Publication details
ANCA asociované vaskulitidy v pneumologické praxi: zkušenosti jednoho centra
| Title in English | ANCA-associated vasculitis in pulmonary practice: single-center experiences |
|---|---|
| Authors | |
| Year of publication | 2026 |
| Type | Peer-reviewed scientific article |
| Magazine / Source | STUDIA PNEUMOLOGICA ET PHTHISEOLOGICA |
| MU Faculty or unit | |
| Citation | |
| web | odkaz na plný text |
| Keywords | ANCA-associated vasculitis; immunosuppressive therapy; interstitial lung disease; pulmonary involvement |
| Description | This report aims to provide a descriptive analysis of a patient cohort with ANCA-associated vasculitis, focusing on pulmonary involvement and treatment experiences within a specialized center. Materials and methods: Patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) who were followed at our center between 2018 and 2025 were included in this descriptive overview. The diagnosis was established based on a synthesis of clinical, imaging, histopathological, and laboratory findings. Histological verification was performed in 57 patients, primarily from kidney and lung biopsies. Demographic data, ANCA type, pulmonary manifestations, lung function parameters, and immunosuppressive treatment were recorded. The study had a descriptive design and was not intended as an analytical study assessing prognosis or the effect of individual treatment strategies. Results: A total of 126 patients were included (52 men, 74 women; mean age 63 years). The most common diagnosis was GPA (69; 55%), followed by MPA (29; 23%), and EGPA (28; 22%). Pulmonary involvement was present in most patients, predominantly in the form of nodules or cavitary lesions, diffuse alveolar hemorrhage (16%), interstitial lung fibrosis (8%) and tracheobronchial stenosis. Proteinase 3 and myeloperoxidase antibodies were detected in 50.8% and 28.6% of patients, respectively. Immunosuppressive treatment included corticosteroids in nearly all patients (99%), cyclophosphamide (75%), rituximab (29%), azathioprine (53%), mycophenolate mofetil (13%), and methotrexate (7%). Plasmapheresis was performed in 20% of cases. Biological treatment with mepolizumab or ben-ralizumab was administered to seven and two EGPA patients, respectively. The median follow-up time was 42 months. During the follow-up period, 15 patients died (11.9%). Conclusion: Pulmonary involvement is a common feature of the clinical presentation of ANCA-associated vasculitis; it includes a broad spectrum of findings, ranging from diffuse alveolar hemorrhage to interstitial lung disease. Our experiences suggest that these manifestations may significantly influence both diagnostic and therapeutic management. The presented cohort documents the clinical characteristics of patients and provides an overview of the treatment strategies used in multidisciplinary care at a specialized center. © 2026, Trios spol. s.r.o.. All rights reserved. |