Female gender and steroid treatment as main influencing factors in the perception of quality of life in anca-associated vasculitis: final results from the italian version of anca-associated vasculitis patient-reported outcome (aav-pro-ita) questionnaire.

Abstract

Background: The ANCA-associated vasculitis patient-reported outcome (AAVPRO) questionnaire is a 29-item disease-specific PRO measure for AAV [1]. Originally only available in English, it has been translated into several languages in recent years to spread its use. The Italian version of the AAV-PRO questionnaire (AAV-PRO-ita) was translated in collaboration with Oxford and Bristol University (UK) and, after a preliminarily test on a single-center Italian cohort [2], it was evaluated on a large Italian cohort [3]. Objective(s): To assess the internal consistency, feasibility, and reliability of the AAV-PRO-ita in the multicentric cohort of Italian AAV patients belonging to the Vasculitis Study Group of the Italian Society of Rheumatology. Method(s): The AAV-PRO-ita is describes the following disease domains: (1) organ-specific and systemic symptoms and signs(SSS); (2) physical function( PF); (3)social and emotional impact(SEI). In this study, Italian-speaking AAV patients were recruited from Italian Centres (N=17) with a large experience in the diagnosis and treatment of systemic vasculitis, belonging to the Vasculitis Study Group of the SIR. Inclusion criteria were: a confirmed diagnosis of GPA, MPA, or EGPA; ANCA positivity in at least once occasion or biopsy-proven AAV; and age >=18 years old. Participants completed the AAV-PRO-ita at three different time-points: baseline, after 5-7 days, and at month 3. Result(s): 276 AAV-patients (56.5% women) with a median age of 61 (IQR 51.5-71.6) were recruited and completed the questionnaires. The subtype of AAV was mainly GPA (146, 52.9%), followed by EGPA (77, 27.9%), and MPA (53, 19.2%). Median BVASv3 at baseline was 0 (IQR 0-3), whereas the median BVASv3 at the onset of disease was 13 (IQR 8-18). Participants had a median duration of disease of 62 (IQR 23.8-118.5) months. Patients who experienced at least one relapse, one hospitalization, and one severe infection were 41.7%, 53.3%, and 22.1%, respectively. 81.2% of the patients were on immunosuppressant therapy and 72.1% were still receiving GCs. AAV-PRO-ita questionnaire had good internal consistency (Cronbach's Alpha range 0.81-0.93) and good test-retest reliability (ICCs range 0.93-0.96). Item response rates were high overall (maximum 1.8% missing data), supporting the feasibility of the questionnaire. Concerning the domains of the questionnaire, female AAV patients scored higher (i.e. worse) in all 3 domains, especially in the SEI domain (p-value<0.001). There were also differences between patients on steroid therapy (n=199) and patients without steroid therapy (n=77), in fact, the former had higher scores on all domains, especially in the PF domain (p-value<0.001). Furthermore, patients who had at least one relapse of disease (n=114) had higher scores compared with who had never had one (n=161) on all domains (p-value<0.05). Differently, there were no differences in median scores between younger and older responders and between AAV subtypes. Duration of disease, previous hospitalizations and/or infections, BVASv3 and VDI at baseline did not influence the scores. Conclusion(s): The AAV-PRO-ita questionnaire is a new 29-item, disease-specific PRO measure for use in AAV in the Italian language. It is a self-administered Italian questionnaire with good internal consistency, feasibility, and reliability. Research on treatment strategies based on steroid-sparing regimen and spreading awareness of the existence of gender differences in medicine may improve the perceived QoL of AAV patients, reducing the psychosocial impact of the chronic disease.