Background/Purpose: ANCA-associated vasculitis (AAV) is a chronic autoimmune condition that significantly impacts patients’ quality of life. It is unknown whether this impact varies based on age at diagnosis, from childhood through older adulthood. This study examined the relationship between age at diagnosis of AAV and health-related quality of life (HRQoL).

Methods: The data source was the Vasculitis Patient-Powered Research Network, an online, longitudinal, prospective cohort of adults with AAV (granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis) enrolled from 2014-2024. Cohorts were stratified by age (years) at diagnosis: children (< 18), young adults (18–40), middle-aged adults (41–65), and older adults ( > 65). Descriptive statistics were summarized. Differences were tested via Student’s t-tests or Kruskal-Wallis tests for continuous variables and Pearson’s chi squared tests or Fisher’s exact tests for categorical variables. Multivariable linear regressions assessed associations between age at diagnosis and Patient-Reported Outcomes Measurement Information System (PROMIS) results at last follow-up visit. Models were adjusted for gender and age at HRQoL assessment.

Results: Analysis included 2009 patients with AAV. The majority had GPA and there was a female predominance that decreased with age at diagnosis. Those diagnosed at younger ages had longer follow-up at time of last PROMIS measure (Table 1). All cohorts had impaired HRQoL in every domain (Figure 1). When adjusted for gender and age at HRQoL assessment, those diagnosed with AAV in childhood had worse HRQoL related to anxiety than those diagnosed as young, middle-aged, or older adults. Additionally, those diagnosed in childhood had better HRQoL related to fatigue, sleep disturbance, depression, pain interference, satisfaction with participation in social roles, and physical functioning (Table 2).

Conclusion: Patients with AAV have impaired HRQoL, especially related to anxiety. Additionally, age at diagnosis with AAV is associated with differences in HRQoL. Compared to patients diagnosed with AAV at older ages, those diagnosed at younger ages have worse HRQoL related to anxiety and better HRQoL related to fatigue, sleep disturbance, depression, pain interference, satisfaction with participation in social roles, and physical functioning. Understanding the mechanisms by which age at diagnosis impacts HRQoL will be useful for developing targeted clinical interventions in AAV.

Figure 1. Patient-Reported Outcomes Measurement Information System T-Scores Based on Age at Diagnosis with ANCA-associated vasculitis. Boxplot of the distribution of Patient-Reported Outcomes Measurement Information System T-Scores stratified by age at diagnosis with ANCA-associated vasculitis. The boxes represent the 25th to 75th percentile T scores, with the middle line within each box representing the median T score. The solid lines extending out signify the range with dots representing possible outliers. The dashed gray line represents the mean score of the referent general pediatric population (50), with one standard deviation above and below represented in gray. Better health-related quality of life is represented by a lower score in the top five measures and a higher score in the bottom two measures. Statistical significance indicates that the median T score for at least one age at diagnosis group is different from another age at diagnosis group within that measure. *statistical significance at p < 0.05; **statistical significance at p < 0.01.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-association-between-age-at-diagnosis-and-health-related-quality-of-life-in-anca-associated-vasculitis/