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Objective. We studied whether in ANCA-associated vasculitis patients, duration of AZA maintenance influenced relapse rate during long-term follow-up. Methods. Three hundred and eighty newly diagnosed ANCA-associated vasculitis patients from six European multicentre studies treated with AZA maintenance were included; 58% were male, median age at diagnosis 59.4 years (interquartile range: 48.3-68.2 years); granulomatosis with polyangiitis, n = 236; microscopic polyangiitis, n = 132; or renal limited vasculitis, n = 12. Patients were grouped according to the duration of AZA maintenance after remission induction: 418 months, ≼24 months, ≼36 months, ≼48 months or>48 months. Primary outcome was relapse-free survival at 60 months. Results. During follow-up, 84 first relapses occurred during AZA-maintenance therapy (1 relapse per 117 patient months) and 71 after withdrawal of AZA (1 relapse/113 months). During the first 12 months after withdrawal, 20 relapses occurred (1 relapse/119 months) and 29 relapses >12 months after withdrawal (1 relapse/186 months). Relapse-free survival at 60 months was 65.3% for patients receiving AZA maintenance >18 months after diagnosis vs 55% for those who discontinued maintenance ≼18 months (P = 0.11). Relapse-free survival was associated with induction therapy (i.v. vs oral) and ANCA specificity (PR3-ANCA vs MPO-ANCA/negative). Conclusion. Post hoc analysis of combined trial data suggest that stopping AZA maintenance therapy does not lead to a significant increase in relapse rate and AZA maintenance for more than 18months after diagnosis does not significantly influence relapse-free survival. ANCA specificity has more effect on relapse-free survival than duration of maintenance therapy and should be used to tailor therapy individually. [ABSTRACT FROM AUTHOR]

In the early 1990s, an international working group of experienced renal pathologists, the Renal Histology group, set up a scoring system for biopsies with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis. This scoring system subdivided glomerular, interstitial and vascular lesions and served as a tool for the evaluation of all renal biopsies from studies of the European Vasculitis Study Group (EUVAS). Histopathological studies gave new insights into the prediction of renal outcome in patients with ANCA-associated glomerulonephritis. Percentage of normal glomeruli and a selected number of interstitial parameters were reliable predictors of long-term follow-up glomerular filtration rate in all studies. Out of these results, a histopathological classification distinguishing focal, crescentic, mixed and sclerotic classes of ANCAassociated glomerulonephritis was developed. Until today, 13 studies have validated this classification system. Future studies will try to determine if and how renal histology could be helpful in guiding treatment of ANCA-associated glomerulonephritis. [ABSTRACT FROM AUTHOR]

An international meeting, The Asia Pacific Meeting of Vasculitis and ANCA Workshop 2012, was held on March 28-31, 2012 at Shinagawa, Tokyo, Japan. The meeting focused on vasculitis and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis in the Asia-Pacific area. This was a high-profile event and attracted scientists and physicians from around the world, all of whom are committed to the care of patients with vasculitis, ANCA and related diseases. The Asia Pacific international meeting was able to extend insights into Kawasaki disease, Takayasu arteritis, and myeloperoxidase ANCA-associated vasculitis. The program addressed the multidisciplinary nature of vasculitis, including genomics, genetics, pathogenesis, epidemiology, biomarkers, clinical features, and therapeutic trials. [ABSTRACT FROM AUTHOR]