Alpha-1 antitrypsin deficiency and systemic necrotizing vasculitis.
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.
Department of Rheumatology, Montreal General Hospital, PQ, Canada.
P R
PR
Fortin
R S
RS
Fraser
C S
CS
Watts
J M
JM
Esdaile
D000328:Adult; D001706:Biopsy; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications; D006801:Humans; D008099:Liver / Q000473:pathology; D009336:Necrosis / Q000150:complications; D009443:Neuritis / Q000150:complications; D010488:Polyarteritis Nodosa / Q000150:complications; D013256:Steroids / Q000627:therapeutic use; D014657:Vasculitis / Q000150:complications / Q000188:drug therapy / Q000378:metabolism*; D019896:alpha 1-Antitrypsin Deficiency
D013256:Steroids; D003520:Cyclophosphamide