Background/Purpose: There are limited published studies about patterns of arthropathy seen in cystic fibrosis (CF) patients. The objective of this study was to determine the musculoskeletal involvement, clinical presentation, serological patterns, and treatments used in a cohort of CF patients.

Methods:  This is a retrospective chart review of patients followed in the Pulmonary CF Clinic in a tertiary medical center from 2004 through 2014.

Results:  Of 204 adult CF patients (age 18-67, median age 41, 100 males and 104 females), 88 patients presented with some form of musculoskeletal complaints. Of these, 66 had nonspecific arthropathy. Fourteen out of 66 had flare of arthralgia following CF pulmonary exacerbations. Ten out of 88 were diagnosed with CF arthropathy based on patterns described in other published cohorts (4.9%), 2 with osteoarthritis, 4 with fibromyalgia, and 6 with nonspecific myalgia/arthralgia. Of the 204 patients, ANA was measured in 25 patients and only 6 were elevated. RF was measured in 19 patients; 3 had low to moderate titers.  CCP was negative in all 16 measured patients. None of the patients met the diagnostic or classification criteria for any rheumatologic diseases except for one who was diagnosed with Sjogren’s syndrome with a positive ANA and SSA.

Six out of 10 CF arthropathy patients (7 women, 3 men, age 23-59) were seen by a rheumatologist and all 10 patients had polyarticular disease. Large joints in a symmetrical pattern were more often involved than small joints. Three patients not seen by a rheumatologist had documented ‘generalized’ polyarthralgia and a specific joint group was not identified. Only 1/10 had documented synovitis of a 4^thPIP. Five out of 10 patients had x-rays of the affected joints which did not show erosions or deformities. Three out of 10 patients had elevated ANA and 1 had a positive SSA diagnosed as Sjogren’s by a Rheumatologist. RF and CCP were measured in 5/10 and 4/10 patients, respectively, and all were negative. Of the 10 CF arthropathy patients, 6 had documented flares of arthralgia with CF pulmonary exacerbations; in 2 patients arthropathy did not correlate with CF exacerbations; and 2 patients did not have clear documented arthropathy with CF exacerbations. Arthralgia responded to NSAIDs in 7/10 patients. Arthralgia responded to 3/6 patients treated with varying doses of prednisone initiated either for arthropathy or for pulmonary symptoms.  Four patients were treated with hydroxychloroquine and one with both hydroxychloroquine and sulfasalazine with some possible response.  One patient who was managed by an outside rheumatologist was treated with leflunomide and previously with  methotrexate and cyclosporine.  Arthralgia improved in 2 patients treated with tramadol and in 1 patient treated with hydrocodone/acetaminophen. Arthralgia did not respond to acetaminophen alone in any of the patients.

Conclusion:  In this large cohort of cystic fibrosis patients, CF arthropathy was diagnosed in about 5% of patients.  Seronegative nonerosive noninflammatory polyarticular large joint disease was the dominant pattern.  In some patients, the arthralgia appeared to respond to NSAIDs. The flare of arthropathy appeared to correlate with CF pulmonary exacerbations in some patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/patterns-of-arthropathy-in-patients-with-cystic-fibrosis/