112 Musculoskeletal symptoms represent a significant burden for adults with cystic fibrosis

Abstract

Background: Cystic fibrosis (CF) is a multi-system disease with a large inflammatory burden. The impact of CF on the musculoskeletal system includes inflammatory joint disease, effects on bone mineral density, muscle dysfunction, and postural changes. Despite this, it remains an under-researched area. Methods: Our multidisciplinary team designed a bespoke screening questionnaire to quantify musculoskeletal symptoms affecting adults with CF. We drew on a screening tool for psoriatic arthritis in patients with skin psoriasis (Psoriasis Epidemiology Screening Tool), as a tool for screening an at-risk population for inflammatory arthritis, and with an awareness that the inflammatory arthritis seen in CF in a pilot study fitted a predominantly seronegative disease pattern. This questionnaire is being used to identify patients who potentially have inflammatory joint disease that may need review by Rheumatology, and those who may benefit from physiotherapy review. Results: To date, 250 adult patients across 2 tertiary CF centres have completed the questionnaire at routine clinic appointments. Demographics: median age 30; 57% male; 52% homozygous for dF508 CF mutation; FEV1 mean 52%predicted; 73% colonised with pseudomonas aeruginosa, 86% pancreatic insufficient and 40% CFrelated diabetes. 77% reported back and/or joint pains within the last year. Activities of daily living were impacted by joint pain in the last year for 35% and by back pain for 38%. 29% had predominantly peripheral disease, with knees the most commonly affected. 28% of patients reported having had a swollen joint and 14% of patients reported early morning stiffness which may indicate underlying inflammatory arthritis. Conclusion: Musculoskeletal symptoms constitute a significant burden for adults with CF, affecting activities of daily living for a third of patients. Previous reports highlight the lack of a universal definition for cystic fibrosis associated arthropathy, and the range of conditions represented here include inflammatory arthritis, osteoarthritis, and deconditioning. The need for appropriate rheumatological assessment and treatment from a multidisciplinary team is clear. Better phenotyping for those with inflammatory arthritis is now possible in terms of imaging with ultrasound, as well as serological tests including ACPA. In addition to the significant burden from musculoskeletal symptoms, exercise is vital to the treatment of CF lung disease: the impact of joint pain on ability to exercise could have significant consequences in terms of lung disease progression.