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.