Vasculitis & polymyalgia rheumatica

Pulmonary manifestations of granulomatosis with polyangiitis and microscopic polyangiitis

Semin Arthritis RheumPublished Retrospective cohort study

Authors' conclusion

Pulmonary manifestations are common in AAV and are associated with permanent damage.

Study context

Population
Individuals with ANCA-associated vasculitis (AAV) followed between 2006 and 2023
Participants
1,026 individuals
Exposure
Granulomatosis with polyangiitis (GPA) vs microscopic polyangiitis (MPA)
  • GPAn = 860
  • MPAn = 166
Outcome
Pulmonary manifestations at any time in the disease course
Methodological detailsmulticentre, longitudinal
  • Multicentre
  • Longitudinal

Main finding

As reported in the abstract

Pulmonary manifestations at any time in disease course
Overall cohort
81.3%

Observational data: associations, not effects.

Further findings

Pulmonary manifestations at disease onset

Overall cohort
644 (62.7%)

Pulmonary manifestations during follow-up

Overall cohort
456 (44.4%)

Most common manifestations

Nodules and cavities, diffuse alveolar hemorrhage, and inflammatory infiltrates were the three most common across the cohort
Nodules and cavities most frequent in GPA; diffuse alveolar hemorrhage most frequent in MPA

Multiple pulmonary manifestations, among those with pulmonary manifestations

Multiple pulmonary manifestations
51.9%

Pulmonary manifestations in relapse episodes

Episodes of relapse with pulmonary manifestations
41.4%

Safety

Pulmonary damage among those with pulmonary manifestations

Sustained pulmonary damage
17.4%

Original publication

Pulmonary manifestations of granulomatosis with polyangiitis and microscopic polyangiitis. Semin Arthritis Rheum. Published 16 February 2026. doi:10.1016/j.semarthrit.2026.152949

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