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.

OBJECTIVES: To explore health-related quality of life (HRQoL), anxiety, depression and fatigue among persons with ANCA-associated vasculitis (AAV).

METHODS: In this cross-sectional study, patients were assessed with the EuroQoL five-dimension three-level questionnaire (EQ-5D-3L) and visual analogue scale (EQ-VAS), the Hospital Anxiety and Depression Score (HADS), and the Multidimensional Assessment of Fatigue questionnaire (MAF).

RESULTS: HRQoL measured with EQ-VAS was higher in younger patients, those with inactive disease (BVAS = 0), and those with long disease duration (≥3 years), while EQ-5D-3L only varied with disease activity. Women, patients with active disease, and patients with shorter disease duration reported more anxiety, and younger persons with active disease and short disease duration reported more fatigue. In the total group, disease activity and disease duration were both associated with HRQoL, anxiety, depression and fatigue. Four clusters based on EQ-5D, EQ-5D index, and HADS were identified, containing patients with various levels of HRQoL and psychological distress across the disease course.

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

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.

Background: Patients with antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), which includes Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA), and Eosinophilic Granulomatosis with Polyangiitis (EGPA), can experience a substantial disease burden with lower Health-Related Quality of Life (HRQoL), higher levels of anxiety, depression and fatigue [1]. Several earlier studies have investigated the extent of patient experiences among two or three patient groups, however, only a few has studied any differences in between the three different AAV patient groups.

Objectives: The aim of this study is to analyse HRQoL, fatigue, anxiety and depression in patients with AAV and to identify any differences between patients with GPA, MPA, and EGPA.

Methods: This is a cross sectional study, consecutively including both newly diagnosed and current adult patients with AAV. For this study, the EQ Visual analogue scale (EQ-VAS, scale 0-100), and the first question in MAF (scale 1-10), assessing degree of fatigue were included in the analysis, besides the two dimensions of HADS anxiety (HADS-A, scale 0-21), and HADS-depression (HADS-D, scale 0-21). Disease activity was measured by Birmingham Vasculitis Activity Score (BVAS). BVAS scores of zero was considered as inactive disease, whereas a BVAS scores ≥ 1 was defined as active disease.

Patient reported outcomes (PROM) were analysed by Kruskal Wallis H test for differences between the tree diagnosis, and Mann-Whitney U test for differences between two groups, e.g short versus long disease duration. Additional bivariate correlations between PROMS and disease durations for patient groups were analysed with Spearman’s rank-order correlation.

Results: Three hundred and fifty-nine patients were included in the analysis, GPA n=250 (70%), MPA n=95 (27%), and EGPA n=14 (4%). In all, the AAV patients were equally distributed between men; 172 (48%) and women; 187 (52%), with a mean age of 59 years (SD 16.3, range 18-90). Forty-two percent of patients had a BVAS of zero, and 58 % a BVAS of ≥ 1. Mean disease duration was 3.7 years (SD 5.3, range 0-31). Sixty percent of patients had a disease duration of ≤ 2 years, and 40% ≥ 3 years.

Distributions of HRQoL, fatigue, anxiety and depression scores were similar for all AAV groups. HRQoL, fatigue, anxiety and depression scores for groups GPA, EGPA, MPA were not statistically significantly different (Table 1).

Patients with disease duration < 2 years compared to disease duration ≥ 3 years reported lower HRQoL (median 60, IQR 47-75, vs median 70, IQR 50-80, p= 0.005), higher fatigue (median 7, IQR 5-8 vs median 7, IQR 4-8, p= 0.014) and depression (median 6, IQR 3-9 vs median 4, IQR 2-7, p= 0.004). No significant differences were found for anxiety (median 4, IQR 2-7, vs median 3, IQR 1-6, p= 0.080), between short and long disease duration.

Significant, however weak associations were found between disease duration and HRQoL, fatigue, anxiety and depression in all patients (rs <0.29) and the subgroups GPA (rs < 0.30) and MPA (rs <0.31). In the small group of EGPA, disease duration had stronger associations than GPA and MPA, for disease duration and all explored PROMs (rs < 0.61) (Table 2.).

Conclusion: No significant differences in HRQoL, fatigue, anxiety and depression median scores were found between the three patient groups GPA, MPA, and EGPA. In all patients, a weak correlation was found between disease duration and HRQoL, fatigue, anxiety and depression. However, the group with EGPA had a stronger association between disease duration and both HRQoL and fatigue, which indicates some differences between diagnoses, that should be further explored. Additionally, patients with disease duration < 2 years reported worse HRQoL, fatigue, and depression which indicate that patients with AAV need special attention during the first two years after diagnosis.

OBJECTIVE: The aim was to describe how the patient perspective is captured in clinical research on ANCA-associated vasculitis (AAV).

METHODS: This integrative review included 2149 publications found in four different databases and manual searches. After screening, 156 articles remained. All articles were sorted and categorized, and 77 original articles were analysed further.

RESULTS: The patient perspective was captured with patient-reported outcome measures (PROMs), single-item questionnaires, project-specific questionnaires and interviews. The most common aspects measured were health-related quality of life, anxiety and depression, and fatigue, and the least common were lifestyle habits, relationships and self-management.

CONCLUSION: The patient perspective was captured predominantly with generic PROMs and occasionally with a qualitative approach. AVV is a lifelong disease, and the results from this review show that not all aspects of importance to patients are covered with the PROMs used in research. Future studies should include the areas that are the most important for patients.