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.

Patient-reported outcome assessment in vasculitis may provide important data and a unique perspective.

OBJECTIVE: To assess patients' self-estimates of the burden of disease in vasculitis and to compare data across patient populations from the US, Germany, and the UK. Outcome assessment in vasculitis primarily focuses on physician evaluations of disease activity and damage. Little is known about the patients' perspectives regarding the burden of disease. METHODS: Patients ranked (scale 0-5) a list of vasculitis-related items to estimate a combination of pain, interference with daily function, discomfort and/or annoyance, anxiety/psychological impact, and medical importance. Patients were also asked to list the 5 most troublesome aspects of the disease. RESULTS: Data from 264 patients with vasculitis from 3 countries were collected. Wegener's granulomatosis was the predominant disease, comprising 63% of the cohort. Diagnoses were confirmed by physicians in 98% of cases. The most common item from the free-text sections was fatigue (75%), followed by pain (31%), musculoskeletal symptoms (24%), difficulty breathing (19%), financial aspects (13%), nasal discharge/crusting (14%), and weight gain (10%). Rankings of individual items varied substantially: fatigue (3.5), loss of energy (3.4), weight gain (3.1), joint pain (3.0), and sinusitis (3.0) were the highest-ranked symptoms among those manifestations experienced by at least 50% of patients. Several severe manifestations (e.g., dialysis, seizures, oxygen dependency) were ranked lower (<3.0). CONCLUSION: Fatigue and reduced energy level are considered the most important disease burdens by patients with vasculitis; manifestations associated with organ damage were rated lower. The patients' perspectives of the impact of vasculitis differ from the physicians' ratings. Future outcome assessment in vasculitis should include the patients' perspectives.

Commentary: "Who was caring for Mary?" revisited: a call for all academic physicians caring for patients to focus on systems and quality improvement.

Over 15 years have passed since Mary's near death (Annals of Internal Medicine. 1993;118:146-148). Disappointment in the care by fellow academic physicians persists; however, a reanalysis of her case through the lens of complex systems design and performance yields a more accurate and actionable perspective. Mary's suffering was not due to human failure alone. Human failure was provoked and exacerbated by broken processes including ambiguous assignments of responsibility; inadequate transfers of information and authority; unreliable or unavailable protocols for providing safe, effective treatment; and a failure to integrate the deep but narrow perspectives of individual specialists into a complete picture of Mary's condition. Her case exemplifies, in personal terms, many of the system challenges academic medical centers face: Faculty have other missions that can conflict with patient care; disease complexity is high, requiring input from multiple subspecialists; clinical departments serve as roadblocks to communication; and novice physicians, requiring close supervision, have primary responsibility for the day-to-day care of acutely ill patients. The academic physicians who first cared for Mary unwittingly accepted flawed systems, and they failed to work around them. At great monetary and emotional expense, last-minute heroics saved Mary. In a dysfunctional system, even the most conscientious physician may be viewed as uncaring. As Mary's case so clearly illustrates, patients and their families see the system and the physician as one. Only by working to improve the systems of delivery will academic physicians again be consistently viewed as caring.

Outpatient assessment of systemic vasculitis.

The vasculitides are complex, multisystem diseases, the natural history of which has been transformed by the use of immunosuppressive therapy. In many cases, survival is characterized by the presence of ongoing low-grade disease activity and, now that patients can survive acute, life-threatening manifestations of vasculitis, the new challenge is to provide accurate descriptions of these problems so as to plan appropriate changes in therapy to ensure optimal control of disease, with minimal toxicity, damage, and impairment of function. To achieve these aims, it is necessary to undertake structured evaluation of patients, with a significant dependence on careful clinical observation, supported by appropriate laboratory investigation at all stages from diagnosis to long-term care of these patients. This chapter reviews the important aspects of disease assessment and recommends a strategy for managing these complex patients. We can make use of standardized disease assessment instruments, such as the Birmingham Vasculitis Assessment Score and the Vasculitis Damage Index, to serve as an aide memoire in documenting the clinical features of disease. Routine monitoring for drug toxicity in the short-term and long-term is essential. Above all, however, it is very important to review these patients regularly and see them urgently in case of flares or complications, which are a common problem in most forms of vasculitis.

The association of social status and blood pressure with markers of vascular inflammation.

OBJECTIVE: Low socioeconomic status (SES) is associated with low-grade inflammation. Vascular inflammation often accompanies high blood pressure (BP) and has clinical implications for future vascular diseases, including atherosclerosis. Elevated plasma levels of soluble intercellular adhesion molecule-1 (sICAM-1) and endothelin-1 (ET-1) are reliable indicators of vascular inflammation. We examined the associations among BP, social status, and sICAM-1 and ET-1 levels in 121 European American and African American men and women. METHODS: Social status of the subjects was determined by using the Hollingshead Two Factor Index of Social Position scale, and plasma sICAM-1 and ET-1 levels were assessed using immunoassays. RESULTS: Correlation analyses revealed positive correlations among plasma sICAM-1 levels, BP, and social status. Levels of ET-1 were also significantly correlated with BP (p < .01) and social status (p < .001). When subjects were categorized into three social classes, sICAM-1 levels were significantly higher in the lowest social class as compared with the upper (p < .05) or middle (p < .01) class. The levels of ET-1 were higher in the low (p < .01) and middle (p < .05) social classes as compared with the upper class. Multiple hierarchic regression analyses revealed that even after controlling for demographic and health characteristics (gender, ethnicity, age, body mass index, and smoking) and systolic BP, social status accounted for additional variance of sICAM-1 or ET-1 levels. CONCLUSION: These results suggest that low-social-status individuals may incur risk for future vascular diseases through vascular inflammation.

Should C-reactive protein be added to metabolic syndrome and to assessment of global cardiovascular risk?

Of novel risk factors for cardiovascular disease currently under investigation, high-sensitivity C-reactive protein (hsCRP) is the most promising. To date, more than 20 prospective epidemiologic studies have demonstrated that hsCRP independently predicts vascular risk, 6 cohort studies have confirmed that hsCRP evaluation adds prognostic information beyond that available from the Framingham Risk Score, and 8 cohort studies have demonstrated additive prognostic value at all levels of metabolic syndrome or in the prediction of type 2 diabetes. In contrast to several other biomarkers that also reflect biological aspects of inflammation, hypofibrinolysis, and insulin resistance, hsCRP measurement is inexpensive, standardized, widely available, and has a decade-to-decade variation similar to that of cholesterol. Given the consistency of prognostic data for hsCRP and the practicality of its use in outpatient clinical settings, we believe the time has come for a careful consideration of adding hsCRP as a clinical criterion for metabolic syndrome and for the creation of an hsCRP-modified coronary risk score useful for global risk prediction in both men and women. Toward this end, we believe experts in the fields of epidemiology, prevention, vascular biology, and clinical cardiology should be convened to begin discussing the merits of this proposal.

The national database of the German Collaborative Arthritis Centres: I. Structure, aims, and patients.

OBJECTIVE: To describe the aims, principles, and content of the German rheumatological database and to present data on patient mix and healthcare provision for the year 1998. METHODS: The German rheumatological database contains clinical and patient derived data of the outpatients with inflammatory rheumatic diseases seen at one of the 24 collaborative arthritis centres. The case mix, institutional context, and demographic features of 25 653 patients from the year 1998 were analysed. RESULTS: 51% of the patients had rheumatoid arthritis, 23% seronegative spondyloarthropathies, including ankylosing spondylitis, psoriatic arthritis, and reactive arthritis, and 19% had vasculitis, including SLE (5%). The distribution of the age at onset of patients with RA with < or = 2 years' disease duration was comparable with recent incidence data from population studies. The case mix differed between university departments and rheumatology hospitals as well as individual practices. 65% of the male and 46% of the female patients at ages 18-60 were still in gainful employment, the rates of employment were 14% below the population rates for women, and 11% below those for men. 62% of all patients had seen a rheumatologist within the first year of disease, 73% within the first two years. Ankylosing spondylitis was seen in rheumatological care much later than all other diseases (only 39% within the first year). The mean number of contacts with a rheumatologist was five a year; rheumatologists in individual practices saw their patients seven times a year on the average. Together with visits to the non-specialist doctor mainly treating the patient, the mean number of visits to the doctor for a rheumatic condition was 20 a year. CONCLUSION: Large databases like this one give information about the patient case mix in different healthcare settings, about treatment practice, and about the consequences of disease. Patients treated in specialised rheumatology units in Germany are referred earlier than in the past, which probably reflects better regional cooperation due to the implementation of arthritis centres. University departments and outpatient clinics of rheumatology hospitals contribute considerably to the specialised care of patients with arthritis and connective tissue diseases.

Population based assessment of uveitis in an urban population in southern India.

AIM: To assess the prevalence of active and inactive uveitis unrelated to previous surgery or trauma in an urban population in southern India. METHODS: As part of the Andhra Pradesh Eye Disease Study, 2522 subjects (85.4% of those eligible), a sample representative of the population of Hyderabad city in southern India, underwent interview and detailed dilated eye examination. Presence of sequelae of uveitis without current active inflammation was defined as inactive uveitis. RESULTS: Unequivocal evidence of active or inactive uveitis unrelated to previous surgery or trauma was present in 21 subjects, an age-sex adjusted prevalence of 0.73% (95% confidence interval (CI) 0.44-1.14%). Active uveitis was present in eight subjects, an age-sex adjusted prevalence of 0.37% (95% CI 0. 19-0.70), of which 0.06% was anterior, 0.25% intermediate, and 0.06% posterior. The 0.36% (95% CI 0.17-0.68%) prevalence of inactive uveitis included macular chorioretinitis scars (0.26%), anterior (0. 07%) and previous vasculitis involving the whole eye (0.03%). The prevalence of visual impairment due to uveitis of less than 6/18 in at least one eye was 0.27%, less than 6/60 in at least one eye was 0. 16%, and less than 6/60 in both eyes was 0.03%. CONCLUSION: These population based cross sectional data give an estimate of the prevalence of various types of uveitis in this urban population in India. Active or past uveitis that might need treatment at some stage was present in one of every 140 people in this population.