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[Pulmonary fibrosis as a presentation of Wegener's granulomatosis]

W Ketata1, S Msaad, H Gharsalli

  • 1Service de Pneumo-allergologie, CHU Hédi Chaker, Sfax, Tunisie. wajdi.ketata@yahoo.fr

Revue Des Maladies Respiratoires
|December 3, 2009
PubMed
Abstract

Insights

Pulmonary fibrosis is a rare manifestation of antineutrophil cytoplasmic antibody-associated vasculitis. This case highlights interstitial lung disease in Wegener's granulomatosis, emphasizing its infrequent occurrence.

Area of Science:

  • Rheumatology and Pulmonology
  • Immunologically mediated lung diseases

Background:

  • Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) rarely presents with pulmonary fibrosis.
  • Microscopic polyangiitis is the most common AAV subtype associated with interstitial lung disease.
  • Wegener's granulomatosis (now Granulomatosis with Polyangiitis) is an uncommon cause of pulmonary fibrosis in AAV.

Observation:

  • An 82-year-old male presented with symptoms of pulmonary fibrosis.
  • Computed tomography (CT) revealed honeycombing and traction bronchiectasis, predominantly in the lung periphery and bases.
  • Diagnosis of Wegener's granulomatosis was confirmed by positive ANCA, urinalysis abnormalities, and renal biopsy.

Findings:

  • The case demonstrates interstitial fibrosis as an unusual pulmonary manifestation of AAV.
  • The patient's presentation underscores the heterogeneity of lung involvement in ANCA-associated vasculitis.
  • Diagnostic confirmation involved a multi-system approach, including serological, urinary, and renal pathology findings.

Implications:

  • Highlights the importance of considering AAV in patients with unexplained pulmonary fibrosis, even with atypical presentations.
  • Contributes to understanding the pathogenesis of interstitial lung disease in granulomatosis with polyangiitis.
  • Suggests a need for comprehensive diagnostic workups in suspected cases of AAV-related lung disease.

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