Emma E van Daalen1, Maria A C Wester Trejo2, Arda Göçeroğlu2, Franco Ferrario3, Kensuke Joh4, Laure-Hélène Noël5, Yayoi Ogawa6, Suzanne Wilhelmus7, Miriam J Ball8, Eva Honsova9, Zdenka Hruskova10, Renate Kain8, Tomoyoshi Kimura11, Marek Kollar9, Andreas Kronbichler12, Kristine Lindhard13, Xavier Puéchal14, Steven Salvatore15, Wladimir Szpirt13, Hideki Takizawa16, Vladimir Tesar10, Annelies E Berden2,17, Olaf M Dekkers18, E Christiaan Hagen19, Jan Oosting2, Chinar Rahmattulla2, Ron Wolterbeek20, Willem Jan Bos21,22, Jan A Bruijn2, Ingeborg M Bajema2. 1. Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands emmavd@hotmail.com. 2. Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands. 3. Nephropathology Center, San Gerardo Hospital, Monza, Italy. 4. Department of Pathology, The Jikei University School of Medicine, Tokyo, Japan. 5. Department of Pathology, Necker Hospital, René Descartes University, Paris, France. 6. Hokkaido Renal Pathology Center, Sapporo, Japan. 7. Pathology Laboratory Pathan, Rotterdam, The Netherlands. 8. Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria. 9. Department of Pathology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic. 10. Department of Nephrology, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic. 11. Department of Nephrology, Japan Community Healthcare Organization, Sendai Hospital, Sendai, Japan. 12. Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, Innsbruck, Austria. 13. Department of Nephrology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. 14. Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France. 15. Department of Pathology, Weill Cornell Medical College, New York, New York. 16. Department of Nephrology, Teine Keijinkai Hospital, Sapporo, Japan. 17. Department of Rheumatology, Leiden University Medical Center, Leiden, The Netherlands. 18. Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands. 19. Department of Nephrology, Meander Medical Center, Amersfoort, The Netherlands. 20. Department of Medical Statistics and Bioinformatics, Leiden University Medical Center, Leiden, The Netherlands. 21. Department of Internal Medicine, St. Antoniusziekenhuis, Nieuwegein, The Netherlands. 22. Department of Nephrology, Leiden University Medical Center, Leiden, The Netherlands.
Abstract
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.
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.
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