Vasculitis & polymyalgia rheumatica

Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in interstitial lung disease: a retrospective cohort study

Rheumatology (Oxford)Published Retrospective cohort study

Authors' conclusion

The prevalence of ANCAs in patients with ILD is not rare, and ANCA testing in ILD patients is necessary. Oddly shaped cysts with or without a UIP pattern may be a characteristic chest imaging manifestation of ANCA-positive ILDs.

Study context

Population
Chinese patients with interstitial lung disease (ILD) (including idiopathic pulmonary fibrosis) with available ANCA results, First Affiliated Hospital of Ningbo University, March 2010 to October 2023
Participants
706 patients
Exposure
ANCA-positive ILD vs ANCA-negative ILD
  • ANCA-POSITIVE ILDn = 158
Outcome
Prevalence of ANCAs in ILD
Methodological detailssingle-centre
  • Single-centre

Main finding

As reported in the abstract

ANCA positivity in ILD

ANCA-POSITIVE ILD

22.4%

158

Observational data: associations, not effects.

Further findings

All-cause mortality

P = 0.033

OutcomeANCA-POSITIVE ILDANCA-NEGATIVE ILD
ANCA positivity after ILD diagnosis · on average (41.6 ± 31.4) months after ILD diagnosis58.2%— not stated in the abstract

— not stated in the abstract

Honeycombing on chest CT · significantly greater in ANCA-positive ILD
P < 0.001
Oddly shaped cysts on chest CT · significantly greater in ANCA-positive ILD
P < 0.001

Clinical features, ANCA-positive vs ANCA-negative ILD

ANCA-positive ILD patients tended to be older, had higher CRP and ESR levels, and a significantly greater proportion of RF positivity

Usual interstitial pneumonia (UIP) pattern (most common chest HRCT pattern)

UIP pattern
56.3%

Survival in ANCA-positive ILD (independent factors)

Acute exacerbation of ILD, shorter survival: HR
2.40 (95% CI 1.37-4.22, P = 0.002)
GC combined with immunosuppressants, longer survival: HR
0.30 (95% CI 0.16-0.57, P < 0.001)

Original publication

Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in interstitial lung disease: a retrospective cohort study. Rheumatology (Oxford). Published 25 February 2025. doi:10.1093/rheumatology/keaf108

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