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Development of microscopic polyangiitis-related pulmonary fibrosis in a patient with autoimmune pulmonary alveolar

Yuhei Kinehara, Hiroshi Kida1, Yoshikazu Inoue

  • 1Department of Respiratory Medicine, Allergy and Rheumatic Diseases, Osaka University Graduate School of Medicine, 2-2 Yamada-oka, Suita, Osaka 565-0871, Japan. hiroshi.kida@imed3.med.osaka-u.ac.jp.

BMC Pulmonary Medicine
|November 5, 2014
PubMed
Abstract

Insights

Autoimmune pulmonary alveolar proteinosis (aPAP) can lead to fatal fibrosis. This case suggests ANCA-associated vasculitis may drive pulmonary fibrosis in aPAP patients, warranting further research into this link.

Area of Science:

  • Pulmonology
  • Rheumatology
  • Immunology

Background:

  • Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare lung disease driven by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF).
  • Severe aPAP cases can progress to lethal respiratory failure due to pulmonary fibrosis, though its pathogenesis remains unclear.
  • Pulmonary fibrosis development in aPAP is a critical, understudied complication.

Observation:

  • A 75-year-old man with aPAP presented with characteristic high-resolution computed tomography (HRCT) findings and anti-GM-CSF antibodies.
  • Over three years, the patient developed hematuria and proteinuria, with a marked increase in myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA).
  • Follow-up imaging revealed resolution of aPAP signs and development of pulmonary fibrosis.

Findings:

  • The case demonstrates a temporal association between the development of pulmonary fibrosis and the emergence of ANCA-associated systemic vasculitis in a patient with aPAP.
  • Resolution of aPAP indicators coincided with the onset of fibrotic changes, suggesting a potential interplay between the conditions.
  • This observation highlights a possible novel pathway for pulmonary fibrosis in the context of aPAP.

Implications:

  • This report is the first to suggest a pathogenetic link between ANCA-associated systemic vasculitis and pulmonary fibrosis in aPAP.
  • Further investigation is crucial to elucidate the relationship between ANCA-associated vasculitis and aPAP-related pulmonary fibrosis.
  • Understanding this connection could lead to new diagnostic and therapeutic strategies for severe aPAP cases.

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