Immunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatment

Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized clinical entity that often involves multiple organs; it is characterized by high levels of serum immunoglobulin G4 (IgG4), dense infiltration of IgG4+ cells, and storiform fibrosis. Cellular immunity, particularly T cell-mediated i...

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Autores principales:	Ke Zheng, Fei Teng, Xue-Mei Li
Formato:	article
Lenguaje:	EN
Publicado:	KeAi Communications Co., Ltd. 2017
Materias:	Medicine (General) R5-920
Acceso en línea:	https://doaj.org/article/8ccf1e6a64994429afeb06164de5f0de
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spelling	oai:doaj.org-article:8ccf1e6a64994429afeb06164de5f0de2021-12-02T13:35:02ZImmunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatment2095-882X10.1016/j.cdtm.2017.05.003https://doaj.org/article/8ccf1e6a64994429afeb06164de5f0de2017-09-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2095882X1630130Xhttps://doaj.org/toc/2095-882XImmunoglobulin G4-related disease (IgG4-RD) is a recently recognized clinical entity that often involves multiple organs; it is characterized by high levels of serum immunoglobulin G4 (IgG4), dense infiltration of IgG4+ cells, and storiform fibrosis. Cellular immunity, particularly T cell-mediated immunity, has been implicated in the pathogenesis of IgG4-RD. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulonephropathy (MGN), and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis, prostatitis, or ureter inflammation. Kidney function impairment can be acute or chronic. In IgG4-MGN, proteinuria can be in the nephrotic range. The diagnosis of IgG4-related kidney disease should not be based solely on serum IgG4 levels or the number of tissue-infiltrating IgG4+ plasma cells. Diagnosis should be based on specific histopathological findings, confirmed by tissue immunostaining and an appropriate clinical context. Steroid treatment is the first-line therapy. For relapsing or refractory cases, immunosuppressants could be combined with steroids. In hydronephrosis patients, appropriate immunosuppressive therapy could preclude the implantation of a double J ureteral catheter. Keywords: IgG4, Kidney, Pathogenesis, Diagnosis, TreatmentKe ZhengFei TengXue-Mei LiKeAi Communications Co., Ltd.articleMedicine (General)R5-920ENChronic Diseases and Translational Medicine, Vol 3, Iss 3, Pp 138-147 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine (General) R5-920
spellingShingle	Medicine (General) R5-920 Ke Zheng Fei Teng Xue-Mei Li Immunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatment
description	Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized clinical entity that often involves multiple organs; it is characterized by high levels of serum immunoglobulin G4 (IgG4), dense infiltration of IgG4+ cells, and storiform fibrosis. Cellular immunity, particularly T cell-mediated immunity, has been implicated in the pathogenesis of IgG4-RD. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulonephropathy (MGN), and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis, prostatitis, or ureter inflammation. Kidney function impairment can be acute or chronic. In IgG4-MGN, proteinuria can be in the nephrotic range. The diagnosis of IgG4-related kidney disease should not be based solely on serum IgG4 levels or the number of tissue-infiltrating IgG4+ plasma cells. Diagnosis should be based on specific histopathological findings, confirmed by tissue immunostaining and an appropriate clinical context. Steroid treatment is the first-line therapy. For relapsing or refractory cases, immunosuppressants could be combined with steroids. In hydronephrosis patients, appropriate immunosuppressive therapy could preclude the implantation of a double J ureteral catheter. Keywords: IgG4, Kidney, Pathogenesis, Diagnosis, Treatment
format	article
author	Ke Zheng Fei Teng Xue-Mei Li
author_facet	Ke Zheng Fei Teng Xue-Mei Li
author_sort	Ke Zheng
title	Immunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatment
title_short	Immunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatment
title_full	Immunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatment
title_fullStr	Immunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatment
title_full_unstemmed	Immunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatment
title_sort	immunoglobulin g4-related kidney disease: pathogenesis, diagnosis, and treatment
publisher	KeAi Communications Co., Ltd.
publishDate	2017
url	https://doaj.org/article/8ccf1e6a64994429afeb06164de5f0de
work_keys_str_mv	AT kezheng immunoglobuling4relatedkidneydiseasepathogenesisdiagnosisandtreatment AT feiteng immunoglobuling4relatedkidneydiseasepathogenesisdiagnosisandtreatment AT xuemeili immunoglobuling4relatedkidneydiseasepathogenesisdiagnosisandtreatment
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Immunoglobulin G4-related kidney disease: Pathogenesis, diagnosis, and treatment

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