Fortin P R, Fraser R S, Watts C S, Esdaile J M
Department of Rheumatology, Montreal General Hospital, PQ, Canada.
J Rheumatol. 1991 Oct;18(10):1613-6.
We describe a 42-year-old woman with polyarteritis nodosa characterized by polyarthritis, purpuric rash, mononeuritis multiplex, focal segmental glomerulonephritis and necrotizing arteritis. alpha-1 antitrypsin deficiency was diagnosed incidentally on a liver biopsy. This is the third reported case of systemic necrotizing vasculitis in association with alpha-1 antitrypsin deficiency of the PI ZZ type, and the first to show significant response to cyclophosphamide and steroids.
