Management of fatigue with physical activity and behavioural change support in vasculitis: a feasibility study.

OBJECTIVE: Patients with ANCA-associated vasculitis (AAV) experience high levels of fatigue, despite disease remission. This study assessed the feasibility and acceptability of a definitive randomized controlled trial of a behavioural-based physical activity intervention to support fatigue self-management in AAV patients. METHODS: AAV patients in disease remission with fatigue (Multidimensional Fatigue Inventory-20 general fatigue domain ≥14) were randomly allocated to intervention or standard care in this single-centre open-label randomized controlled feasibility study. The intervention lasted 12 weeks and comprised eight face-to-face physical activity sessions with a facilitator and 12 weekly telephone calls. Participants were encouraged to monitor their physical activity using a tracker device (Fitbit). Standard care involved sign-posting to fatigue websites. The primary outcome was feasibility of a phase III trial assessed against three stop/go traffic light criteria, (recruitment, intervention adherence and study withdrawal). A qualitative study assessed participant views about the intervention. RESULTS: A total of 248 patients were screened and 134 were eligible to participate (54%). Stop/go criteria were amber for recruitment; 43/134 (32%, 95% CI: 24, 40) eligible participants randomized, amber for adherence; 73% of participants attended all eight physical activity sessions, but only 11/22 (50%, 95% CI: 29, 71%) completed the intervention as per the intended schedule, and green for study withdrawal; 2/43 participants withdrew before 24 weeks (5%, 95% CI: 0, 11). Qualitative results suggested the intervention was acceptable. CONCLUSION: This study suggests a behavioural-based physical activity intervention targeting fatigue self-management was acceptable to patients with AAV, although recruitment and protocol adherence will need modification prior to a definitive trial. CLINICAL TRIAL REGISTRATION NUMBER: ISRCTN11929227.

Cognitive deficits in vasculitis of the nervous system: a cross-sectional study.

Objectives: To identify the cognitive and functional deficits in a well-characterized group of patients with vasculitis of the nervous system. Methods: Sixty-seven patients diagnosed with Central Nervous System (CNS) or Peripheral nervous System (PNS) vasculitis over a 14-year period were retrospectively identified. Data on clinical presentation, laboratory, radiographic and tissue biopsy investigations, and treatment were collated. Cognitive, functional and quality of life evaluation assessments were performed in 31 patients who agreed to participate and included Addenbrooke's Cognitive Examination-revised (ACE-R), Nottingham Extended Activities of Daily Living (NEADL) and EQ-5D-3L quality of life questionnaires. Results: CNS vasculitis patients exhibited cognitive impairment, with a mean ACE-R score of 74/100 (standard deviation (SD) 16). NEADL and EQ-5D-3L scores were in the impaired range at 41/66 (SD 21) and 57/81 (SD 22), respectively. Patients with just PNS vasculitis exhibited fewer cognitive deficits with ACE-R and NEADL scores of 87 (SD 8) and 46 (SD 16) respectively. EQ-5D-3L score was in the impaired range of 65 (SD 22). Conclusions: Vasculitis of the nervous system and, in particular, CNS vasculitis causes cognitive impairment and deficits in functional ability. Such patients should be targeted for cognitive rehabilitation.

[Applying Swanson's Theory of Caring to ManagePowerlessness in an Older Patient With Vasculitis].

This case report describes a nursing care experience that applied Swanson's Caring Theory to a powerless elderly patient who was suffering from vasculitis problems that had made her incapable of walking and had delayed her discharge from the hospital. The associated health problems, including impaired physical mobility, infection, and powerlessness, were affirmed after an integrated physical, psychological, social, and spiritual assessment was conducted during the nursing care period, which lasted between April 26th and May 5th, 2018. The five categories (knowing, being with, doing for, enabling, maintaining belief) of Swanson's Caring Theory were applied. The patient was encouraged to participate in the treatment plan and motivated to participate in rehabilitation. In addition, the caring capacity and care confidence of family members was enhanced in order to support the patient's further care after discharge. Furthermore, religious support was involved, which allowed the patient to communicate her emotions, reduce her stress, and achieve spiritual comfort. By providing care in the realms of physiology, psychology, and spirit, the author helped the patient regain her self-worth and enhance her positive beliefs in the future. This case report is hoped to provide medical staffs with a reference for facilitating the recovery of elderly patients experiencing disease-related powerlessness.

Psychiatric and functional neuroimaging abnormalities in chronic hepatitis C virus patients: Is vasculitis a contributing factor?

BACKGROUND AND STUDY AIMS: Central nervous system (CNS) involvement in hepatitis C virus (HCV) infection has different facets such as anxiety, depression, cognitive impairment and vasculitis. We were interested in detecting subclinical CNS involvement in chronic HCV infected subjects with and without systemic vasculitis. PATIENTS AND METHODS: Nineteen patients (15 females and 4 males) with chronic HCV infection (mean age 46.5 ±â€¯7 and mean duration since diagnosis of HCV infection 4.7 ±â€¯4 years, including 6 (32%) Child-Pugh class A cirrhotic patients) and 30 age, sex and education matched healthy control subjects were studied. Thirteen patients had associated vasculitis. Patients and control subjects were assessed using the block design and comprehension subtests of Wechsler Bellevue Adult Intelligence Scale, Wechsler Memory scale (WMS), Beck Anxiety Inventory (BAI) and Beck Depression Inventory (BDI). Brain HMPAO Single Photon Emission Computed Tomography (SPECT) was performed for HCV patients. RESULTS: Patients with HCV had lower scores on the block design test compared to control subjects (8.37 ±â€¯1.89 versus 10.37 ±â€¯1.47, p < 0.001), lower total WMS scores (43.15 ±â€¯10.49 versus 60.27 ±â€¯8.08, p < 0.001) and higher anxiety and depression scores (16.94 ±â€¯10.46 and 37.17 ±â€¯10.38 versus 10.3 ±â€¯4.67 and 28.9 ±â€¯5.99, p = 0.004 and 0.001, respectively). Total WMS were lower in HCV patients with vasculitis compared to those without vasculitis (39.14 ±â€¯9.3 versus 51.17 ±â€¯8.3, p = 0.019) while the block design and comprehension tests, BAI and BDI were not significantly different between both groups. The block design and comprehension tests, WMS, BAI and BDI were not significantly different between cirrhotic and non-cirrhotic patients. Seven patients had different patterns of cerebral hypoperfusion on SPECT, and all of them had associated vasculitis. Abnormal SPECT was associated with lower total WMS scores (35.87 ±â€¯10.8 versus 46.79 ±â€¯8.6 in those with normal SPECT, p = 0.049). CONCLUSIONS: Vasculitis may contribute to the development of neuropsychiatric involvement in HCV patients.

The role of pain, perseverative cognition and goal adjustment in vasculitis-associated fatigue.

Fatigue is a common symptom associated with vasculitis and contributes significantly to impaired quality of life. Motivational control theory suggests a role for perseverative cognition and goal adjustment in fatigue. Therefore, this study investigated these potential predictors of fatigue in individuals with vasculitis. A total of 249 participants completed online questionnaires assessing fatigue, perseverative cognition, goal disengagement and goal reengagement, in addition to demographic and disease-related variables. Hierarchical regression analysis found only pain, sleep disturbance, disease activity and perseverative cognition to significantly predict fatigue. This highlights the importance of psychological factors in determining fatigue in those with vasculitis.

What matters for patients with vasculitis?

Advances in clinical care for patients with vasculitis have improved survival rates and created new challenges related to the ongoing management of chronic disease. Lack of curative therapies, burden of disease, treatment-related side effects, and fear of relapse contribute to patient-perceived reduction in quality of life. Patient-held beliefs about disease and priorities may differ substantially from the beliefs of their health care providers, and research paradigms are shifting to reflect more emphasis on understanding vasculitis from the patient's perspective. Efforts are ongoing to develop disease outcome measures in vasculitis that better represent the patient experience. Health care providers who care for patients with vasculitis should be sensitive to the substantial burdens of disease commonly experienced by patients living with the disease and should strive to provide comprehensive care directed towards the medical and biopsychological needs of these patients.

The invisible part of the iceberg: qualitative aspects of childhood vasculitis.

OBJECTIVES: The aim of this study was to develop a multidimensional assessment instrument named 'Juvenile Vasculitis Multidimensional Assessment Report' (J-VAMAR) to measure all the domains of the vasculitis. In this qualitative study, it is primarily aimed to enrich the item generation for the J-VAMAR. METHODS: Twelve children with vasculitis and their mothers (n=12) were enrolled in this study. The data were collected using both a demographic data form and a semi-structured interview form. The study was performed on individual patient face-to face interview. Data were analysed by grounded theory and the N Vivo 9 software program. RESULTS: Four categories were obtained. These categories were (i) physical effects of the illness, (ii) emotional effects of the illness, (iii) social effects of the illness and (iv) experienced challenges related to treatment process. In the physical effect category severe pain, physical limitations, weakness and fatigue; in emotional effect category thought of death, hopelessness and dissatisfaction about body image; in the social effects category decrease in academic performance, absenteeism to school and concealing the sickness from friends were the most common features. In the fourth category, subjects complained of lifelong drug use and frequency of daily drug consumptions. CONCLUSIONS: These results provide evidence-based data for the assessment of children with vasculitis by several domains including physical, emotional and social aspects as well as treatment protocols. The study provides the basis and/or justification for selecting the domains that the developing multidimensional instrument should include.

Quality of life and outcome measures in vasculitis.

The primary vasculitides are a life- and organ-threatening set of diseases with a course often marked by alternating periods of active vasculitis and remission. As opposed to clinical trials within the fields of cardiology and oncology, where treatment interventions have been tested in a controlled fashion using hard 'end' points, such as mortality and hospitalisation, surrogate 'end' points have to be used in randomised clinical trials (RCTs) in vasculitis. Given the multisystem nature of the vasculitides, their heterogeneous clinical presentations and rarity, outcome-measure development is a challenging task. The objective of this review is to summarise the data on how health-related quality of life is affected by vasculitis, to describe the currently used outcome measures and provide insight into future outcome-measures development. The primary focus is on anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, where the most data exist and outcome measure development is farthest along. Data on other vasculitides will also be briefly discussed.

Predictors of medication non-adherence for vasculitis patients.

The primary purpose of this article is to document whether demographic, clinical, regimen-related, intrapersonal, and interpersonal factors predict medication non-adherence for vasculitis patients. A secondary purpose is to explore whether adherence varies by medication type and whether patients experienced drug-related side effects. Vasculitis patients (n = 228) completed online baseline and 3-month follow-up surveys. Demographic (age, gender, education, race, marital status, and insurance status), clinical (perceived vasculitis severity, disease duration, vasculitis type, and relapse/remission status), regimen-related (experience of side effects), intrapersonal (depressive symptoms), and interpersonal (adherence-related support from family and friends) factors were measured at baseline. Medication non-adherence was assessed at follow-up using the Vasculitis Self-Management Survey medication adherence subscale (α = 0.89). Variables that significantly correlated (p < 0.05) with non-adherence were included in a linear regression model to predict non-adherence. Younger age (r = -0.23, p < 0.001), female sex (r = 0.16, p < 0.05), experience of side effects (r = 0.15, p < 0.05), and more depressive symptoms (r = 0.22, p < 0.001) were associated with more medication non-adherence. In the regression model, younger age (ß = -0.01, p = 0.01) and more depressive symptoms (ß = 0.01 p = 0.02) predicted worse adherence. For six out of eight vasculitis medication types, patients who experienced side effects were less adherent than patients who did not experience side effects. Multiple factors are associated with medication non-adherence for vasculitis patients. Providers should discuss medication adherence and drug-related side effects with vasculitis patients. Providers may want to particularly target younger patients and patients with clinical signs of depression.

Health-related quality of life in severe cryoglobulinaemic vasculitis and improvement after B-cell depleting therapy.

OBJECTIVES: To study the health-related quality of life (HRQOL) in severe cryoglobulinaemic vasculitis (CV) associated with hepatitis C virus infection (HCV) and to describe the effect of rituximab on HRQOL. METHODS: HRQOL was evaluated with the Medical Outcomes Study Short Form 36 (SF-36). Health Survey questionnaire was submitted to 15 patients with severe CV. SF-36 questionnaire was evaluated at baseline and after rituximab. Physical Health Composite Summary (PCS) and Mental Health Composite Summary (MCS) scores were calculated according to standard protocols, and normalised to healthy controls. SF-36 summary scores were compared with those of HCV positive patients without CV, and other vasculitis published in the literature. European Quality of Life-5 dimensions (EQ5D) scores were also derived. RESULTS: Physical and mental domain scores were all reduced if compared with those of the healthy population, with physical domains being greatly affected. HRQOL of CV was comparable with HRQOL reported for the other small vessel vasculitis. The development of CV in HCV positive patients worsened PCS rather than MCS score. Birmingham Vasculitis Activity Score (BVAS) did not correlate with HRQOL, while the presence of peripheral neuropathy was associated with a worse HRQOL. Early rituximab treatment improved both PCS and MCS scores, with long-term effects. CONCLUSIONS: PCS rather than MCS was affected in HCV positive patients when CV is present. Rituximab improved both physical and mental domains, thus supporting its use before antiviral therapy in severe HCV-related CV. The cost/benefits ratio of a sequential therapy may be supported.

Ameliorative potential of montelukast on ischemia-reperfusion injury induced vasculitic neuropathic pain in rat.

AIMS: Ischemia-reperfusion (I/R) event in vascular and nervous system has been documented to rising ischemic and vasculitic neuropathic pain, clinically resembles the complex regional pain syndrome (CRPS). The present study evaluated the effect of montelukast, a cysteinyl leukotriene receptor (Cys-LTC(4) and Cys-LTD(4)) antagonist on ischemia -reperfusion (I/R) induced vasculitic neuropathic pain in rats. MAIN METHODS: Behavioral parameters were assessed at different time intervals (i.e. 0, 1, 7, 14 and 21st day) and biochemical analysis in sciatic nerve tissue samples were also performed along with histopathological studies. KEY FINDINGS: Behavioral pain assessment has shown increase in paw and tail withdrawal threshold in montelukast treated groups against thermal and mechanical stimuli as compared to I/R control group. We observed a decrease in the total calcium, thiobarbituric acid reactive substance (TBARS) and myeloperoxidase (MPO) activity levels, whereas there is rise in reduced glutathione level in montelukast treated groups as compared to I/R control group. However, significant behavioral and biochemical results were observed only in medium and high dose of treated groups which were comparable to normal control group. Moreover, histopathological study has revealed the reduction of I/R induced neuronal edema and axonal degeneration due to montelukast. SIGNIFICANCE: Montelukast has ameliorated I/R induced vasculitic neuropathic pain, these effects may be due to inhibition of lipid peroxidation, reduction of oxidative stress, release of inflammatory mediators and neuroprotective actions. Hence, it could be used as a novel therapeutic agent for the management of vasculitic inflammation related neuropathic pain.

The impact of vasculitis on patients' social participation and friendships.

OBJECTIVES: Our objective is to explore how vasculitis affects patients' friendships and social participation. METHODS: Vasculitis patients (n=221) completed an online questionnaire that asked if, and how, relationships with friends have changed since receiving a vasculitis diagnosis. Participants' written responses were imported into Atlas.ti, and two independent researchers used both structured and unstructured coding to identify themes. After reaching 100% consensus on the themes present in each participant's responses, the coders determined how themes were interrelated across participants. RESULTS: Over half of patients (52%) expressed that vasculitis negatively impacted their friendships and 25% noted a negative impact on their social participation. At times, this negative impact was related to structural changes in patients' social networks due to loss of friendships. Reduced social participation was also associated with friends' inability to understand vasculitis and its effects, vasculitis-related fatigue, and lifestyle changes such as not being able to drink alcohol and avoiding infection-prone events. Additionally, patients withdrew from social engagements due to fatigue or because of physical symptoms and side effects. CONCLUSIONS: The unique circumstances associated with a rare chronic illness like vasculitis can create significant barriers to friendships, including loss of these relationships. Interventions designed to help patients cope with the social impact of vasculitis are implicated, especially if they increase patients' ability to engage in dialogue about their illness with their friends.

Patients with rare diseases using pharmacists for medication information.

OBJECTIVES: To determine whether patients with a rare illness (1) use pharmacists for medication information more or less frequently than physicians and the Internet, (2) perceive pharmacists as a more or less credible medical information resource than physicians and the Internet, and (3) obtain different types of medication information from pharmacists, physicians, and the Internet. DESIGN: Cross-sectional survey. SETTING: Online data collected between 2008 and 2009. PATIENTS: Adult, English-proficient vasculitis patients (n = 232) who were taking at least one medication to treat their vasculitis. INTERVENTION: Administration of online survey. MAIN OUTCOME MEASURES: Patient use of pharmacists, physicians, and the Internet for medication information; perceived credibility of pharmacists, physicians, and the Internet as sources of medication information; and types of medication information obtained from pharmacists, physicians, and the Internet. RESULTS: Participants consulted physicians and the Internet more than pharmacists for medication information; only 96 participants (41.4%) ever used pharmacists for vasculitis medication information. Females and participants who used community pharmacies were significantly more likely to consult pharmacists for medication information as compared with males and patients who did not use community pharmacies. Participants perceived pharmacists were a less credible source of medication information than physicians and the Internet. Participants used physicians and/or the Internet more than pharmacists for five of eight types of medication information, including adverse effects and drug effectiveness. CONCLUSION: Vasculitis patients consulted sources other than pharmacists for medication information. Several factors, including perceived pharmacist credibility and a noncommunity-based pharmacy, may contribute to infrequent patient use of pharmacists as a medication information source. Future qualitative research should document how patients with rare disease perceive and interact with pharmacists to understand why many view pharmacists as only moderately credible sources of medication information.

Patient perceptions about illness self-management in ANCA-associated small vessel vasculitis.

OBJECTIVES: To characterize patient perceptions, related to eight self-management behaviours relevant for adults with ANCA-associated small vessel vasculitis (ANCA-SVV), and to determine if these perceptions were associated with performance of each behaviour. METHODS: Adults with ANCA-SVV (n = 202) completed a self-administered questionnaire that assessed eight self-management behaviours (adherence to recommendations for medication, health service use, diet, exercise, infection avoidance and symptom monitoring; prompt reporting of symptoms and side effects; and adjusting activities in response to symptoms), perceptions about these behaviours, socio-demographics, clinical factors and social desirability bias. Descriptive statistics were generated to characterize patients' perceptions about difficulty of, importance of, and specific barriers to performing each behaviour. Regression analyses explored whether these variables were associated with performing each behaviour, controlling for potential confounders. RESULTS: With few exceptions, higher perceived importance and lower perceived difficulty of each behaviour were associated with more frequent performance of the behaviour. For each behaviour, several specific barriers were frequently endorsed by patients and a number of these were associated with lower levels of self-management. CONCLUSION: This study reveals that patient perceptions about the illness and its treatment influence ANCA-SVV self-management. Perceived barriers to medication, health services, diet and exercise adherence were similar to those in other illnesses. This study also provides insight into barriers experienced by patients in performing behaviours (infection avoidance, symptom monitoring, reporting symptoms and side-effects and adjusting activities) not often previously studied. How the identification of these barriers can help inform future interventions for ANCA-SVV patients is to be discussed.

Autoerythrocyte sensitization syndrome (Gardner-Diamond syndrome) associated with cutaneous vasculitis.

Autoerythrocyte sensitization syndrome (ASS) (Gardner-Diamond syndrome) is characterized by painful ecchymotic lesions affecting mostly women with emotional stress. Although it is widely accepted as a non-inflammatory disease, ASS can be accompanied by some autoimmune diseases. In this case report, we present a case with ASS associated with cutaneous vasculitis. We also briefly discuss the possible inflammatory features of ASS.

Development and initial evaluation of a measure of self-management for adults with antineutrophil cytoplasmic antibody-associated small-vessel vasculitis.

OBJECTIVE: To develop a measure of illness self-management for adults living with antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis (ANCA-SVV) and to gather evidence of its reliability and validity. METHODS: Development of the Vasculitis Self-Management Scale (VSMS) was guided by previous research on self-management in other chronically ill populations, a review of the current treatment literature for ANCA-SVV, interviews with patients, and consultation with experts. A total of 205 patients living with ANCA-SVV or a closely related condition then completed the VSMS, along with measures of sociodemographic and clinical variables, social desirability bias, and general adherence to medical recommendations, using a self-administered mailed questionnaire. A principal components analysis was conducted on the VSMS items. Internal consistency reliability and construct validity of the resulting subscales were assessed. Forty-four patients completed the VSMS a second time, for the purpose of assessing test-retest reliability. RESULTS: Analyses suggested an 8-factor solution. The final VSMS consisted of 43 items representing these 8 behavioral domains. Correlations among the 8 domains were null to modest in magnitude. The internal consistency reliability of the 8 subscales ranged from minimally acceptable (alpha = 0.67) to excellent (alpha = 0.94), and correlations between subscale scores at time 1 and time 2 suggested good temporal stability. Preliminary evidence for validity was mixed. CONCLUSION: These findings suggest that the VSMS is a promising method for assessing illness self-management in adults with ANCA-SVV. More research exploring the validity of the measure is warranted.

Inflammation, coagulation, and depressive symptomatology in cardiovascular disease-free people; the ATTICA study.

BACKGROUND: Depression is associated with an increase in cardiovascular disease, but the underlying mechanisms are not well understood. The aim of this study was to examine the associations of depressive symptoms with inflammation and coagulation factors related to cardiovascular risk in persons free of cardiovascular disease. METHODS: A random algorithm was developed and stratified by gender-age and multistage sampling was performed during 2001-2002. In this study, we analysed data from 453 men (19-89 years old) and 400 women (18-84 years old). Inflammation markers were C-reactive protein (CRP), serum amyloid A, white blood cell (WBC) and total platelet counts; and coagulation factors including homocysteine and fibrinogen. Depression was assessed with the Zung Self-Rating Depression Scale (ZDRS range 0-100) after validation for the study population. A ZDRS score of 50 or more classified a person as mildly depressive. Statistical adjustments were made for risk factors (age, gender, smoking, diabetes mellitus, and physical activity level). RESULTS: Women had significantly higher scores on the Zung depression scale than men (47 +/- 9 vs. 43 +/- 10, p<0.001). Thus, 21% of men and 27% of women had mild depression, while 4% of men and 6% of women had severe depressive symptomatology. The depression scale correlated positively with C-reactive protein levels (p<0.05), white blood cell count (p<0.05), and fibrinogen (p<0.05) in both genders after adjustment for control variables. CONCLUSION: This study revealed that depression was associated with inflammation and coagulation factors in cardiovascular disease-free people, suggesting a possible pathway leading to an increased frequency of events of coronary heart disease in depressive individuals.