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. The aim of this study was 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 diagnosis 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), and 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 median age of 63 years (range: 18–90, IQR: 50–70). Two hundred and nine had GPA (70,6%), 75 had MPA (25,3%), and twelve had EGPA (4,1%). Their median BVAS score was 1 (range: 0–33, IQR: 0–12), and 46% had an inactive disease. Median disease duration was 2 years (range: 0–31, IQR: 0–6), and 55% had a disease duration of ≤ 2 years. The median current dose of prednisolone was 5 mg (range: 0–80, IQR: 0–12.5).

HRQoL, measured with EQ-VAS, was higher in patients < 65 years old, those with inactive disease, and those with long disease duration (≥3 years) (p = 0.005–0.017), while EQ-5D-3L only varied with disease activity (p = 0.014). Women, patients with active disease, and patients with shorter disease duration reported more anxiety (p = 0.001–0.03), and younger persons with active disease and short disease duration reported more fatigue (p = 0.002–0.03).

In the total group, disease activity and disease duration were both associated with HRQoL, anxiety, depression, and fatigue (rs -0.279 – 0.286). Among patients with EGPA, a moderate association (rs -0.736) between disease activity and EQ-5D-index, moderate association (rs 0.579) between disease duration and EQ-VAS, and a moderate association (rs -0.699) between disease duration and MAF was observed.

Four clusters based on EQ-5D index, EQ-VAS, and HADS were identified, containing patients with various levels of HRQoL and psychological distress across the disease course. The four clusters were denoted A-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. The four clusters had similar distributions between women, men, and age, and the three diagnoses were represented in each of the four clusters and were distinctive separated by EQ-VAS.

Conclusion: The impact of AAV on HRQoL, anxiety, depression, and fatigue, is persistent and varies depending on disease activity, disease duration, gender, and age. Associations between disease activity, disease duration and HRQOL, anxiety, depression, and fatigue were present in all diagnostic subgroups of AAV. Four clusters revealed the ongoing impact of AAV, emphasizing the continuous need for multiprofessional support during the disease course. In this study, EQ-VAS was better able to effectively distinguish levels of HRQoL than EQ-5D-index.