ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 0721

Pulmonary Manifestations of Granulomatosis with Polyangiitis and Microscopic Polyangiitis

Mats Junek1, Sara Choi2, Stephanie Garner3, Saara Rawn4, David Cuthbertson5, Curry Koening6, Carol Langford7, Carol McAlear8, Paul Monach9, Larry Moreland10, Christian Pagnoux11, Philip Seo12, Ulrich Specks13, Kenneth Warrington13, Gerard Cox1, Nader Khalidi14 and Peter Merkel8, 1St Joseph's Healthcare Hamilton, Hamilton, ON, Canada, 2Queen's University, Kingston, ON, Canada, 3University of Calgary, Calgary, AB, Canada, 4NOSM School of Medicine, Sudbury, ON, Canada, 5University of South Florida, Tampa, 6University of Texas Dell Medical School, Austin, TX, 7Cleveland Clinic, Moreland Hills, OH, 8University of Pennsylvania, Philadelphia, PA, 9VA Boston Healthcare System, Boston, MA, 10University of Colorado, Denver, CO, 11Mount Sinai Hospital, Toronto, ON, Canada, 12Johns Hopkins University, Baltimore, MD, 13Mayo Clinic, Rochester, MN, 14McMaster University, Hamilton, ON, Canada

Meeting: ACR Convergence 2025

Keywords: , , , ,

Session Information

Date: Sunday, October 26, 2025

Title: (0711–0730) Vasculitis – ANCA-Associated Poster I

Session Type: Poster Session A

Session Time: 10:30AM-12:30PM

Background/Purpose: There is limited knowledge of the epidemiology and complications of pulmonary manifestations in ANCA-associated vasculitis (AAV). This analysis focused describing the range of pulmonary disease in two forms of AAV: granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).

Methods: Patients with GPA or MPA enrolled in a prospective longitudinal cohort between April 2006 and December 2023 were included. Patients satisfied a modified ACR 1990 classification criteria for GPA or the Chapel Hill Consensus Conference definition for MPA. Comprehensive data concerning diagnosis, demographics, pulmonary manifestations at onset of disease and/or follow up, and pulmonary damage were collected in a protocolized manner.

Results: Data from 1033 individuals (865 with GPA and 105 with MPA) were included for analysis, including 4511 patient-years of follow up. The mean age at diagnosis was 51.3 years and 55.0% of patients were female. Pulmonary manifestations at any time in the disease course were seen in 70.3% of individuals with AAV: 621 (71.8%) with GPA and 105 (62.5%) with MPA (Figure 1). In GPA, 63.6% had pulmonary involvement at the time of diagnosis and an additional 11.4% at follow-up. In MPA, 56% had manifestations at diagnosis and 4.8% at follow up. Among patients with GPA with pulmonary disease, nodules/cavities were most common manifestation (62.6%); among patients with MPA with pulmonary disease diffuse alveolar hemorrhage was the most common manifestation (61%). Among patients with pulmonary involvement, 285 (45.9%) of those with GPA and 45 (42.9%) of those with MPA had more than one pulmonary manifestation at the same time. 185 (17.9%) individuals had irreversible pulmonary damage, most commonly breathlessness, loss of lung function, or interstitial lung disease (Table 1).

Conclusion: Pulmonary manifestations are common in AAV, differ in frequency between GPA and MPA, and are associated with accumulation of damage. Individuals may have multiple pulmonary manifestations concurrently, suggesting multiple pathophysiologic processes. Systematic assessments of individuals with AAV for pulmonary involvement could potentially lead to earlier detection of these manifestations and reduce pulmonary damage.

Supporting image 1Figure 1: Frequency of pulmonary manifestations in individuals with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA)

Supporting image 2Table 1: Pulmonary damage in patients with granulomatosis with polyangiitis

or microscopic polyangiitis.

Disclosures: M. Junek: AbbVie/Abbott, 1, Roche, 5; S. Choi: None; S. Garner: None; S. Rawn: None; D. Cuthbertson: None; C. Koening: Amgen, 1, Boehringer-Ingelheim, 1; C. Langford: AbbVie/Abbott, 12, Non-paid consultant, AstraZeneca, 5, 12, Non-paid consultant, Bristol-Myers Squibb(BMS), 5, 12, Non-paid consultant, GlaxoSmithKlein(GSK), 5, NS Pharma, 5; C. McAlear: None; P. Monach: Boehringer-Ingelheim, 2; L. Moreland: None; C. Pagnoux: AstraZeneca, 1, ChemoCentryx, 1, GlaxoSmithKlein(GSK), 1, 5, InflaRx GmbH, 1, otsuka, 1, 5, Pfizer, 1, 5, Roche, 1, 5; P. Seo: None; U. Specks: Amgen, 1, 2, 5, Argenx, 2, AstraZeneca, 1, 5, Boehringer-Ingelheim, 1, 2, Bristol-Myers Squibb(BMS), 5, CSL Vifor, 1, Genentech, 5, GlaxoSmithKlein(GSK), 5, Mayo Foundation for Education and Research, 9, Melodia Therapeutics, 2, NorthStar Medical Radioisotopes, 5, Novartis, 1, 2, 5, NS Pharma, 5, UpToDate, 9; K. Warrington: Bristol-Myers Squibb(BMS), 5, Sanofi, 2; G. Cox: None; N. Khalidi: AbbVie/Abbott, 5, Bristol-Myers Squibb(BMS), 5, GlaxoSmithKlein(GSK), 6, Mallinckrodt, 6, NS Pharma, 5, Otsuka, 6, Roche, 2, Sanofi, 5; P. Merkel: AbbVie, 2, 5, Alpine, 2, Amgen, 2, 5, ArGenx, 2, AstraZeneca, 2, 5, Boehringer-Ingelheim, 2, 5, Bristol-Myers Squibb (BMS), 2, 5, CSL Behring, 2, Eicos, 5, Electra, 5, GlaxoSmithKlein (GSK), 2, 5, iCell, 2, Interius, 2, Kinevant, 2, Kyverna, 2, 11, Lifordi, 11, Metagenomia, 2, Neutrolis, 2, 5, 11, Novartis, 2, NS Pharma, 2, Otsuka, 2, Q32, 2, 11, Quell, 2, Regeneron, 2, Sanofi, 2, Sparrow, 2, 11, Takeda, 2, 5, UpToDate, 9, Vistera, 2.

To cite this abstract in AMA style:

Junek M, Choi S, Garner S, Rawn S, Cuthbertson D, Koening C, Langford C, McAlear C, Monach P, Moreland L, Pagnoux C, Seo P, Specks U, Warrington K, Cox G, Khalidi N, Merkel P. Pulmonary Manifestations of Granulomatosis with Polyangiitis and Microscopic Polyangiitis [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/pulmonary-manifestations-of-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis/. Accessed .

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