Clinical features of usual interstitial pneumonia with anti-neutrophil cytoplasmic antibody in comparison with idiopathic pulmonary fibrosis

Respirology. 2016 Jul;21(5):920-6. doi: 10.1111/resp.12763. Epub 2016 Mar 19.


Background and objective: Myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) is occasionally positive in patients with usual interstitial pneumonia (UIP). However, the differences from idiopathic pulmonary fibrosis (IPF/UIP) have not been well documented. We aimed to clarify the clinical, radiological and pathological features of UIP associated with MPO-ANCA (ANCA/UIP).

Methods: We retrospectively reviewed the medical records of 12 consecutive ANCA/UIP patients not manifesting microscopic polyangiitis and 108 IPF/UIP patients with no autoantibodies, both diagnosed by surgical lung biopsy.

Results: There was no significant difference in clinical background, laboratory results and pulmonary function tests between ANCA/UIP patients and IPF/UIP patients except for the percentage of bronchoalveolar lavage neutrophils. HRCT showed subpleural reticulation in both groups. Increased attenuation around honeycombing and cysts was significantly observed in ANCA/UIP. Pathologically, ANCA/UIP had more prominent inflammatory cell infiltration, lymphoid follicles with germinal centres and cellular bronchiolitis. During the disease course, three of 12 patients (25%) developed microscopic polyangiitis. Immunosuppressive treatment tended to be more effective in ANCA/UIP patients, and the survival time in ANCA/UIP patients tended to be longer than those with IPF/UIP.

Conclusion: ANCA/UIP may be distinguishable from IPF/UIP with a combination of HRCT findings of increased attenuation around honeycombing and cysts and some of the characteristic pathological findings. In contrast to IPF/UIP, immunosuppressive treatment could be a therapeutic option for ANCA/UIP.

Keywords: idiopathic pulmonary fibrosis; immunosuppressive treatment; myeloperoxidase anti-neutrophil cytoplasmic antibody; usual interstitial pneumonia.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / analysis*
  • Biopsy / methods
  • Female
  • Humans
  • Idiopathic Pulmonary Fibrosis* / diagnosis
  • Idiopathic Pulmonary Fibrosis* / immunology
  • Idiopathic Pulmonary Fibrosis* / physiopathology
  • Idiopathic Pulmonary Fibrosis* / therapy
  • Immunosuppressive Agents / therapeutic use*
  • Japan / epidemiology
  • Lung* / diagnostic imaging
  • Lung* / immunology
  • Lung* / pathology
  • Male
  • Middle Aged
  • Peroxidase / analysis
  • Pulmonary Fibrosis* / diagnosis
  • Pulmonary Fibrosis* / immunology
  • Pulmonary Fibrosis* / physiopathology
  • Pulmonary Fibrosis* / therapy
  • Respiratory Function Tests / methods
  • Retrospective Studies
  • Survival Analysis
  • Tomography, X-Ray Computed / methods


  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Peroxidase