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In-Depth Analysis of Disease Manifestations in Antineutrophil Cytoplasmic Antibody–Associated Vasculitides Identifies Distinct Clinical Phenotypes

Lindberg, Hanna ; Knight, Ann ; Hellbacher, Erik ; Norling, Olof ; Berglin, Ewa ; Stegmayr, Bernd LU ; Baslund, Bo ; Palm, Øyvind ; Haukeland, Hilde and Gunnarsson, Iva , et al. (2025) In ACR Open Rheumatology 7(3).
Abstract

Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are heterogeneous disorders. The aim of this study was to identify and characterize subgroups of patients based on sex, ANCA, age at diagnosis, and organ involvement. Methods: In total, 1,167 patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) were retrospectively recruited to the study. Data including cumulative involvement of 10 different organ systems, end-stage kidney disease (ESKD), sex, proteinase (PR) 3–ANCA, myeloperoxidase (MPO)-ANCA, age at diagnosis, disease duration, and relapse were obtained from medical records. Clinical variables were analyzed for associations with sex, age at diagnosis, and relapse using... (More)

Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are heterogeneous disorders. The aim of this study was to identify and characterize subgroups of patients based on sex, ANCA, age at diagnosis, and organ involvement. Methods: In total, 1,167 patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) were retrospectively recruited to the study. Data including cumulative involvement of 10 different organ systems, end-stage kidney disease (ESKD), sex, proteinase (PR) 3–ANCA, myeloperoxidase (MPO)-ANCA, age at diagnosis, disease duration, and relapse were obtained from medical records. Clinical variables were analyzed for associations with sex, age at diagnosis, and relapse using logistic regression analysis. Thirteen clinical variables were included in hierarchical cluster analyses using the Ward method. Results: In patients with GPA, PR3-ANCA, renal and pulmonary involvement, and ESKD were significantly associated with male sex, whereas MPO-ANCA was associated with female sex. Patients with GPA who were younger than 32 years of age at diagnosis were significantly more often females and had more ear–nose–throat involvement than patients older than 32 years. In patients with MPA, female patients were significantly younger at diagnosis than male patients. Relapse was significantly associated with young age at diagnosis and pulmonary involvement in GPA and with musculoskeletal involvement in MPA. Hierarchical cluster analyses identified five and seven patient clusters among individuals with GPA and MPA, respectively. PR3-/MPO-ANCA defined the largest clusters, whereas heart, gastrointestinal, and central nervous system involvement were hallmarks for three clusters for both patients with GPA and MPA. Conclusion: Sex, age at diagnosis, and specific organ involvements define clinically relevant subgroups among patients with ANCA-associated vasculitides.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
in
ACR Open Rheumatology
volume
7
issue
3
article number
e70009
publisher
Wiley
external identifiers
  • pmid:40033657
  • scopus:86000238617
ISSN
2578-5745
DOI
10.1002/acr2.70009
language
English
LU publication?
yes
additional info
Publisher Copyright: © 2025 The Author(s). ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.
id
a25eb60d-ec15-48bc-b6d0-041a82e8e549
date added to LUP
2025-05-27 17:13:23
date last changed
2025-07-08 20:58:44
@article{a25eb60d-ec15-48bc-b6d0-041a82e8e549,
  abstract     = {{<p>Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are heterogeneous disorders. The aim of this study was to identify and characterize subgroups of patients based on sex, ANCA, age at diagnosis, and organ involvement. Methods: In total, 1,167 patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) were retrospectively recruited to the study. Data including cumulative involvement of 10 different organ systems, end-stage kidney disease (ESKD), sex, proteinase (PR) 3–ANCA, myeloperoxidase (MPO)-ANCA, age at diagnosis, disease duration, and relapse were obtained from medical records. Clinical variables were analyzed for associations with sex, age at diagnosis, and relapse using logistic regression analysis. Thirteen clinical variables were included in hierarchical cluster analyses using the Ward method. Results: In patients with GPA, PR3-ANCA, renal and pulmonary involvement, and ESKD were significantly associated with male sex, whereas MPO-ANCA was associated with female sex. Patients with GPA who were younger than 32 years of age at diagnosis were significantly more often females and had more ear–nose–throat involvement than patients older than 32 years. In patients with MPA, female patients were significantly younger at diagnosis than male patients. Relapse was significantly associated with young age at diagnosis and pulmonary involvement in GPA and with musculoskeletal involvement in MPA. Hierarchical cluster analyses identified five and seven patient clusters among individuals with GPA and MPA, respectively. PR3-/MPO-ANCA defined the largest clusters, whereas heart, gastrointestinal, and central nervous system involvement were hallmarks for three clusters for both patients with GPA and MPA. Conclusion: Sex, age at diagnosis, and specific organ involvements define clinically relevant subgroups among patients with ANCA-associated vasculitides.</p>}},
  author       = {{Lindberg, Hanna and Knight, Ann and Hellbacher, Erik and Norling, Olof and Berglin, Ewa and Stegmayr, Bernd and Baslund, Bo and Palm, Øyvind and Haukeland, Hilde and Gunnarsson, Iva and Bruchfeld, Annette and Weiner, Maria and Eriksson, Per and Segelmark, Mårten and Ohlsson, Sophie and Mohammad, Aladdin J. and Svärd, Anna and Pullerits, Rille and Herlitz, Hans and Söderbergh, Annika and Rantapää-Dahlqvist, Solbritt and Dahlqvist, Johanna}},
  issn         = {{2578-5745}},
  language     = {{eng}},
  number       = {{3}},
  publisher    = {{Wiley}},
  series       = {{ACR Open Rheumatology}},
  title        = {{In-Depth Analysis of Disease Manifestations in Antineutrophil Cytoplasmic Antibody–Associated Vasculitides Identifies Distinct Clinical Phenotypes}},
  url          = {{http://dx.doi.org/10.1002/acr2.70009}},
  doi          = {{10.1002/acr2.70009}},
  volume       = {{7}},
  year         = {{2025}},
}