Interstital lung disease in ANCA vasculitis

Autoimmun Rev. 2017 Jul;16(7):722-729. doi: 10.1016/j.autrev.2017.05.008. Epub 2017 May 4.


Anti-neutrophil cytoplasmic antibodies (ANCA) vasculitides are immune-mediated disorders that primarily affect small blood vessels of the airway and kidneys. Lung involvement, one of the hallmarks of microscopic polyangiitis and granulomatosis with polyangiitis, is associated with increased mortality and morbidity. In recent years, several retrospective series and case reports have described the association of interstitial lung disease (ILD) and ANCA vasculitis, particularly those positive for ANCA specific for myeloperoxidase. In the majority of these patients pulmonary fibrosis occurs concurrently or predates the diagnosis of ANCA vasculitis. More importantly, these studies have shown that ILD has an adverse impact on the long-term prognosis of ANCA vasculitis. This review focuses on the main clinical and radiologic features of pulmonary fibrosis associated with anti-neutrophil cytoplasmic antibodies. Major histopathology features, prognosis and therapeutic options are summarized.

Keywords: ANCA; Interstitial lung disease; Microscopic polyangiitis; Pulmonary fibrosis.

Publication types

  • Review

MeSH terms

  • Animals
  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Humans
  • Lung / diagnostic imaging
  • Lung / immunology
  • Lung / pathology
  • Lung Diseases, Interstitial / diagnostic imaging
  • Lung Diseases, Interstitial / epidemiology*
  • Lung Diseases, Interstitial / immunology
  • Lung Diseases, Interstitial / pathology
  • Pulmonary Fibrosis / diagnostic imaging
  • Pulmonary Fibrosis / epidemiology*
  • Pulmonary Fibrosis / immunology
  • Pulmonary Fibrosis / pathology
  • Tomography, X-Ray Computed
  • Vasculitis / diagnostic imaging
  • Vasculitis / epidemiology*
  • Vasculitis / immunology
  • Vasculitis / pathology


  • Antibodies, Antineutrophil Cytoplasmic