To test the pathogenetic role of the phenotype MZ of alpha 1-antitrypsin/alpha 1-protease inhibitor (PI) in acute anterior uveitis (AAU) and in different rheumatic diseases we examined 360 unrelated patients including 93 with AAU alone, 24 patients with AAU and ankylosing spondylitis (AS), 21 patients with AAU and Reiter's disease (RD), 26 patients with AAU, AS, and RD 54 patients with AS alone, 16 patients with RD alone, 115 patients with rheumatoid arthritis (RA) alone, and 11 patients with psoriatic arthritis (PA) alone. Of the 164 AAU patients, 80 had a single attack, and 84 had repeated episodes. There were neither significant differences between different groups of the patients and 120 healthy controls nor between patients with AAU alone and patients with AAU and AS or RD in the frequencies of the PI phenotypes tested. The results indicate that the PI MZ type is not closely associated with AAU, AS, RD, RA and PA and that it does not play any role in determining whether AAU shows a pattern of a single attack or repeated episodes, and whether AAU occurs alone or together with AS or RD.