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Objectives: To prospectively evaluate the impact and the rapidity of the effect of mepolizumab on the ANCA-associated vasculitis patient-reported outcomes (AAV-PRO) questionnaire and patient global assessment (PtGA) in an international, multicentre cohort of patients with eosinophilic granulomatosis with polyangiitis (EGPA).
Methods: Patients with active EGPA initiating treatment with mepolizumab were included. PtGA and the AAV-PRO score were assessed at baseline and after 7, 14, 30, 90 and 180 days. Predictors of response of the AAV-PRO questionnaire were investigated.
Results: Seventy patients were included: female 54.3%, median age 56 years (48-65), 63 (90%) with a relapsing/refractory course. PtGA showed a statistically significant decrease within 7 days. At 14 days, all the AAV-PRO domains, except treatment side effects, showed a statistically significant decline. The improvement at 6 months was greatest in organ-specific symptoms (ratio 0.53), physical function (ratio 0.57) and PtGA (ratio 0.58). PtGA and higher disease activity positively correlated with the AAV-PRO scores throughout the study. Female patients reported a greater burden in terms of systemic symptoms, treatment side effects, social and emotional impact and concerns about the future. Conversely, age, educational level, damage accrual, mepolizumab dose and ANCA status had no effect on the AAV-PRO scores.
Conclusions: Mepolizumab was associated with a quick and remarkable improvement of health-related quality of life in patients with EGPA. These findings highlight its early and sustained benefits beyond disease control and support the integration of the AAV-PRO questionnaire into routine clinical practice.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC12407232 | PMC |
http://dx.doi.org/10.1093/rheumatology/keaf232 | DOI Listing |
Rheumatology (Oxford)
September 2025
Immunorheumatology Research Laboratory, IRCCS Istituto Auxologico Italiano, Milan, Italy.
Objectives: To prospectively evaluate the impact and the rapidity of the effect of mepolizumab on the ANCA-associated vasculitis patient-reported outcomes (AAV-PRO) questionnaire and patient global assessment (PtGA) in an international, multicentre cohort of patients with eosinophilic granulomatosis with polyangiitis (EGPA).
Methods: Patients with active EGPA initiating treatment with mepolizumab were included. PtGA and the AAV-PRO score were assessed at baseline and after 7, 14, 30, 90 and 180 days.
Background/objectives: The aim of this work is to validate the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire in a Latin American (Peru) AAV cohort.
Methods: We included patients from the Almenara vasculitis cohort who had at least 1 visit between December 2022 and June 2024. Sociodemographic features, disease activity (Birmingham Vasculitis Activity Score version 3 [BVASv3] score), damage (Vasculitis Damage Index [VDI] score), the AAV-PRO (Spanish version), and the Short Form 36 (SF-36) were obtained.
Rheumatology (Oxford)
May 2025
Otolaryngology Section, Department of Neurosciences, University of Padua, Padua, Italy.
Objectives: Eosinophilic granulomatosis with polyangiitis (EGPA) is frequently associated with refractory chronic rhinosinusitis with nasal polyps (CRSwNP), despite current treatments. Dupilumab demonstrated efficacy in the treatment of severe and uncontrolled CRSwNP, with improvements in patient-reported outcome measures (PROMs) and in objective measurements. This study aims to evaluate efficacy and safety of dupilumab in refractory CRSwNP in EGPA patients.
View Article and Find Full Text PDFRheumatol Adv Pract
January 2024
Division of Rheumatology, Department of Medicine, University of Udine, Udine, Italy.
Objectives: The primary objective of this study was the translation and validation of the ANCA-associated vasculitis patient-reported outcome (AAV-PRO) questionnaire into Italian, denoted as AAV-PRO_ita. The secondary objective was to evaluate the impact of ANCA-associated vasculitis (AAV) on quality of life (QoL) and work impairment in a large cohort of Italian patients.
Methods: The study design took a prospective cohort study approach.
Rheumatology (Oxford)
October 2024
Manchester Centre for Health Psychology, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK.
Objectives: ANCA-associated vasculitis (AAV) is associated with significant morbidity, fatigue, pain and poor health-related quality of life (HRQoL). This review aims to assess the comprehensiveness of existing patient reported outcome measures (PROMs) used in AAV and identify associations with poorer HRQoL outcomes.
Methods: A literature review of studies using PROMs, including those labelled HRQoL in people with AAV as a primary or secondary study outcome were screened and reviewed up to July 2023.