Glomerulonephritis as late manifestation of severe alpha 1-antitrypsin deficiency.
An association of alpha 1-antitrypsin deficiency with glomerulonephritis is rare and has so far been observed only in children or young adults. We report a 63-year-old man with severe alpha 1-antitrypsin deficiency associated with pulmonary emphysema, cirrhosis of the liver, and mesangioproliferative glomerulonephritis with nephrotic syndrome. Following initial presentation with nephropathy, further work-up revealed alpha 1-antitrypsin deficiency of proteinase inhibitor Z. In the absence of glomerular alpha 1-antitrypsin deposits the relationship between renal disease and alpha 1-antitrypsin deficiency remains unclear. alpha 1-Antitrypsin deficiency should be considered in adults with abnormal renal function and chronic liver disease.
Stauber
R E
RE
Medizinische Universitätsklinik, Karl-Franzens Universität, Graz, Austria.
Horina
J H
JH
Trauner
M
M
Krejs
G J
GJ
Ratschek
M
M
Klimpfinger
M
M
D017809:Fatal Outcome; D005922:Glomerulonephritis, IGA / Q000209:etiology*; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology; D008173:Lung Diseases, Obstructive / Q000150:complications; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D011656:Pulmonary Emphysema / Q000209:etiology; D012907:Smoking / Q000009:adverse effects; D019896:alpha 1-Antitrypsin Deficiency