To Örebro University

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.

OBJECTIVE: To develop evidence-based recommendations for the non-pharmacological management of systemic lupus erythematosus (SLE) and systemic sclerosis (SSc).

METHODS: A task force comprising 7 rheumatologists, 15 other healthcare professionals and 3 patients was established. Following a systematic literature review performed to inform the recommendations, statements were formulated, discussed during online meetings and graded based on risk of bias assessment, level of evidence (LoE) and strength of recommendation (SoR; scale A-D, A comprising consistent LoE 1 studies, D comprising LoE 4 or inconsistent studies), following the European Alliance of Associations for Rheumatology standard operating procedure. Level of agreement (LoA; scale 0-10, 0 denoting complete disagreement, 10 denoting complete agreement) was determined for each statement through online voting.

RESULTS: Four overarching principles and 12 recommendations were developed. These concerned common and disease-specific aspects of non-pharmacological management. SoR ranged from A to D. The mean LoA with the overarching principles and recommendations ranged from 8.4 to 9.7. Briefly, non-pharmacological management of SLE and SSc should be tailored, person-centred and participatory. It is not intended to preclude but rather complement pharmacotherapy. Patients should be offered education and support for physical exercise, smoking cessation and avoidance of cold exposure. Photoprotection and psychosocial interventions are important for SLE patients, while mouth and hand exercises are important in SSc.

CONCLUSIONS: The recommendations will guide healthcare professionals and patients towards a holistic and personalised management of SLE and SSc. Research and educational agendas were developed to address needs towards a higher evidence level, enhancement of clinician-patient communication and improved outcomes.

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.

BACKGROUND: Pain is one of the most frequently reported symptoms and often one of the first subjective symptoms in patients with systemic lupus erythematosus (SLE). A previous study indicated that most patients with SLE reported low levels of SLE-related pain. However, a subgroup of patients reported high levels of pain ≥40 mm (0-100 mm) and had a substantial symptom burden in terms of fatigue, anxiety, depression, and reduced health-related quality of life. Thus, there is a need to elucidate the implications of high levels of pain in everyday life.

AIM: This study explored the patient's experiences and implications of SLE-related pain in daily life and the support requested from healthcare providers.

METHOD: A total of 20 patients, previously reported high levels of SLE-related pain intensity measuring ≥40 mm (0-100 mm) in a research context at one or two occasions participated in individual semi-structured interviews, which were transcribed and analysed with content analysis.

RESULTS: The interviews revealed four main categories and 13 generic categories. SLE-associated pain was described by its multifaceted nature, exhibiting longstanding, unpredictable, migrating, and various physical sensations. The pain entailed multidimensional consequences, restricting everyday life by interfering with roles and relationships and causing various emotions, including existential thoughts. The informants used comprehensive strategies to deal with the pain, including their inner resources, support from family and significant others, and pharmaceuticals and relieving treatments. They expressed the need for security and acknowledgement, which involved individualized support and accessibility of healthcare.

CONCLUSION: This study provides comprehensive insights into the nature and multifaceted impact of SLE-related pain in different dimensions of the informants' daily lives. Except for medications the informants used several strategies, including their inner resources and support from family and others, to manage the pain. Support requested from healthcare providers by the informants included understanding, compassion, individualized care and accessibility.

OBJECTIVES: This scoping review aimed to explore and synthesize current literature to advance the understanding of how to design clinical reasoning (CR) curricula for students in health professions education.

METHODS: Arksey and O'Malley's 6-stage framework was applied. Peer-reviewed articles were searched in PubMed, Web of Science, CINAHL, and manual searches, resulting in the identification of 2932 studies.

RESULTS: Twenty-six articles were included on CR in medical, nursing, physical therapy, occupational therapy, midwifery, dentistry, and speech language therapy education. The results describe: features of CR curriculum design; CR theories, models, and frameworks that inform curricula; and teaching content, methods, and assessments that inform CR curricula.

CONCLUSIONS: Several CR theories, teaching, and assessment methods are integrated into CR curricula, reflecting the multidimensionality of CR among professions. Specific CR elements are addressed in several curricula; however, no all-encompassing CR curriculum design has been identified. These findings offer useful insights for educators into how CR can be taught and assessed, but they also suggest the need for further guidance on educational strategies and assessments while learners progress through an educational program.

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.

In this explorative study, we investigated if an adjunctive treatment with one single dose of the monoclonal antibody rituximab would improve symptoms and function in treatment-resistant patients with schizophrenia spectrum disorder (SSD, n = 9) or obsessive-compulsive disorder (OCD, n = 10), based on the inflammatory hypothesis for mental disorders. Patients were followed for one year. Disability was measured with the Personal and Social Performance score (PSP). At baseline, the mean PANSS score in the SSD group was 99 ± 32 and the mean Y-BOCS score in the OCD group was 27.5 ± 7. Mean PSP scores were 32 ± 10.2 and 42.5 ± 9.9 in the SSD and OCD groups, respectively. Seven had Paediatric Acute-Onset Neuropsychiatric Syndrome (PANS) in retrospect, and 3 SSD patients had schizo-obsessive subtype. 4/8 SSD patients showed a ≥40% reduction in PANSS at endpoint I week 20, however, 7/9 were similarly improved already at week 12. Among the OCD patients, 2/10 showed a ≥35% reduction in Y-BOCS at week 20. Disability was significantly improved only in the SSD group. The percentual decrease of PANSS scores in SSD patients was associated with the increase in immunoglobulin levels week 20 (n = 8: IgG r = 0.85, p = .007; IgA r = 0.79, p = .019; IgM r = 0.73, p = .038). Rituximab was generally well tolerated in these patients. Self-rated improvements since baseline were reported for psychic (p = .021), neurological (p = .059), and autonomic (p < .001) side effects (UKU-SERS-Pat side-effect scale). Anxiety was commonly reported by OCD patients, while an initial increase in psychotic symptoms was seen in a few SSD patients. An RCT is underway to evaluate rituximab in SSD.

Objectives: Knowledge and health literacy enable patients to monitor symptoms and disease impact. Educational needs have previously been explored in rheumatology, but scarcely for patients with ANCA-associated vasculitis (AAV). The aim of the study was to assess the educational needs among patients with AAV using the educational needs assessment tool (ENAT).

Methods: This was a cross-sectional observational study including adults with AAV. Educational needs were captured by ENAT. Total ENAT (0-117 points, with higher numbers indicating higher educational need) and the seven domains (managing pain, movement, feelings, disease process, treatment, self-management and support systems) were explored regarding sex, age, education, diagnosis, disease duration and disease activity. To compare domains, a percentage response (0-100%) was calculated.

Results: One hundred and seventy-eight individuals (50% men; 34% with disease duration ≤2 years) were included. The total ENAT mean was 66.5 (s.d. 26.6; 57%), with domains as follows: disease process, 78%; self-management, 69%; treatments, 64%; feelings, 56%; managing pain, 48%; support systems, 47%; and movement, 41%. Higher educational needs were found among women in the domains movement, feelings and disease process and in total ENAT (all P < 0.04) compared with men. Higher educational needs were also seen in patients with disease duration ≤2 years regarding disease process, self-management and support systems and in total ENAT compared with patients with longer disease duration (all P < 0.03).

Conclusion: This study revealed great educational needs among AAV patients. Some groups expressed higher needs (women and those with shorter disease duration). Increased education for patients with AAV might lead to improved self-care and treatment adherence.