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Objective: To investigate the occurrence of cardiovascular events (CVEs) in a large cohort of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) across the European Union, China, Turkey, Russia, the United Kingdom, and the USA., Methods: Patients with a definite diagnosis of AAV who were followed for ≥ 3 months and had sufficient documentation were included. Data on myocardial infarction (MI) and stroke were collected retrospectively from tertiary vasculitis centers. Univariate and multivariate Cox regression models were used to estimate hazard ratios (HRs) and 95% CIs., Results: Over a median follow-up of 62.0 months (IQR 22.6-100.0), CVEs (mostly MIs) occurred in 245 (10.7%) of 2286 patients with AAV, with a higher frequency in China and the UK. On multivariate regression analysis, older age (55-64.9 yrs, HR 2.93, 95% CI 1.99-4.31), smoking (HR 1.98, 95% CI 1.48-2.64), Chinese origin (HR 4.24, 95% CI 3.07-5.85), and pulmonary (HR 1.50, 95% CI 1.09-2.06) and kidney (HR 3.02, 95% CI 2.08-4.37) involvement were independent variables associated with a higher occurrence of CVEs., Conclusion: We showed that geographic region and both traditional and disease-specific (kidney involvement in particular) factors were independently associated with CVEs. Proper assessment and management of modifiable cardiovascular (CV) risk factors are essential for prevention of CV morbidity in patients with AAV., (Copyright © 2023 by the Journal of Rheumatology.)

Background and Objectives: The histopathologic classification for ANCA-associated GN distinguishes four classes on the basis of patterns of injury. In the original validation study, these classes were ordered by severity of kidney function loss as follows: focal, crescentic, mixed, and sclerotic. Subsequent validation studies disagreed on outcomes in the crescentic and mixed classes. This study, driven by the original investigators, provides several analyses in order to determine the current position of the histopathologic classification of ANCA-associated GN., Design, Setting, Participants, & Measurements: A validation study was performed with newly collected data from 145 patients from ten centers worldwide, including an analysis of interobserver agreement on the histopathologic evaluation of the kidney biopsies. This study also included a meta-analysis on previous validation studies and a validation of the recently proposed ANCA kidney risk score., Results: The validation study showed that kidney failure at 10-year follow-up was significantly different between the histopathologic classes ( P <0.001). Kidney failure at 10-year follow-up was 14% in the crescentic class versus 20% in the mixed class ( P =0.98). In the meta-analysis, no significant difference in kidney failure was also observed when crescentic class was compared with mixed class (relative risk, 1.15; 95% confidence interval, 0.94 to 1.41). When we applied the ANCA kidney risk score to our cohort, kidney survival at 3 years was 100%, 96%, and 77% in the low-, medium-, and high-risk groups, respectively ( P <0.001). These survival percentages are higher compared with the percentages in the original study., Conclusions: The crescentic and mixed classes seem to have a similar prognosis, also after adjusting for differences in patient populations, treatment, and interobserver agreement. However, at this stage, we are not inclined to merge the crescentic and mixed classes because the reported confidence intervals do not exclude important differences in prognosis and because an important histopathologic distinction would be lost., (Copyright © 2020 by the American Society of Nephrology.)

Purpose of Review: This review discusses the findings of studies validating the histopathological classification of antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis, which was devised in 2010 by an international working group of pathologists and nephrologists in collaboration with the European Vasculitis Society., Recent Findings: So far, eight studies have validated the histopathological classification of ANCA-associated glomerulonephritis. The studies came from Japan, China, Australia, the United States, the Netherlands, and Turkey. These validation studies confirmed that the histopathological classification of ANCA-associated glomerulonephritis is of predictive value for renal outcome. This was especially the case for patients with either a focal or sclerotic-class renal biopsy, whereas the crescentic and mixed classes showed different results in the validation studies. These differences could be due to differences in patient populations or therapy, inter-rater reliability and lack of inclusion of tubulointerstitial lesions in the classification. Therapy is known to influence renal outcome, but due to the retrospective design of the to-date performed validation studies, this parameter could not be fully accounted for in these validation studies. Inter-rater reliability among three histopathologists was investigated in one study and was moderate., Summary: The histopathological classification of ANCA-associated glomerulonephritis predicts renal outcome during follow-up, especially in patients with a focal or sclerotic-class renal biopsy. A large international validation study is currently being performed., Video Abstract: http://links.lww.com/CONH/A6.