The prognostic value of the EUVAS-proposed histopathological classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis has been evaluated throughout the world. Here, we performed a Japanese nationwide biopsy survey to assess the association between this histopathological classification and renal prognosis after 2-year follow-up in ANCA-associated glomerulonephritis.
We collected 67 renal biopsy materials of the 321 entries in the RemIT-JAV-RPGN cohort study, and assessed their histologies. Based on the EUVAS-proposed histopathological classification and some histological parameters, we statistically evaluated renal survival and the comparison of renal function for 2 years.
Based on the histopathological classification, the largest number of biopsy samples belonged to the Focal class, followed by the Mixed, Crescentic, and Sclerotic classes (n = 30, 19, 10, 8, respectively). Although the number of events might be too low (four patients with renal death) to make this conclusion, the Focal and Mixed classes had higher renal-survival rates compared to the others in the renal-survival curve. Comparing renal function among all classes, the estimated glomerular filtration rate (eGFR) throughout 2-year follow-up period was significantly higher in the Focal class compared to the other 3 classes. The eGFR-values in the Crescentic, Mixed, and Sclerotic classes increased with time. Based on both combined results, the Focal class could be the best prognosis.
This histopathological classification was valuable for both the stratification of renal function and the estimation of partial renal survival during 2-year follow-up in ANCA-associated glomerulonephritis.
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This work was supported by grants from the Research Committee of Intractable Renal Disease and Research Committee of Intractable Vasculitis Syndrome of the Ministry of Health, Labour, and Welfare of Japan.
Research Committee of Intractable Renal Disease and Research Committee of Intractable Vasculitis Syndrome of the Ministry of Health, Labour, and Welfare of Japan: In addition to the authors, the following investigators and institutions participated in this study: Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine (Yoshinari Takasaki); Division of Rheumatology and Nephrology, Department of Internal Medicine, Aichi Medical School of Medicine (Shogo Banno); Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology (Takahiko Sugihara); Department of Rheumatology, Shimane University Faculty of Medicine (Yohko Murakawa); Department of Hematology, Clinical Immunology and Infectious Diseases, Ehime University Graduate School of Medicine (Hitoshi Hasegawa); Department of Hematology, Oncology, Nephrology, and Rheumatology, Akita University School of Medicine (Atsushi Komatsuda); Niigata Rheumatic Center (Satoshi Ito); Department of the Control for Rheumatic Diseases, Graduate School of Medicine, Kyoto University (Takao Fujii); Department of Immunology and Rheumatology, Clinical Neuroscience and Neurology, Endocrinology and Metabolism, Nagasaki University Graduate School of Biomedical Sciences (Atsushi Kawakami); Department of Nephrology, Iwate Prefectural Central Hospital (Izaya Nakaya); Center for Rheumatology, Okayama Saiseikai General Hospital (Masahiro Yamamura); Department of Medical Technology, School of Health Sciences, Faculty of Medicine, Niigata University (Masaaki Nakano); Department of Medicine, Kidney Center, Tokyo Women’s Medical University (Kosaku Nitta); Division of Nephrology and Hypertension, Kashiwa Hospital, Jikei University (Makoto Ogura); Department of Nephrology, Nagoya City University Graduate School of Medical Sciences (Taio Naniwa); Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine (Shoichi Ozaki); Department of Nephrology and Endocrinology, Graduate School of Medicine, The University of Tokyo (Junichi Hirahashi); Third Department of Internal Medicine, Division of Immunology and Rheumatology, Hamamatsu University School of Medicine, Hamamatsu (Noriyoshi Ogawa); Division of Kidney and Hypertension, Department of Internal Medicine, Jikei University School of Medicine (Tatsuo Hosoya); Division of Nephrology, Department of Laboratory Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Faculty of Medicine, Kanazawa University (Takashi Wada); Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine (Satoshi Horikoshi); Institute of Rheumatology, Tokyo Women’s Medical University (Yasushi Kawaguchi); Division of Clinical Immunology, Graduate School of Comprehensive Human Sciences, University of Tsukuba (Taichi Hayashi); Department of Nephrology, Tokyo Medical University Hachioji Medical Center (Masaharu Yoshida); Department of Nephrology, Hypertension, Diabetology, Endocrinology and Metabolic, Fukushima Medical University (Tsuyoshi Watanabe); Department of Nephrology, Japanese Red Cross Nagoya Daini Hospital (Daijo Inaguma); Department of Integrated Therapy for Chronic Kidney Disease, Kyushu University (Kazuhiko Tsuruya); Niigata Prefectural Shibata Hospital (Noriyuki Homma); Department of Pathology, Keio University School of Medicine, Tokyo, Japan (Yasunori Okada); Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine (Tsutomu Takeuchi); Cardiovascular Respiratory and Neurology Division, Department of Internal Medicine, Asahikawa Medical University (Naoki Nakagawa); Kurobe City Hospital (Shinichi Takeda); Fukuoka Higashi Medical Center (Ritsuko Katabuchi); Division of Nephrology, Department of Medicine, Faculty of Medical Sciences, University of Fukui (Masayuki Iwano).
The members of the Research Committee of Intractable Renal Disease and the Research Committee of Intractable Vasculitis Syndrome of the Ministry of Health, Labour, and Welfare of Japan are listed in “Acknowledgements” section.
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Supplemental Table 2. Clinical findings of the Focal class with or without rapidly progressive glomerulonephritis. Abbreviations: MPA, microscopic polyangiitis; GPA, granulomatous polyangiitis; UC, unclassifiable; ANCA, anti-neutrophil cytoplasmic antibodies; MPO, myeloperoxidase; PR3, proteinase-3; BVAS, Birmingham vasculitis activity score; eGFR, estimated glomerular filtration rate (XLS 30 KB)
Supplemental Figure 1. Comparison of eGFR trends between 2 subgroups of the Focal class: patients with and those without rapidly progressive glomerulonephritis. Abbreviations: eGFR, estimated glomerular filtration rate; RPGN, rapidly progressive glomerulonephritis (PPT 135 KB)
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Yamagata, K., Usui, J., Nagata, M. et al. Histopathological classification of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in a nationwide Japanese prospective 2-year follow-up cohort study. Clin Exp Nephrol 23, 387–394 (2019). https://doi.org/10.1007/s10157-018-1656-1
- Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis
- Histopathological classification
- Renal prognosis
- Nationwide prospective cohort study
- Rapidly progressive glomerulonephritis