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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
PubMed
Summary

This case study presents a rare association between alpha-1 antitrypsin deficiency and systemic necrotizing vasculitis. The patient showed a positive response to cyclophosphamide and steroids, offering new treatment insights.

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Area of Science:

  • Rheumatology
  • Genetics
  • Nephrology

Background:

  • Polyarteritis nodosa is a systemic necrotizing vasculitis.
  • Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder that can affect the liver and lungs.
  • The PI ZZ type is the most severe form of AATD.

Observation:

  • A 42-year-old woman presented with polyarthritis, purpuric rash, mononeuritis multiplex, and focal segmental glomerulonephritis.
  • Alpha-1 antitrypsin deficiency (AATD) was incidentally diagnosed via liver biopsy.
  • The patient exhibited necrotizing arteritis, a hallmark of polyarteritis nodosa.

Findings:

  • This is the third reported case linking systemic necrotizing vasculitis with AATD of the PI ZZ type.
  • The patient demonstrated a significant positive response to treatment with cyclophosphamide and steroids.

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  • The findings suggest a potential therapeutic avenue for this rare condition.

    • Implications:

    • This case highlights the importance of considering AATD in patients with unexplained systemic necrotizing vasculitis.
    • Successful treatment with immunosuppressants offers a potential therapeutic strategy for this rare vasculitis-AATD association.
    • Further research is warranted to elucidate the pathophysiological mechanisms connecting AATD and vasculitis.