Outcomes of retroperitoneal fibrosis-related hydronephrosis and its risk factors for poor prognosis: a multi-center retrospective cohort study in Chinese patients

ObjectiveRetroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibroinflammatory tissue that surrounds the abdominal aorta and the iliac arteries and often entraps the ureters. Hydronephrosis is a common complication of RPF, however, its clinical features and outcomes hav...

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Main Authors: Hongyan Liu, Hui Gao, Jin-xia Zhao, Ut-kei Wong, Shi-bo Liu, Jian Liu, Ge Zhang, Kuan-ting Wang, Yan Wang, Lin Zhao, Xiang-bo Ma, Yue-wu Lu, Xue-wu Zhang
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-12-01
Series:Frontiers in Medicine
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Online Access:https://www.frontiersin.org/articles/10.3389/fmed.2024.1435870/full
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author Hongyan Liu
Hui Gao
Jin-xia Zhao
Ut-kei Wong
Shi-bo Liu
Jian Liu
Ge Zhang
Kuan-ting Wang
Yan Wang
Lin Zhao
Xiang-bo Ma
Yue-wu Lu
Xue-wu Zhang
author_facet Hongyan Liu
Hui Gao
Jin-xia Zhao
Ut-kei Wong
Shi-bo Liu
Jian Liu
Ge Zhang
Kuan-ting Wang
Yan Wang
Lin Zhao
Xiang-bo Ma
Yue-wu Lu
Xue-wu Zhang
author_sort Hongyan Liu
collection DOAJ
description ObjectiveRetroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibroinflammatory tissue that surrounds the abdominal aorta and the iliac arteries and often entraps the ureters. Hydronephrosis is a common complication of RPF, however, its clinical features and outcomes have not been well elucidated.MethodsA total of 115 RPF-related hydronephrosis patients have been recruited from 9 clinical centers in China since March 2010. They were followed up until death or September 2021, whichever came first.ResultsThe mean age at diagnosis was 58.83 ± 12.13 years, and 80 patients (69.57%) were men. The median disease duration was 3.00 (1.00, 9.00) months. Renal impairment was observed in 88.35% of the patients, and 49.57% showed bilateral ureteral involvement. Elevated ESR and CRP were presented in 80.28 and 62.02% of the patients, respectively. Overall, 28.21% (11/39) of the patients had increased IgG4 levels, and 41.38% (12/29) showed positive pathological IgG4 staining (IgG4+/IgG+ ≥ 40% or IgG4+ ≥ 10/HPF). Among them, three patients were diagnosed as IgG4RD. After 60.43 ± 34.53 months of follow-up, 36 patients had poor prognosis, which was associated with severe kidney impairment, bilateral hydronephrosis and inflammation status (elevated ESR and IgG) at diagnosis by case–control study. eGFR and creatinine were independent risk factors after adjusting for all other significant associations (p = 0.002 and p = 0.067, respectively). Glucocorticoid-based therapy could reduce the time of stenting, decrease the need for long-term ureteral stenting/percutaneous nephrostomy (PNS)/ureterolysis, increase the rate of mass shrinkage, and reduce the new requirement of hemodialysis compared to surgery-only strategy for RPF-related hydronephrosis patients in need of renal drainage, but did not reduce new-onset renal atrophy.ConclusionSeverity of kidney dysfunction and inflammation status were related to the poor prognosis of hydronephrosis induced by RPF. More efficient interventions and strategies are needed to further improve outcomes.
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spelling doaj-art-3ceb92acf71648a5ab3fcbdeb6031ad52025-08-20T02:20:40ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2024-12-011110.3389/fmed.2024.14358701435870Outcomes of retroperitoneal fibrosis-related hydronephrosis and its risk factors for poor prognosis: a multi-center retrospective cohort study in Chinese patientsHongyan Liu0Hui Gao1Jin-xia Zhao2Ut-kei Wong3Shi-bo Liu4Jian Liu5Ge Zhang6Kuan-ting Wang7Yan Wang8Lin Zhao9Xiang-bo Ma10Yue-wu Lu11Xue-wu Zhang12Department of Rheumatology and Immunology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, ChinaDepartment of Rheumatology and Immunology, Peking University International Hospital, Beijing, ChinaDepartment of Rheumatology and Immunology, Peking University Third Hospital, Beijing, ChinaDepartment of Rheumatology and Immunology, Peking University International Hospital, Beijing, ChinaDepartment of Retroperitoneal Tumor Surgery, Peking University International Hospital, Beijing, ChinaDepartment of Rheumatology and Immunology, Aerospace Center Hospital, Peking University Aerospace School of Clinical Medicine, Beijing, ChinaDepartment of Rheumatology and Immunology, Beijing Haidian Hospital, Beijing Haidian Section of Peking University Third Hospital, Beijing, ChinaDepartment of Rheumatology and Immunology, Peking University Shougang Hospital, Beijing, ChinaDepartment of Rheumatology and Immunology, The Fifth Affiliated Hospital of Zhengzhou University, Zhengzhou, ChinaDepartment of Endocrinology, Rheumatology, and Immunology, The Fourth Clinical College of Xinxiang Medical University, Xinxiang, China0Department of Rheumatology and Immunology, Handan First Hospital, Handan, ChinaDepartment of Rheumatology and Immunology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China1Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, ChinaObjectiveRetroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibroinflammatory tissue that surrounds the abdominal aorta and the iliac arteries and often entraps the ureters. Hydronephrosis is a common complication of RPF, however, its clinical features and outcomes have not been well elucidated.MethodsA total of 115 RPF-related hydronephrosis patients have been recruited from 9 clinical centers in China since March 2010. They were followed up until death or September 2021, whichever came first.ResultsThe mean age at diagnosis was 58.83 ± 12.13 years, and 80 patients (69.57%) were men. The median disease duration was 3.00 (1.00, 9.00) months. Renal impairment was observed in 88.35% of the patients, and 49.57% showed bilateral ureteral involvement. Elevated ESR and CRP were presented in 80.28 and 62.02% of the patients, respectively. Overall, 28.21% (11/39) of the patients had increased IgG4 levels, and 41.38% (12/29) showed positive pathological IgG4 staining (IgG4+/IgG+ ≥ 40% or IgG4+ ≥ 10/HPF). Among them, three patients were diagnosed as IgG4RD. After 60.43 ± 34.53 months of follow-up, 36 patients had poor prognosis, which was associated with severe kidney impairment, bilateral hydronephrosis and inflammation status (elevated ESR and IgG) at diagnosis by case–control study. eGFR and creatinine were independent risk factors after adjusting for all other significant associations (p = 0.002 and p = 0.067, respectively). Glucocorticoid-based therapy could reduce the time of stenting, decrease the need for long-term ureteral stenting/percutaneous nephrostomy (PNS)/ureterolysis, increase the rate of mass shrinkage, and reduce the new requirement of hemodialysis compared to surgery-only strategy for RPF-related hydronephrosis patients in need of renal drainage, but did not reduce new-onset renal atrophy.ConclusionSeverity of kidney dysfunction and inflammation status were related to the poor prognosis of hydronephrosis induced by RPF. More efficient interventions and strategies are needed to further improve outcomes.https://www.frontiersin.org/articles/10.3389/fmed.2024.1435870/fullretroperitoneal fibrosishydronephrosisrisk factorpoor prognosistreat to cohort study
spellingShingle Hongyan Liu
Hui Gao
Jin-xia Zhao
Ut-kei Wong
Shi-bo Liu
Jian Liu
Ge Zhang
Kuan-ting Wang
Yan Wang
Lin Zhao
Xiang-bo Ma
Yue-wu Lu
Xue-wu Zhang
Outcomes of retroperitoneal fibrosis-related hydronephrosis and its risk factors for poor prognosis: a multi-center retrospective cohort study in Chinese patients
Frontiers in Medicine
retroperitoneal fibrosis
hydronephrosis
risk factor
poor prognosis
treat to cohort study
title Outcomes of retroperitoneal fibrosis-related hydronephrosis and its risk factors for poor prognosis: a multi-center retrospective cohort study in Chinese patients
title_full Outcomes of retroperitoneal fibrosis-related hydronephrosis and its risk factors for poor prognosis: a multi-center retrospective cohort study in Chinese patients
title_fullStr Outcomes of retroperitoneal fibrosis-related hydronephrosis and its risk factors for poor prognosis: a multi-center retrospective cohort study in Chinese patients
title_full_unstemmed Outcomes of retroperitoneal fibrosis-related hydronephrosis and its risk factors for poor prognosis: a multi-center retrospective cohort study in Chinese patients
title_short Outcomes of retroperitoneal fibrosis-related hydronephrosis and its risk factors for poor prognosis: a multi-center retrospective cohort study in Chinese patients
title_sort outcomes of retroperitoneal fibrosis related hydronephrosis and its risk factors for poor prognosis a multi center retrospective cohort study in chinese patients
topic retroperitoneal fibrosis
hydronephrosis
risk factor
poor prognosis
treat to cohort study
url https://www.frontiersin.org/articles/10.3389/fmed.2024.1435870/full
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