Vasculitis & polymyalgia rheumatica

Interstitial lung disease in ANCA-associated vasculitis: associated factors, radiographic features and mortality

Rheumatology (Oxford)Published Retrospective cohort study

Main finding · All-cause mortalityResult — go to the resultAdjusted hazard ratio 1.58 95% CI 1.06–2.37

Authors' conclusion

ILD is a frequent complication of AAV, especially MPO-ANCA+ AAV, often preceding recognition of AAV. Fibrotic AAV-ILD is associated with a higher risk of death than AAV without ILD.

Study context

Population
PR3- or MPO-ANCA+ ANCA-associated Vasculitis (AAV) patients, 2002–2019 Mass General Brigham AAV Cohort, United StatesMGB AAV Cohort
Participants
684 participants
Exposure
AAV with interstitial lung disease (ILD) vs AAV without ILD
  • AAV-ILDn = 91
Outcome
Prevalence of ILD in AAV and association of AAV-ILD with all-cause mortality
Methodological detailsconsecutive inception cohort, Cox proportional hazard models with age as the time scale, radiologist-confirmed ILD
  • Consecutive inception cohort
  • Cox proportional hazard models with age as the time scale
  • Radiologist-confirmed ILD

Main finding

All-cause mortality

Adjusted hazard ratio

1.58

95% CI 1.06–2.37

Events by group

AAV-ILD

13%

91

Fibrotic AAV-ILDvs Without ILD
aHR 1.58 (95% CI 1.06–2.37)

As reported in the abstract

Observational data: associations, not effects.

Further findings

Age at AAV diagnosis

P < 0.001

MPO-ANCA+

P < 0.001

Age at AAV diagnosis

Distribution of sex and race similar

ILD prevalence among MPO-ANCA+ patients (n = 470) (subgroup)

Had ILD
85 (18%)

ILD radiographic features

Fibrotic ILD
76%
UIP pattern (most common)
42%

Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.

Original publication

Interstitial lung disease in ANCA-associated vasculitis: associated factors, radiographic features and mortality. Rheumatology (Oxford). Published 14 June 2022. doi:10.1093/rheumatology/keac339

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