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.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Adult
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Biopsy
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Cyclophosphamide / therapeutic use
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Female
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Glomerulosclerosis, Focal Segmental / complications
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Humans
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Liver / pathology
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Necrosis / complications
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Neuritis / complications
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Polyarteritis Nodosa / complications
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Steroids / therapeutic use
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Vasculitis / complications
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Vasculitis / drug therapy
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Vasculitis / metabolism*
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alpha 1-Antitrypsin Deficiency*
Substances
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Steroids
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Cyclophosphamide