British Journal of Rheumatology 1992;31:535-538 CYSTIC FIBROSIS ARTHRITIS. A REPORT OF FIVE CASES

In a retrospective study of 206 patients with cystic fibrosis (CF), five cases of CF arthritis were recorded. This is a frequency of 2.5 % and of 4.5 % in patients aged over 10. Four patients had episodic arthritis, which was related to the course of pul-monary disease in two cases. In three patients, synovial fluid examination revealed minimal evidence of inflammation. In one of these three cases, synovial biopsy revealed a mild and non-specific synovitis. The fifth patient had chronic arthro-pathy and was positive for rheumatoid factor, but did not fulfil the criteria for rheumatoid arthritis. There were no radio-graphic abnormalities in any of these cases. CF arthritis is a rare syndrome of unknown pathogenesis. KEY WORDS: Cystic fibrosis arthritis, Synovitis, Episodic arthritis. CYSTIC fibrosis (CF) is characterized by a generalized exocrinopathy and is the most frequent of the severe genetic diseases in white European populations. The gene responsible has recently been identified on chro-mosome 7 [1]. Recurrent pulmonary infection fol-lowed by chronic bronchopulmonary colonization with Pseudomonas aeruginosa (PA) play a major role in the development of chronic obstructive pulmonar