Background/Purpose: Arthropathy is a rare but debilitating manifestation of cystic fibrosis (CF) that has no formal definition. This study attempts to characterize the spectrum of arthropathy in CF patients.

Methods: A retrospective chart review was conducted on 246 adult CF patients within the Pulmonary CF Clinic who were seen at a single tertiary care center between January 1, 2008 and December 31, 2017. Charts were individually reviewed for description of joint symptoms. Patients were excluded if they had an alternative explanation for joint symptoms. Association of joint symptoms with pulmonary exacerbation were abstracted and defined based on clinical diagnosis of the treating physician. Pattern of joint involvement, duration of symptoms (acute as defined by < 6 weeks and chronic >6 weeks), and therapies utilized for joint pain were also abstracted.

Results: In the overall cohort of 246 adult CF patients, 43 (17%) had unexplained joint symptoms; 128 (52%) were female and 42 (97%) self-reported as Caucasian.  In those with unexplained arthralgia, 28 of 43 (65%) were female.  The joint symptoms were not associated with pulmonary exacerbations in 22 patients (51%).  In 15 (35%) patients, there was association between joint symptoms and pulmonary exacerbations while in 6 (14%) it was not specified.  In 18 patients, the duration of symptoms was described and 14/18 had symptoms lasting less than 6 weeks.  Knee (23), ankles (13), hips (10), and wrists (10) were the most affected joints.  However, both small and large joints were affected.  Both symmetric and asymmetric presentations were noted.  No individuals had findings of sacroiliac joint involvement.  No patients had evidence of erosions.  The most commonly used medications were NSAIDs.  In the overall cohort of CF patients who had unexplained joint symptoms, therapies utilized were NSAIDs (25, 58%), acetaminophen (10, 23%), and prednisone (9, 21%).  Hydroxychloroquine was the most frequently used DMARD (7, 16%) followed by sulfasalazine (1, 2.3%).  No biologics were used in this cohort.  There were no individuals with hypertrophic osteoarthopathy in this cohort.  There were no reports of fluoroquinolone associated tendinopathy or voriconazole induced periostitis.

Conclusion: This study characterizes the variety of joint symptoms in CF patients and expands on current knowledge. Females were most likely to experience arthralgia and the knee was the most commonly affected joint. Arthralgia was associated with pulmonary exacerbations in only 35% of patients. Understanding the diverse spectrum of arthropathy will result in greater recognition and improvement in quality of life for CF patients.

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