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(Chest. 1999;116:253-255.)
© 1999 American College of Chest Physicians

Wegener's Granulomatosis and {alpha}1-Antitrypsin-Deficiency Emphysema*

Proteinase-Related Diseases

V. Theodore Barnett, MD, FCCP; Marin Sekosan, MD and Abid Khurshid, MD, FCCP

* From the Section of Respiratory and Critical Care Medicine, Department of Medicine (Drs. Barnett and Khurshid), and Department of Pathology (Dr. Sekosan), University of Illinois at Chicago, Chicago, IL.

Correspondence to: V. Theodore Barnett, MD, FCCP, Section of Respiratory and Critical Care, Department of Medicine (MC 787), University of Illinois, 840 S Wood St, Chicago, IL 60612; e-mail: vtbarnet{at}uic.edu

Wegener's granulomatosis (WG) and {alpha}1-antitrypsin ({alpha}1-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 {alpha}1-AT-deficiency emphysema occurring in the same patient. Previous studies concerning the correlation between abnormal {alpha}1-AT alleles and WG are discussed. Potential mechanisms for the relationship and recommendations for screening are given.

Key Words: {alpha}1-antitrypsin deficiency • antibodies, antineutrophil cytoplasmic • emphysema • Wegener's granulomatosis






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