Vasculitis & polymyalgia rheumatica

Interstitial lung disease in patients with antineutrophil cytoplasmic antibody-associated vasculitis: chest CT patterns and correlation with survival

Semin Arthritis RheumPublished Retrospective cohort study

Authors' conclusion

The typical UIP pattern in AAV-ILD is associated with better survival, particularly in MPO-ANCA patients, suggesting distinct mechanisms for ILD development in MPO vs. PR3-AAV.

Study context

Population
Patients with AAV (MPA and GPA) and interstitial lung disease (ILD) confirmed by chest CT, 1997–2021, Mayo AAV Cohortof 1862 in Mayo AAV Cohort
Participants
143 patients
Exposure
ILD patterns on chest CT in AAV-ILD
  • Typical usual interstitial pneumonia (UIP)n = 44
  • Probable UIPn = 13
  • Indeterminate for UIPn = 37
  • Non-UIPn = 49reference
Outcome
Distribution of ILD patterns on chest CT

Main finding

As reported in the abstract

Observational data: associations, not effects.

Further findings

OutcomeIRR (ratio as printed; typical UIP related with higher survival)
Survival in MPO-ANCA patients, typical UIP vs other ILD patterns · 12 months and 10 yearsTypical UIP vs Other ILD patterns · at 12 months8.201
Survival in MPO-ANCA patients, typical UIP vs other ILD patterns · 12 months and 10 yearsTypical UIP vs Other ILD patterns · at 10 years2.179

ILD pattern by antineutrophil cytoplasmic antibody (ANCA) type

%

Typical UIP pattern most common among MPO-ANCA patients
37.4
Non-UIP pattern most common among PR3-ANCA patients
58.3

Characteristics of typical UIP vs non-UIP pattern

%

Typical UIP: male
70.5
Typical UIP: MPO-ANCA
90.0
Typical UIP: ILD diagnosed before onset of AAV
40.9
Typical UIP: reduced diffusion capacity on pulmonary function tests

Original publication

Interstitial lung disease in patients with antineutrophil cytoplasmic antibody-associated vasculitis: chest CT patterns and correlation with survival. Semin Arthritis Rheum. Published 10 April 2025. doi:10.1016/j.semarthrit.2025.152726

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