Alpha-1-antitrypsin deficiency and nephropathy

Glomerulonephritis occurring in association with alpha(1)-antitrypsin deficiency has been sporadically reported in the literature but it is assumed to be a rare and poorly investigated disease. The complete pathologic pattern of glomerular lesions has not yet been established. The aim of our work was to investigate the correlation between the extent of antiprotease deficiency and the expression of nephropathy evaluated in two groups of patients (47 heterozygotic subjects with the PiMZ phenotype and 12 homozygotic subjects with the PiZZ phenotype) by a noninvasive approach with urinalyses and proteinuria measurement. No statistical differences between proteinuria in the two groups were observed suggesting that nephropathy is not a direct and single expression of the protein deficiency.