Wegener's granulomatosis (WG) and alpha(1)-antitrypsin (alpha(1)-AT)-defici
ency emphysema are both uncommon disorders. A relationship may exist betwee
n these diseases involving the proteinase and antiproteinase balance in the
lung. A case is presented of WC and alpha(1)-AT-deficiency emphysema occur
ring in the same patient. Previous studies concerning the correlation betwe
en abnormal alpha(1)-AT alleles and WG are discussed. Potential mechanisms
for the relationship and recommendations for screening are given.