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Alpha-1 antitrypsin deficiency and systemic necrotizing vasculitis
P R Fortin1, R S Fraser, C S Watts
1Department of Rheumatology, Montreal General Hospital, PQ, Canada.
The Journal of Rheumatology
|October 1, 1991
Abstract:
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.