these has been limited. We used a mixed methods approach to understand the scale of the problem and to identify a subset presenting with an inflammatory arthritis phenotype not attributable to other rheumatic disease. Methods 1: Collaborative design of a questionnaire which was then used to gain an overview of the scale and impact of musculoskeletal symptoms. Results: 49% of participants reported musculoskeletal symptoms impacting their activities of daily living in the last year. 44% reported back pain in the last week. The knee was the most commonly painful peripheral joint, with a quarter of participants reporting knee pain within the last week and 50% within the last year. Early morning stiffness and joint swelling were markedly less common, suggesting that the majority of musculoskeletal pain in CF is not due to an inflammatory arthritis. Methods 2: Assessment of people reporting joint swelling and early morning stiffness using ultrasound and blood tests alongside history and clinical examination to identify patterns of disease. Results: Cystic fibrosis associated arthritis (CFA) with clinical and ultrasound evidence of inflammatory arthritis was seen in 25 participants. 8 were found to have other rheumatological diagnoses. Methods 3: A literature review of the use of disease modifying drugs for any indication in people with CF pre-lung transplant alongside departmental data. Interpretation of this was supported by data from disease modifying drugs used in other lung pathologies to consider the safety issues associated with treating inflammatory arthritis in CF. Results: Disease modifying treatment does not appear to be associated with worsening of CF lung disease. Conclusion: Musculoskeletal problems are common in CF. A minority of these are inflammatory arthritis, some of which is CFA and some other rheumatological disease. Those who have non-CFA inflammatory arthritis should be treated according to national guidelines for that disease. Pre-treatment screening should be tailored but follow both national and local protocols. In the context of CFA, no treatment guideline exists, but where there is definitive evidence of synovitis treatment with disease modifying medications would seem appropriate and safe.
|Date of Award||1 Aug 2022|
- The University of Manchester
|Supervisor||Andrew Jones (Supervisor), Alexander Horsley (Supervisor) & Pippa Watson (Supervisor)|
- Cystic Fibrosis