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Aim: Currently, several different instruments are used to measure disease activity and extent in clinical trials of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, leading to division among investigative groups and difficulty comparing study results. An exercise comparing six different vasculitis instruments was performed., Methods: A total of 10 experienced vasculitis investigators from 5 countries scored 20 cases in the literature of Wegener granulomatosis or microscopic polyangiitis using 6 disease assessment tools: the Birmingham Vasculitis Activity Score (BVAS), The BVAS for Wegener granulomatosis (BVAS/WG), BVAS 2003, a Physician Global Assessment (PGA), the Disease Extent Index (DEI) and the Five Factor Score (FFS). Five cases were rescored by all raters., Results: Reliability of the measures was extremely high (intraclass correlations for the six measures all = 0.98). Within each instrument, there were no significant differences or outliers among the scores from the 10 investigators. Test/retest reliability was high for each measure: range = 0.77 to 0.95. The scores of the five acute activity measures correlated extremely well with one another., Conclusions: Currently available tools for measuring disease extent and activity in ANCA-associated vasculitis are highly correlated and reliable. These results provide investigators with confidence to compare different clinical trial data and helps form common ground as international research groups develop new, improved and universally accepted vasculitis disease assessment instruments.

Investigators form the National Institutes of Health (NIH), in the United States, have prospectively studied 60 patients with Takayasus's arteritis over a period of 20 years. Unique observations resulted from the requirement that all patients be part of Institutional Review Board-approved standardized protocols that required periodic complete clinical, laboratory and angiographic evaluations, regardless of degree of disease activity. Patients judged to have active disease received protocol defined treatment that always included prednisone, and to which cytotoxic agents were added in the event that relapse followed tapering or withdrawal of corticosteroids. The prospective standard approach applied to this cohort has provided new insights into the presentation, course and treatment-responsiveness of Takayasu's arteritis. This article will focus on the questions raised by this study and implications for patient care.

Objective: To estimate the prevalence, annual mortality, and geographic distribution of Wegener's granulomatosis., Methods: Analysis of national vital statistics data and hospitalization data from a national survey and from all New York State inpatient facilities., Results: Between 1979 and 1988, 1,784 death certificates in the United States listed Wegener's granulomatosis as a cause of death. Nationally, an estimated 10,771 hospitalizations included Wegener's granulomatosis among the discharge diagnoses. In New York State, there were 978 hospitalizations among 571 individuals with Wegener's granulomatosis., Conclusion: The prevalence of Wegener's granulomatosis in the United States is approximately 3.0 per 100,000 persons. Clear differences in the geographic distribution of Wegener's granulomatosis are apparent when analysis consider rates of disease in individual counties. Contrary to previous reports, associations between disease exacerbations and season were not apparent.