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Microscopic polyangiitis associated with diffuse panbronchiolitis.

Jangchul Park1, Shogo Banno, Yoshiki Sugiura

  • 1Department of Internal Medicine and Molecular Science, Nagoya City University Graduate School of Medical Science, Nagoya, Aichi.

Internal Medicine (Tokyo, Japan)
|June 1, 2004
PubMed
Summary
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This case report highlights a patient with diffuse panbronchiolitis (DPB) who developed ANCA-associated vasculitis. Chronic bacterial infection may contribute to the development of this autoimmune condition.

Area of Science:

  • Immunology
  • Pulmonology
  • Nephrology

Background:

  • Systemic vasculitis is occasionally reported in patients with chronic suppurative lung diseases.
  • Diffuse panbronchiolitis (DPB) is a chronic inflammatory airway disease.
  • Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) encompasses several autoimmune conditions.

Observation:

  • A 46-year-old female with a history of DPB presented with hemoptysis and dyspnea.
  • Laboratory tests revealed positive myeloperoxidase-ANCA (MPO-ANCA) and BPI-ANCA titers.
  • Imaging and renal biopsy confirmed pulmonary hemorrhage and necrotizing crescentic glomerulonephritis.

Findings:

  • The patient was diagnosed with microscopic polyangiitis (MPA), a subtype of AAV, co-occurring with DPB.

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  • Treatment involved methylprednisolone pulse therapy and intravenous cyclophosphamide.
  • The case suggests a potential link between chronic bacterial infections and AAV pathogenesis.

    • Implications:

    • This case underscores the importance of considering AAV in patients with DPB and respiratory symptoms.
    • Chronic bacterial infections in the lungs may trigger or exacerbate autoimmune responses in susceptible individuals.
    • Further research is warranted to elucidate the role of microbial triggers in the pathogenesis of AAV.