[Early childhood oligoarthritis (antinuclear antibody positive) in alpha 1-antitrypsin deficiency of the PiIZ type].

A 15 months old girl with gonarthritis, and positive antinuclear autoantibodies was found to have a genetically determined alpha 1-antitrypsin deficiency of the rare protease inhibitory (Pi) type IZ. The possible significance of a disturbed equilibrium of cellular inflammation-promoting proteases and extracellular protease inhibition for the pathogenesis of chronic arthritis is discussed using as illustration the presented case.