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We report the clinical and laboratory characteristics of eight patients with cystic fibrosis (CF) and arthropathy. Five patients had frequently relapsing, episodic arthritis; one had chronic arthritis with high titers of rheumatoid factors that could not be distinguished from juvenile chronic arthritis. In two patients the arthropathy did not relapse. Forty-six patients with CF but without arthropathy, seen during the same 1-year period, served as control subjects. Results of lung function tests and sputum cultures were similar in both groups. The sera of six of the eight patients with CF-arthropathy had high levels of circulating immune complexes, measured both by C1q-binding and the indirect granulocyte phagocytosis test, whereas only 1 of 12 control patients tested had positive C1q binding (p < 0.023). Serum complement levels were normal in both groups. Synovial biopsies, performed in three patients, showed only scanty lymphocytic infiltrates; immunofluorescent staining showed deposits of IgM, IgG, and components of complement C1q, C3, and C4. These findings suggest that the arthropathy found in CF is an immune-mediated phenomenon.
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