IMMUNE-COMPLEXES AND HUMORAL RESPONSE TO PSEUDOMONAS-AERUGINOSA IN CYSTIC-FIBROSIS

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Abstract
The humoral immune status of 51 patients with cystic fibrosis (CF) as compared to 25 patients with other respiratory diseases (RD) was studied. CF patients had higher serum concentrations of Ig[immunoglobulin]G and IgA (P < 0.001), C5 [complement component 5] and CH50 [total hemolytic C] (P < 0.05) than did RD patients. Of CF patients, 23% had increased IgE concentrations. Of 32 Ch and 1 RD patients colonized with mucoid P. aeruginosa (PA), 91% had serum precipitins to PA; no precipitins were found in patients not colonized with mucoid PA. Of CF patients, 51% had circulating immune complexes detected by 125I-Clq binding (for CF patients mean values .+-. SD, 14.5 .+-. 12% vs. 7.5 .+-. 3.4% for RD patients; P < 0.005). Complexes were correlated with higher serum IgA concentrations but not other Ig, C components, response to PA or pulmonary function at time of assay. Extra vascular formation of complexes was suggested by uniform absence of plasma C3 activation in vivo.