Vasculitis & polymyalgia rheumatica

Progression to antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in patients with idiopathic interstitial pneumonia and positive ANCA

Semin Arthritis RheumPublished Retrospective cohort study

Authors' conclusion

Approximately 10 % of ANCA-positive IIP patients developed AAV after an IIP diagnosis. Anti-MPO or co-occurring positive RF poses a significant risk for subsequent AAV occurrence.

Study context

Population
Patients with idiopathic interstitial pneumonia (IIP) and positive ANCA without AAV, single tertiary centre, South Korea, January 2013 to August 2023
Participants
154 patients
Exposure
Patients who developed AAV vs those who did not
Outcome
Development of AAV after IIP diagnosis and its risk factors
Methodological detailssingle-centre, tertiary centre, Cox regression, Kaplan-Meier analysis
  • Single-centre
  • Tertiary centre
  • Cox regression
  • Kaplan-Meier analysis

Main finding

As reported in the abstract

Development of AAV

Proportion of ANCA-positive IIP patients who eventually developed AAV

10.4 %

Observational data: associations, not effects.

Further findings

RF positivity

P = 0.007

OutcomeAAVNon-AAVP value
Anti-MPO positivityAll48.8 %P < 0.001

Baseline characteristics (sex, age, smoking status, urinalysis, chest CT findings)

No significant difference between AAV and non-AAV groups

Risk factors for AAV occurrence (multivariate Cox regression and Kaplan-Meier)

Rheumatoid factor (RF) positivity, HR (P = 0.004)
4.02
Anti-MPO positivity, HR (P < 0.001)
38.10

Original publication

Progression to ANCA-associated vasculitis in patients with idiopathic interstitial pneumonia and positive ANCA. Semin Arthritis Rheum. Published 30 May 2024. doi:10.1016/j.semarthrit.2024.152484

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