# Granulomatosis with Polyangiitis (GPA) in a Polish Tertiary Centre (2010–2025): Sex-Stratified Phenotypes, Serology, and Evolving Treatment Patterns

**Authors:** Aleksandra Hus, Małgorzata Wisłowska, Krzysztof Bonek

PMC · DOI: 10.3390/jcm14217884 · Journal of Clinical Medicine · 2025-11-06

## TL;DR

This study examines GPA in Poland, highlighting common symptoms, treatment changes, and sex-based differences over 15 years.

## Contribution

The paper provides real-world, sex-stratified insights into GPA management and evolving treatment trends in a Polish tertiary center.

## Key findings

- ENT disease was the most common manifestation, with rhinosinusitis affecting 76% of patients.
- Kidney involvement was frequent but variable, with 22% experiencing rapidly progressive kidney disease.
- Treatment shifted toward rituximab and steroid minimisation, with tofacitinib emerging as a potential maintenance therapy.

## Abstract

Background/Objectives: GPA is a PR3-ANCA–predominant small vessel vasculitis with organ involvement. Real-world, single-centre data are needed to interpret evolving therapies and phenotype patterns in national conditions. Material and Methods: Retrospective cohort study of consecutive GPA patients managed at the National Institute of Geriatrics, Rheumatology and Rehabilitation (Warsaw, Poland) from 1 September 2010 to 1 September 2025. Data included demographics, phenotype, BVAS, organ involvement, PR3/MPO-ANCA serology, and induction/maintenance therapies. Results: Fifty patients were included (54.0% men). Mean age was 52.5 years; mean BMI was 26.15 kg/m2. Ear-nose-throat (ENT) disease was frequent: rhinosinusitis 76.0%, nasal cartilage destruction 64.0%, subglottic stenosis 34.0%. Pulmonary nodules occurred in 52.0%, cavitation in 28.0%, and diffuse alveolar haemorrhage in 34.0%. Renal involvement included haematuria in 42.0%, chronic kidney disease (CKD) in 32.0%, and rapidly progressive kidney disease in 22.0%. Orbital inflammation was 36.0%, and PR3-ANCA was positive in 70.0%. All patients received glucocorticoids for induction; cyclophosphamide 28/50 (56.0%), rituximab 6/50 (12.0%), and mycophenolate with methotrexate 6/50 (32%). Maintenance therapy included methotrexate (78.0%), mycophenolate (64.0%), rituximab (52.0%), and azathioprine (12.0%). Conclusions: This Polish single-centre cohort shows an ear-nose-throat-lung-kidney (ELK)-dominant, PR3-predominant GPA phenotype and frequent but variable kidney involvement. Over 2010–2025, practice changed toward rituximab-based strategies, steroid minimisation, selective use of plasma exchange, and early avacopan uptake, with tofacitinib for maintenance therapy as a possible new therapeutic option.

## Linked entities

- **Diseases:** Granulomatosis with Polyangiitis (MONDO:0012105), chronic kidney disease (MONDO:0005300)

## Full-text entities

- **Genes:** MPO (myeloperoxidase) [NCBI Gene 4353], PRTN3 (proteinase 3) [NCBI Gene 5657] {aka ACPA, AGP7, C-ANCA, CANCA, MBN, MBT}
- **Diseases:** small vessel vasculitis (MESH:C565222), kidney disease (MESH:D007674), CKD (MESH:D051436), Orbital inflammation (MESH:D007249), Ear-nose-throat (ENT) disease (MESH:D004427), rhinosinusitis (MESH:D000092562), nodules (MESH:D016606), Renal involvement (MESH:C565423), subglottic stenosis (MESH:D007829), nasal cartilage destruction (MESH:C562753), ANCA (MESH:D056648), GPA (MESH:D014890), haemorrhage (MESH:D006470)
- **Chemicals:** methotrexate (MESH:D008727), cyclophosphamide (MESH:D003520), azathioprine (MESH:D001379), tofacitinib (MESH:C479163), avacopan (MESH:C000620232), rituximab (MESH:D000069283), steroid (MESH:D013256), mycophenolate (MESH:D009173)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## References

18 references — full list in the complete paper: https://tomesphere.com/paper/PMC12607949/full.md

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Source: https://tomesphere.com/paper/PMC12607949