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Antineutrophil Cytoplasmic Antibody-Associated Lung Fibrosis

Raphael Borie1,2, Bruno Crestani1,2

  • 1Assistance Publique Hôpitaux de Paris, Bichat Hospital, Rare Pulmonary Diseases Reference Center, Paris, France.

Seminars in Respiratory and Critical Care Medicine
|November 8, 2018
PubMed

Insights

Pulmonary fibrosis is linked to ANCA-associated vasculitis (AAV), often preceding diagnosis. While ANCA positivity occurs in pulmonary fibrosis, progression to AAV is infrequent, guiding treatment based on established guidelines.

Area of Science:

  • Rheumatology
  • Pulmonology
  • Immunology

Background:

  • Pulmonary fibrosis is a significant comorbidity in ANCA-associated vasculitis (AAV), particularly in Asian populations.
  • Anti-neutrophil cytoplasmic antibodies (ANCA) are typically of anti-myeloperoxidase (anti-MPO) specificity, with microscopic polyangiitis being the most common vasculitis subtype.
  • Pulmonary fibrosis can elevate mortality risk in AAV patients.

Purpose of the Study:

  • To review the relationship between pulmonary fibrosis and AAV.
  • To discuss diagnostic and treatment considerations for patients with both conditions.
  • To evaluate the significance of ANCA positivity in pulmonary fibrosis.

Main Methods:

  • Literature review of studies on pulmonary fibrosis and AAV.
  • Analysis of ANCA prevalence and specificity in pulmonary fibrosis patients.
  • Discussion of current treatment guidelines for AAV and pulmonary fibrosis.

Main Results:

  • Pulmonary fibrosis is frequently diagnosed before or concurrently with AAV.
  • A notable percentage of pulmonary fibrosis patients are ANCA-positive, but a smaller proportion develop AAV.
  • The role of anti-fibrotic drugs in AAV-related pulmonary fibrosis remains undetermined.

Conclusions:

  • Treatment for AAV in patients with lung fibrosis should adhere to international vasculitis guidelines.
  • In cases of idiopathic pulmonary fibrosis without vasculitis, ANCA detection does not alter the diagnostic pathway.
  • Anti-fibrotic therapy is recommended for idiopathic pulmonary fibrosis according to guidelines.

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