Patient-reported outcomes in vasculitis.

Systemic vasculitis encompasses a group of multisystem disorders; both the diseases and the treatment strategies can have a significant impact on a patient's health-related quality of life (HRQoL). Using patient-reported outcome measures (PROMs) and patient-reported experience measures (PREMs) to evaluate the patient's view of their condition, treatments, and healthcare journey is essential to the patient-centered care approach. In this paper, we discuss the use of generic, disease-specific, and treatment-specific PROMs and PREMs in systemic vasculitis and future research goals.

ANCA Associated Vasculitis Subtypes: Recent Insights and Future Perspectives.

The ANCA associated vasculitides (AAVs) affect a range of internal organs including ear nose and throat, respiratory tract, kidneys, skin and nervous system. They include granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA). The AAVs are treated with high dose glucocorticoids, immunosuppressants, and targeted biological medications. Since the 1990s classification criteria for the AAVs have been based on clinical features, laboratory tests and basic imaging; an initiative to update the classification criteria incorporating newer tests, for example, anti-neutrophil cytoplasmic antibodies (ANCA) and novel imaging techniques will be published this year. There is also evidence for classification of patients based on ANCA subtype; those with anti-proteinase 3 antibodies (PR3) or anti-myeloperoxidase antibodies (MPO) have differences in response to treatment and clinical outcomes. An update is described within this review. The pathogenesis of AAV involves necrotizing inflammation of small to medium blood vessels involving multiple immunological pathways. We present an update on emerging evidence related to auto-antibodies, complement and lymphocyte pathways. This review describes emerging treatment regimens, including evidence for plasma exchange in severe disease and the inhibitor of the complement C5a receptor (C5aR) inhibitor, Avacopan. Lastly, patient reported outcomes are key secondary outcomes in randomised controlled trials and increasingly clinical practice, we report development in disease specific and glucocorticoid-specific PROs.

Patient Perceptions of Physical Activity After a Diagnosis of Giant Cell Arteritis: Analysis of Multinational Qualitative Data.

OBJECTIVE: To explore patient perceptions of physical activity in giant cell arteritis (GCA). METHODS: This was a multinational qualitative study, analyzing interview data collected from participants from the UK (n = 25) and Australia (n = 11) with a definitive diagnosis of GCA from imaging or biopsy. Interview transcripts were analyzed using thematic analysis to identify themes related to physical activity. This was secondary analysis of data collected to explore health-related quality of life in people with GCA. RESULTS: A total of 108 individual codes pertaining to physical activity were identified. These were grouped into 2 overarching themes: barriers to and facilitators of physical activity, each with 4 subthemes. Barriers were categorized into physical symptoms (including visual loss, fatigue, weakness, pain, and stiffness), perceptions of personal capability (including poor stamina, confidence, and mobility), negative perceptions of physical activity, and negative consequences. Facilitators of physical activity were categorized into external facilitators (including motivation from health care professionals and support groups), access to appropriate facilities, personal strategies (including pacing and goal-setting), and personal facilitators (including internal motivation to improve symptoms, and positive reinforcement). CONCLUSION: A range of barriers and facilitators to physical activity were identified in relation to GCA. Future work could include development of an intervention to support physical activity in patients with GCA; ideally this intervention should be underpinned by an appropriate behavioral change framework and codesigned with patients.

Patient reported outcomes in systemic vasculitis.

PURPOSE OF REVIEW: This review paper evaluates the use of patient reported outcome (PROs) in systemic vasculitis and the increasing incorporation of these measures in the evaluation of clinical outcomes and healthcare provision. RECENT FINDINGS: Generic PROs such as the SF-12, SF-36, EQ-5D have been used to evaluate health-related quality of life (HRQOL) across the spectrum of vasculitis; including giant cell arteritis, antineutrophil cytoplasmic antibody (ANCA)-related vasculitis and immunoglobulin A vasculitis (IgA) vasculitis. More recently disease-specific PROs have been developed including the associated vasculitis (AAV)-PRO and GCA-PRO, whilst further work is ongoing including a Steroid-PRO. SUMMARY: Generic and disease-specific PROs are complimentary in nature, but the advent of disease-specific PROs allows evaluation of the impact of specific symptoms and intervention on patient HRQOL. Following the COVID-19 pandemic, the advent of increasing virtual work has brought the potential for electronic-PRO measures to the forefront and is a current area of interest.

Psychological and self-management support for people with vasculitis or connective tissue diseases: UK health professionals' perspectives.

OBJECTIVES: CTD and systemic vasculitis impact on health-related quality of life. Treatment can be complex, involving multiple medical specialities. The aim of this study was to investigate psychological and self-management support for patients in secondary care. METHODS: An online survey of health professionals in the UK, including 45 multiple-choice and free-text questions, was analysed descriptively. Free-text survey responses were analysed thematically to identify health professionals' perceptions of best practice and unmet needs. RESULTS: The online survey included 120 health professionals (34% specialist nurses, 51% doctors and 12% allied health professionals), predominantly working in rheumatology (52.9%) and nephrology (21.5%) departments. Access to self-management programmes or clinics for people with CTD or vasculitis was available in 23% of rheumatology and 8% of nephrology departments. In response to 'How well is your team providing self-management support to people with CTD or vasculitis?', 38% of respondents reported 'not very well' or 'not well at all'. Direct access to psychological support was available in 76.9% of nephrology and 32.8% of rheumatology departments. More than 80% of respondents would like additional training. Key themes from the qualitative data (free-text survey responses) included the importance of: dedicated psychological support and self-management programmes for people with CTD and vasculitis, a whole-team approach (specialist teams empowering people to manage their own care), staff training (e.g. brief psychological interventions) and signposting to resources, including patient charities. CONCLUSION: People with CTD and vasculitis have complex needs, and improvements in self-management and psychological support are required in UK rheumatology and nephrology departments.

Mechanisms determining the amelioration of rheumatoid arthritis in pregnancy: A systematic review.

BACKGROUND: The phenomenon of pregnancy-induced remission of rheumatoid arthritis (RA) was first reported by Philip Hench in 1938. Despite extensive efforts, the underlying scientific basis has remained elusive. A number of different potential mechanisms have been investigated. We have undertaken a systematic review of the available peer-reviewed articles involving pregnant patients with RA in order to establish the depth of current scientific understanding of this important topic. METHODS: This review was conducted according to guidelines of preferred reporting items for systematic reviews and meta-analyses. Studies were identified by a thorough search of multiple databases including Medline, PubMed and EMBASE. Search terms used were different combinations of the keywords: rheumatoid arthritis, inflammatory arthritis, pregnancy, mechanisms, disease activity, relapse and remission. Non-English language articles and studies that were not directly relevant were excluded. Two independent reviewers (CR and KA) screened the retrieved articles by reading the title and abstract to identify studies that addressed potential mechanisms determining RA activity in pregnancy. Articles were further refined after reading the full text. A data extraction sheet was developed for the purpose of this review and used by the independent reviewers. RESULTS: After exclusion of irrelevant, duplicate and foreign language articles, a final total of 37 original articles were identified. The largest body of literature concerned glycosylation of immunoglobulins, with 9 published articles. There is evidence of an association between increasing levels of galactosylation of immunoglobulins and reduced RA disease activity in pregnancy. Other identified articles comprised 5 on cytokine changes in pregnancy, 5 on human leucocyte antigen (HLA) incompatibility, 5 on changes in peripheral blood mononuclear cell (PBMC) gene expression; 4 on changes in corticosteroids; 3 on pregnancy associated α2-glycoprotein; 2 on changes in rheumatoid factor (RF)/anti-citrullinated protein antibody (ACPA); and 1 each on microchimerism, gamma delta T cells, regulatory T cells, and mannose-binding lectin. The results of these studies were heterogenous and occasionally conflicting. Selected studies varied greatly in terms of population size, methodology and use of controls and disease activity assessments. CONCLUSION: This systematic review has found that the cause of the pregnancy-induced amelioration of RA remains to be determined, despite extensive efforts. It is unclear which of the various transitory changes in pregnancy may be responsible for initiating downstream anti-inflammatory immunological mechanisms. We discuss limitations of the current literature and suggest areas for future study.

Retrospective Cohort Study of Venous Thromboembolism Rates in Ambulatory Cancer Patients: Association With Khorana Score and Other Risk Factors.

BACKGROUND: Guidelines do not recommend that cancer outpatients receive thromboprophylaxis unless at high venous thromboembolism (VTE) risk, with the Khorana score suggested for risk stratification. This study investigated VTE incidence in outpatients with pancreatic, endometrial, colorectal, ovarian and cervical cancer, the role of Khorana score in risk assessment and potential risk factors. METHODS: Data were retrospectively collected 1 year after cancer diagnosis. VTE associated with inpatient admissions was excluded. RESULTS: Seven hundred forty-six patients were included. VTE rates varied: 26.8% pancreatic; 5.7% endometrial; 9.8% colorectal; 10.2% ovarian; and 0.0% cervical cancer. Excluding VTE at diagnosis, potentially preventable VTE rates were 16.5% in pancreatic, 3.8% in endometrial, 9.8% in colorectal and 8.7% in ovarian cancer. Khorana score was associated with VTE in endometrial cancer only (high-risk: 16.7% vs. low-risk: 1.5%; P < 0.001). VTE rates for patients with central venous catheters (CVCs) were 22.6-34.8% in pancreatic, endometrial, colorectal and ovarian cancers. VTE was associated with CVCs in endometrial, colorectal and ovarian; chemotherapy and Hb < 100 g/L in pancreatic; surgery in endometrial and ovarian; and body mass index > 35 in ovarian cancers following adjusted analysis (P < 0.05). CONCLUSIONS: VTE is a significant burden in pancreatic, endometrial, colorectal and ovarian cancers. Khorana score was not predictive in most cancers. The major VTE-associated variable was CVC. Our data suggest a role for clinical trials of thromboprophylaxis in targeted cancer outpatients.