Immunochemical quantitation of 18 proteins in sera from patients with cystic fibrosis: concentrations correlated to class of fibroblast metachromasia, clinical and radiological lung symptoms.

Sera from 32 patients with cystic fibrosis classified according to fibroblast metachromasia 
into three classes have been analysed immunochemically for the concentrations of 
18 serum proteins. In addition, the clinical and radiological status of 
the lungs were evaluated, using two scoring systems. Eleven of 16 
serum proteins differed from the values of a thousand-donor serum pool. 
The most pronounced differences from the control sera were elevated acute-phase 
proteins, notably haptoglobin, alpha1-antichymotrypsin, IgG, and IgA, and low serum albumin 
and alpha2-HS-glycoprotein. Patients from metachromatic class III presented the most pronounced 
rise in acute-phase proteins and, furthermore, significantly decreased complement C3 and 
IgM, IgG and IgA were normal in class III patients. These 
differences from the control sera possibly reflect inflammatory tissue damage involving 
immune reactions. The clinical score was positively correlated to the radiologic 
score, and both these scores were positively correlated to the concentrations 
of eight and seven, respectively, of the serum proteins, and negatively 
correlated to three of the serum proteins. Based upon the correlations 
between the scores and the concentrations of some of the serum 
proteins, it is recommended that longitudinal prospective studies follow individual patients 
for the concentrations in serum of haptoglobin, albumin, IgA, IgG, C3, 
and, eventually, IgM and alpha1-antichymotrypsin. The present results suggest that these 
serum proteins will yield valuable information as regards prognosis and therapy 
of the lung disease in CF. 