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.

Development of comprehensive disease assessment in systemic vasculitis.

The systemic vasculitides are multisystem disorders with considerable mortality and morbidity and frequent relapses. In the absence of reliable serological markers, accurate clinical tools are required to assess disease activity and damage for treatment decisions, and for the performance of clinical trials. This article reviews and summarises the development and use of disease assessment tools for determining activity and damage in systemic vasculitis and reports ongoing initiatives for further development of disease assessment tools. A literature search was conducted using PubMed and reference lists for vasculitis, assessment, clinical trials, outcome and prognosis. The findings indicate that comprehensive disease assessment in vasculitis requires documentation of disease activity, chronic irreversible damage and impairment of function.

[A new training program--a status report: vasculitis]. / Ein neues Schulungsprogramm-ein Statusbericht: Vaskulitis.

Referring to the literature, it is known that structured standardized patient education represents an effective additional treatment in patients with chronic diseases. Programs are based on cognitive behavioral interventions. Within the last years an interdisciplinary approach for providing information on disease, therapies, side effects, coping strategies, nutrition and physiotherapy has been developed for patients with primary systemic vasculitides (PSV) in the vasculitis center Medical University of Luebeck/Bad Bramstedt. The contents of the seminars were revised and condensed into five modules. To evaluate the new program a documentation system consisting of patient and physician-administered questionnaires assessing socioeconomic, knowledge and disease-related outcome-parameters has been designed. Patients completed the questionnaires at baseline, 4 weeks, 6 and 12 months after training. First results show statistically significant improvement of knowledge and health-related quality of life.

Impairment of lung function, health status and functional capacity in patients with ANCA-associated vasculitis.

OBJECTIVE: To determine the effects of lung involvement on respiratory function in patients with ANCA-associated vasculitis and the relation to impaired health status. METHODS: Thirty patients with ANCA-associated vasculitis in remission (15 with lung involvement at diagnosis as determined by an abnormal chest X-ray) were examined. We measured lung function, skeletal muscle strength [quadriceps force (QF), respiratory muscle strength (Pi(max))], exercise capacity (VO(2) peak) using treadmill exercise tests, and health status using the Short Form 36 and St George's respiratory questionnaires. RESULTS: Exercise capacity was reduced compared with predicted values (58.2%, range 23-123%) and 18 patients showed functional aerobic impairment. Respiratory muscle function was reduced (72.1% predicted, range 20-108%) and was not related to lung involvement or steroid usage. Transfer factor correlated significantly with exercise capacity, suggesting inadequate delivery of oxygen to muscles. Nine patients had reduced transfer factor (seven with lung involvement). Patients with lung involvement had impaired gas transfer compared with those without lung involvement (96.9 +/- 6 vs 113.3 +/- 4.7% predicted, P = 0.04). However, there were significant abnormalities in other lung function parameters not related to previous lung involvement (eight patients had reduced forced expiratory volume in 1 s, and five patients had reduced residual volume). Twelve patients (five with previous lung involvement) had obstructive airways disease. Physical health status was impaired to a greater degree than mental health status across the whole group and was not related to lung involvement or original disease severity, but correlated with transfer factor. CONCLUSION: Patients with ANCA-associated disease may have significant lung function impairment irrespective of lung involvement at the time of diagnosis. Patients showed reduced respiratory muscle strength, health status and exercise capacity, which correlated with reduced transfer factor.

[Scientifically based patient education exemplified by patients with primary systemic vasculitis. The vasculitis patient education program--development, contents and initial results of evaluation]. / Wissenschaftlich begründete Patientenschulung am Beispiel der Patienten mit primär systemischen Vaskulitiden. Das Vaskulitis-Schulungsprogramm--Entwicklung, Inhalte und erste Ergebnisse der Evaluation.

Referring to the literature, it is known that structured standardized patient education represents an effective additional treatment in patients with chronic diseases. Most programs are based on cognitive behavioral interventions. Within the last few years, an interdisciplinary approach for providing information on disease, therapies, side effects, coping strategies, nutrition and physiotherapy has been developed for patients with primary systemic vasculitides (PSV) in the vasculitis center of the Medical University of Lübeck/Bad Bramstedt. The contents of the seminars were revised and condensed into five modules. New slides and handouts for patients were developed. To evaluate the new form of the program, a documentation system consisting of patient and physician-administered questionnaires assessing socioeconomic, knowledge and disease-related outcome-parameters was designed. Patients are trained in closed groups (n = 10-15) and asked to complete questionnaires at baseline, 4 weeks and 6 months after training. First results show statistically significant improvement of knowledge and health-related quality of life.

[Long-term prognosis and outcome of patients with primary systemic vasculitis: initial results in Germany]. / Langzeitprognose und -outcome von Patienten mit primär systemischen Vaskulitiden (PSV): Erste Ergebnisse in der BRD.

Some outcome parameters have been shown to represent valuable measures for the long-term assessment of primary systemic vasculitides (PSV). These parameters include disease extent, disease activity, damage, and the health-related quality of life. We describe a concept for the establishment of a detailed documentation system for PSV with a duration of at least 5 years to obtain a reliable characterization of these patients. This database will provide the basis for a PSV cohort and the development of an interdisciplinary patient education concept followed by an evaluation with a multicenter, trial.

Surto de pseudo-exacerbaçao em pacientes dimorfo com vasculites granulomatosas especificas dissemidadas / I occasion of pseudo exacerbacion in bordeline patient with spread specific granulomatous vasculites

Um paciente idoso de cor negra com diagnostico de hanseniase virchowiana e tuberculoide ganglionar, apos apresentar surtos de eritema nodoso no primeiro ano de tratamento e internado com surto reacional caracterizado por difusa infiltraçao eritematosa cutanea, edema intenso de maos e pes e lesoes necroticas, tipo vasculite, nos membros inferiores que evoluem para ulceraçoes e extensas necroses dos segmentos anteriores dos pes. Falece em choque. Os achados de autopsia permitem demonstrar reaçao granulomatosa tuberculoide, disseminada nas localizaçoes cutaneo-neurais, mucosas e viscerais normalmente envolvidas na hanseniase virchowiana, chamando atençao o comprometimento quase generalizado dos vasos nestas localizaçoes por reaçao granulomatosa em todos os niveis da parede vascular. Nas extremidades ha nitida correlaçao entre estas vasculites que envolvem veias e arterias de pequeno calibre e as necroses cutaneas. A interpretaçao para estes achados e de que este paciente apresentou na vigencia do tratamento um surto reacional do tipo pseudo-exacerbaçao ou "reversal reaction" que caprichosamente envolveu pequenos vasos...