Wegener's granulomatosis and alpha1-antitrypsin-deficiency emphysema: proteinase-related diseases.
Wegener's granulomatosis (WG) and alpha1-antitrypsin (alpha1-AT)-deficiency emphysema are both uncommon disorders. A relationship may exist between these diseases involving the proteinase and antiproteinase balance in the lung. A case is presented of WG and alpha1-AT-deficiency emphysema occurring in the same patient. Previous studies concerning the correlation between abnormal alpha1-AT alleles and WG are discussed. Potential mechanisms for the relationship and recommendations for screening are given.
Barnett
V T
VT
Department of Medicine, University of Illinois at Chicago, 60612, USA. vtbarnet@uic.edu
Sekosan
M
M
Khurshid
A
A
D000328:Adult; D000483:Alleles; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000201:enzymology; D006801:Humans; D008168:Lung / Q000201:enzymology / Q000473:pathology; D011656:Pulmonary Emphysema / Q000150:complications* / Q000201:enzymology; D000515:alpha 1-Antitrypsin / Q000235:genetics; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications* / Q000201:enzymology
D000515:alpha 1-Antitrypsin