PMID- 25749534 OWN - NLM STAT- MEDLINE DCOM- 20160519 LR - 20220331 IS - 1555-905X (Electronic) IS - 1555-9041 (Print) IS - 1555-9041 (Linking) VI - 10 IP - 5 DP - 2015 May 7 TI - Clinicopathologic characteristics and outcomes of renal thrombotic microangiopathy in anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis. PG - 750-8 LID - 10.2215/CJN.07910814 [doi] AB - BACKGROUND AND OBJECTIVES: Thrombotic microangiopathy (TMA) in ANCA-associated vasculitis (AAV) has been mainly reported in isolated case reports. The aim of this study was to analyze clinical and pathologic characteristics and prognosis of patients with renal TMA in ANCA-associated GN in a large cohort of Chinese patients. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Clinical and renal histopathologic data of 220 patients with biopsy-proven ANCA-associated GN from 1996 to 2013 were retrospectively analyzed. Patients were followed up for a median period of 32 (interquartile range [IQR], 12-65) months, and outcomes of patients were analyzed. RESULTS: Among the 220 patients with ANCA-associated GN, 30 were identified having concomitant renal TMA by pathologic evaluation. Compared with the non-TMA group, patients with renal TMA presented with more severe renal injury, as evidenced clinically by a higher level of serum creatinine at diagnosis (5.0 [IQR, 3.5-9.0] versus 3.2 [IQR, 1.7-6.8] mg/dl; P=0.02) and pathologically by a higher percentage of cellular crescents (15.0% [IQR, 6.9%-34.9%] versus 6.9% [IQR, 0%-21.1%]; P=0.04) and more severe interstitial infiltration (2 [IQR, 2-2] versus 2 [IQR, 1-2]; P=0.03) in renal biopsies. Furthermore, multivariate analysis showed that renal TMA was independently associated with mortality of patients with AAV after adjusting for age, sex, initial serum creatinine, tubular atrophy, and interstitial fibrosis (hazard ratio, 1.92; 95% confidence interval, 1.08 to 3.41; P=0.03) or for age, sex, the histopathologic classification scheme proposed by Berden et al. (J Am Soc Nephrol 21: 1628-1636, 2010), tubular atrophy, and interstitial fibrosis (hazard ratio, 1.95; 95% confidence interval, 1.07 to 3.55; P=0.03). CONCLUSIONS: Renal TMA in ANCA-associated GN is not rare and presents with more severe renal injury. Renal TMA is independently associated with all-cause mortality in patients with AAV. CI - Copyright (c) 2015 by the American Society of Nephrology. FAU - Chen, Su-Fang AU - Chen SF AD - Renal Division, Department of Medicine, Peking University, First Hospital, Beijing, China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China; Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Peking University, Ministry of Education, Beijing, China; and. FAU - Wang, Huan AU - Wang H AD - Renal Division, Department of Medicine, Peking University, First Hospital, Beijing, China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China; Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Peking University, Ministry of Education, Beijing, China; and. FAU - Huang, Yi-Min AU - Huang YM AD - Renal Division, Department of Medicine, Peking University, First Hospital, Beijing, China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China; Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Peking University, Ministry of Education, Beijing, China; and. FAU - Li, Zhi-Ying AU - Li ZY AD - Renal Division, Department of Medicine, Peking University, First Hospital, Beijing, China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China; Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Peking University, Ministry of Education, Beijing, China; and. FAU - Wang, Su-Xia AU - Wang SX AD - Renal Division, Department of Medicine, Peking University, First Hospital, Beijing, China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China; Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Peking University, Ministry of Education, Beijing, China; and. FAU - Yu, Feng AU - Yu F AD - Renal Division, Department of Medicine, Peking University, First Hospital, Beijing, China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China; Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Peking University, Ministry of Education, Beijing, China; and. FAU - Zhao, Ming-Hui AU - Zhao MH AD - Renal Division, Department of Medicine, Peking University, First Hospital, Beijing, China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China; Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Peking University, Ministry of Education, Beijing, China; and Peking-Tsinghua Center for Life Sciences, Beijing, China. FAU - Chen, Min AU - Chen M AD - Renal Division, Department of Medicine, Peking University, First Hospital, Beijing, China; Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, China; Key Laboratory of Chronic Kidney Disease Prevention and Treatment, Peking University, Ministry of Education, Beijing, China; and chenmin74@sina.com. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20150306 PL - United States TA - Clin J Am Soc Nephrol JT - Clinical journal of the American Society of Nephrology : CJASN JID - 101271570 RN - 0 (Adrenal Cortex Hormones) RN - 0 (Anti-Inflammatory Agents) RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Hemoglobins) RN - 0 (Immunosuppressive Agents) RN - 8N3DW7272P (Cyclophosphamide) RN - AYI8EX34EU (Creatinine) RN - EC 3.4.24.- (ADAM Proteins) RN - EC 3.4.24.87 (ADAMTS13 Protein) RN - EC 3.4.24.87 (ADAMTS13 protein, human) RN - MRK240IY2L (Azathioprine) RN - X4W7ZR7023 (Methylprednisolone) SB - IM MH - ADAM Proteins/blood MH - ADAMTS13 Protein MH - Adrenal Cortex Hormones/therapeutic use MH - Adult MH - Aged MH - Anti-Inflammatory Agents/therapeutic use MH - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications/mortality MH - Antibodies, Antineutrophil Cytoplasmic/analysis MH - Azathioprine/therapeutic use MH - Creatinine/blood MH - Cyclophosphamide/therapeutic use MH - Female MH - Glomerulonephritis/*complications/drug therapy/immunology/*pathology MH - Hemoglobins/metabolism MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Methylprednisolone/therapeutic use MH - Middle Aged MH - Prognosis MH - Recurrence MH - Retrospective Studies MH - Severity of Illness Index MH - Survival Rate MH - Thrombotic Microangiopathies/*complications/drug therapy/mortality/*pathology MH - Treatment Outcome PMC - PMC4422242 OTO - NOTNLM OT - ANCA OT - outcomes OT - thrombosis OT - vasculitis EDAT- 2015/03/10 06:00 MHDA- 2016/05/20 06:00 PMCR- 2016/05/07 CRDT- 2015/03/10 06:00 PHST- 2014/08/08 00:00 [received] PHST- 2015/01/21 00:00 [accepted] PHST- 2015/03/10 06:00 [entrez] PHST- 2015/03/10 06:00 [pubmed] PHST- 2016/05/20 06:00 [medline] PHST- 2016/05/07 00:00 [pmc-release] AID - CJN.07910814 [pii] AID - 07910814 [pii] AID - 10.2215/CJN.07910814 [doi] PST - ppublish SO - Clin J Am Soc Nephrol. 2015 May 7;10(5):750-8. doi: 10.2215/CJN.07910814. Epub 2015 Mar 6.