Background/Purpose:

Arthritis in cystic fibrosis (CF) can be very incapacitating, and it is mainly of three types: CF arthritis, hypertrophic osteoarthropathy, and arthritis due to co-existent conditions and drug reactions [1]. Episodic arthritis occurring in patients with cystic fibrosis has been recognised [2]. However, although joint manifestations are common in children with cystic fibrosis (CF), they have received little attention in adults [3]. The prevalence of joint pain in the CF patients has been reported variously as:

 12.9% [4], 2-8.5% of patients [3] and 4.5% in patients aged over 10 [5]. Our experience suggested that the prevalence may be higher than that previously reported.

Methods:

Rheumatic symptoms and signs of 55 adult patients with CF (age 18 to 63 years) were determined by questionnaire and phone interview. The questionnaire was designed to elicit the pattern of joint problems, as well as to determine features of definite inflammatory problems which included joint swelling, difficulty with function (e.g. making a fist) and morning stiffness lasting greater than 1 hour.

Results:

63.6% (35/55) reported ever having experienced joint pain, but only 37.1% of those who reported experiencing pain at any point said that it had affected important activities in their lives. 16.4% (9/55) reported more than one hour of morning stiffness in their joints. 30.9% (17/55) reported joint swelling or difficulty making a fist.

In those that reported pain, 60% (21/35) reported wrist pain, with 42.9% (9/21) of these patients  reporting wrist swelling. 16.4% (9/55) of the total cohort had experienced both wrist pain and swelling.

In those that reported pain, 57.1% (20/35) reported hand pain, with 60% (12/20) reporting swelling and 65% (13/20) having difficulty making a fist. Inflammatory symptoms (difficulty making a fist or hand swelling) were reported in 80% (16/20) of those with hand pain.

Knee 80% (28/35), ankle 60% (21/35) and foot 31.4% (11/35) pain were cited by those reporting pain. 

Symmetrical disease in time was reported in 48.6% (17/35) patients.

Conclusion:

The prevalence of joint symptoms by self-report in our CF cohort far exceed that reported in previous studies. The age of the cohort would suggest that joint pain is being reported more commonly in patients with CF than in subjects of similar age who do not have CF.

The reported symptoms of swelling and morning stiffness suggest that the problems may be inflammatory in up to 30% of patients with CF.

No definite pattern of involvement could be identified in this study.

References:

1.            Parasa, R.B. and N. Maffulli, Musculoskeletal involvement in cystic fibrosis. Bulletin-Hospital for Joint Diseases, 1999. 58(1): p. 37-44.

2.            Johnson, S. and A. Knox, Arthropathy in cystic fibrosis. Respiratory medicine, 1994. 88(8): p. 567-570.

3.            Botton, E., et al., Musculoskeletal manifestations in cystic fibrosis. Joint Bone Spine, 2003. 70(5): p. 327-335.

4.            Koch, A.-K., et al., Musculoskeletal manifestations and rheumatic symptoms in patients with cystic fibrosis (CF)—no observations of CF-specific arthropathy. The Journal of Rheumatology, 2008. 35(9): p. 1882-1891.

5.            Pertuiset, E., et al., Cystic fibrosis arthritis. A report of five cases. Rheumatology, 1992. 31(8): p. 535-538.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-prevalence-and-pattern-of-self-reported-joint-symptoms-in-cystic-fibrosis/