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Multicenter Study
. 2025 Sep 1;64(9):4937-4947.
doi: 10.1093/rheumatology/keaf232.

Impact of mepolizumab on the AAV-PRO questionnaire in eosinophilic granulomatosis with polyangiitis: data from a European multicentre study

Paolo Delvino  1   2 , Luca Quartuccio  3 , Joanna C Robson  4   5 , Virginia V Ferretti  6 , Catherine Klersy  6 , Federico Alberici  7   8 , Diego Bagnasco  9 , Alvise Berti  10   11 , Marco Caminati  12   13 , Marta Camoni  7   8 , Maria C Cid  14 , Edoardo Conticini  15 , Giulia Costanzo  16 , Claire de Moreuil  17 , Stefano Del Giacco  16 , Georgina Espigol-Frigole  14 , Franco Franceschini  18   19 , Luca Iorio  20 , Anna Kernder  21   22 , Alberto Lo Gullo  23 , Laura Losappio  24 , Elena Manna  25   26 , Matteo Maule  12   13 , Alessandra Milanesi  25   26   27 , Carlomaurizio Montecucco  25   26 , Simone Negrini  28   29 , Roberto Padoan  20 , Francesca Regola  18   19 , Luisa Ricciardi  30 , Jan Schroeder  24 , Benjamin Terrier  31   32 , Paola Toniati  18 , Francesca Torracca  33 , Elena Treppo  3 , Maria Letizia Urban  34 , Augusto Vaglio  35   36 , Giacomo Emmi  37   38 , Sara Monti  39 ; European EGPA Study Group
Collaborators, Affiliations
Multicenter Study

Impact of mepolizumab on the AAV-PRO questionnaire in eosinophilic granulomatosis with polyangiitis: data from a European multicentre study

Paolo Delvino et al. Rheumatology (Oxford). .

Abstract

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.

Keywords: ANCA-associated vasculitis patient-reported outcomes questionnaire; eosinophilic granulomatosis with polyangiitis; health-related quality of life; mepolizumab; patient-reported outcomes.

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Figures

Figure 1.
Figure 1.
Comparison of the AAV-PRO domains 0–100 score at baseline. AAV-PRO: ANCA-associated vasculitis patient-reported outcomes; CAF: concerns about the future; OSS: organ-specific symptoms; PF: physical function; SEI: social and emotional impact; SS: systemic symptoms; TSE: treatment side effects
Figure 2.
Figure 2.
Temporal trend of age- and gender-adjusted log-transformed AAV-PRO domains 0–100 score during the 6-month follow-up. CAF: concerns about the future; OSS: organ-specific symptoms; PF: physical function; SEI: social and emotional impact; SS: systemic symptoms; TSE: treatment side effects
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