To Örebro University

Background: Antineutrophil cytoplasmic antibody associated vasculitis (AAV), which includes Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA), and Eosinophilic Granulomatosis with Polyangiitis (EGPA) are diseases which can cause patients to experience impaired Health-Related Quality of Life (HRQoL), higher levels of anxiety, depression, and fatigue throughout life.

Objectives: The aim of this study is to explore patient reported HRQoL, anxiety, depression and fatigue among persons with AAV from different perspectives: Compare levels of HRQoL, anxiety, depression, fatigue and disease characteristics; Describe associations between disease characteristics and HRQoL, anxiety, depression and fatigue; Explore clusters of HRQoL, anxiety and depression.

Methods: This cross-sectional cohort study included adults with new and established diagnose of AAV. Anxiety and depression were assessed with Hospital Anxiety and Depression Score, e.g., HADS anxiety (HADS-A), and HADS-depression (HADS-D). HRQoL was assessed with EQ-5D-3L (EQ-5D-index), EQ-Visual analogue scale, (EQ-VAS), fatigue by Multidimensional assessment of Fatigue, (MAF). Disease activity was measured by Birmingham Vasculitis Activity Score (BVAS), scores ≥ 1 was defined as active disease. Disease duration ≤ 2 years was considered short disease duration. Mann-Whitney U-test and Kruskal Wallis H-test was used where appropriate to compare distributions between groups of patients. Bivariate correlations were analysed with Spearman's rank-order correlation. A hierarchical cluster analysis was performed, based on EQ-5D-index, EQ-VAS, HADS-A and HADS-D.

Results: 296 patients were included, equally distributed between women; 157 (53%) and men; 139 (57%), with a mean age of 59 years (SD 15.6, range 18-90). Two hundred and nine had GPA (70,6%), 75 had MPA (25,3%), and twelve had EGPA (4,1%). Mean disease duration was 4.2 years (SD 5.5, range 0-31). 55% of patients had a disease duration of ≤ 2 years, and 45% ≥ 3 years, and mean BVAS score was 6.3 (SD 8.68, range 0-33). Distributions of EQ-5D-index, EQ-VAS, HADS-A, HADS-D and MAF were similar for all three AAV diagnosis (p=0.35 - 0.69). HADS-A was higher in females, patients with active disease, and patients with short disease duration (p=0.001 - 0.013). EQ-5D-index was higher in patients with inactive disease (p=0.014). EQ-VAS was higher in patients with inactive disease, long disease duration and age > 65 years (p=0.005 - 0.017). MAF was higher in patients with active disease, short disease duration and < 65 years old (p=0.03 - 0.05). Weak associations were found among all patients between disease activity respective disease duration and EQ-VAS, MAF, HADS-A and HADS-D (rs -0.279 - 0.286). Among patients with EGPA, a moderate negative association (rs -0.736) between disease activity and EQ-5D-index, moderate positive (rs 0.579) association between disease duration and EQ-VAS, and a moderate negative association between disease duration and MAF (rs -0.699) was observed. Four clusters were identified based on EQ-5D-index, EQ-VAS, HADS-A and HADS-D. A: Low HRQoL, mild anxiety/depression, high disease activity and short disease duration. B: High HRQoL, no anxiety/depression, no disease activity and long disease duration. C: Moderate HRQoL, mild anxiety/depression low disease activity and short disease duration. D: Moderate to high HRQoL, no anxiety/depression, no disease activity and longer disease duration. All four clusters had similar distributions between women, men, and age, and the three diagnoses were represented in each of the four clusters (Table 1).

Conclusion: In this study we evaluated the impact of AAV on patients HRQoL, psychological well-being and fatigue. Disease activity, disease duration, gender and age affected HRQoL, anxiety, depression, and fatigue. In the small group of EGPA strong associations were found between HRQoL measured with EQ-index, and BVAS, as well as between HRQoL measured with EQ-VAS, fatigue and disease duration. The cluster analysis describes the impact of AAV on a person's life in different stages of the disease, which highlights the continuous need for multi professional care during the disease course. Interestingly, in this study it appears that EQ-VAS better distinguish levels of HRQoL, than EQ-5D-index, which should be further investigated.