VASCULITIS AND BRONCHIECTASIS IN A PATIENT WITH ANTIBODIES TO BACTERICIDAL PERMEABILITY-INCREASING PROTEIN AND ALPHA(1)-ANTITRYPSIN DEFICIENCY/

A patient with alpha(1)-antitrypsin deficiency is reported herein; thi s subject developed aggressive bronchial disease and recurrent cutaneo us vasculitis after pulmonary infection with Pseudomonas aeruginosa. A utoantibodies to neutrophil cytoplasmic antigens were detected, which produced granular cytoplasmic staining by indirect immunofluorescence with specificity for a newly characterized antigen: bactericidal/perme ability-increasing protein (BPI). The bronchial disease and vasculitis improved, and the IgA anti-BPI titer fell after antipseudomonal treat ment. This raises the possibility that anti-BPI antibodies contributed to both the bronchial disease and vasculitis.