IMMUNE-COMPLEXES AND COMPLEMENT ABNORMALITIES IN PATIENTS WITH CYSTIC-FIBROSIS - INCREASED MORTALITY ASSOCIATED WITH CIRCULATING IMMUNE-COMPLEXES AND DECREASED FUNCTION OF THE ALTERNATIVE COMPLEMENT PATHWAY

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Abstract
Serum samples from 139 patients with cystic fibrosis (CF) were tested for complement abnormalities and circulating immune complexes (CIC). We found no consistent changes in whole complement activity. However, we found CIC in 29% of these patients and decreased activity of the alternative complement pathway (ACP) in 36%. During 5 yr of observation, mortality was much higher in patients whose sera contained CIC (p < 0.001) or decreased ACP activity (p < 0.01). Of patients with both abnormalities, 31% died; however, no deaths occurred in patients with normal ACP activity and negative tests for CIC (p < 0.001). During a subsequent 2.5-yr, 55% of patients .gtoreq. 21 yr old with both findings died. In contrast, no deaths occurred in older patients lacking this combination (p = 0.0062). Circulating immune complexes but not decreased ACP activity were an independent risk factor for death. Our findings support the hypothesis that humoral immune mechanisms may contribute to morbidity and mortality in CF.

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