{"title":"Case of IgG4-related Disease with Crescentic Glomerulonephritis: An Unusual Presentation of the Disease.","authors":"Azin Ghaffari, Mitra Mehrazma, Shahrzad Ossareh","doi":"10.5254/dt6zs176","DOIUrl":null,"url":null,"abstract":"IgG4-related disease (IgG4-RD) is a chronic systemic inflammatory disease, characterized by tissue infiltration of lymphocytes and IgG4-secreting plasma cells, presenting by fibrosis of different tissues, which is usually responsive only to oral steroids therapy. Kidneys are the most commonly involved organs, exhibiting renal insufficiency, tubulointerstitial nephritis, and glomerulonephritis. Here, we describe a patient with acute renal insufficiency who was presented with edema, weakness, anemia and multiple lymphadenopathies. Kidney and lymph node biopsy showed crescentic glomerulonephritis in kidneys and lymphoplasmacytic infiltration in lymph nodes. After a course of treatment with an intravenous pulse of corticosteroid and cyclophosphamide, the patient's symptoms subsided, and kidney function improved. DOI: 10.52547/ijkd.7788.","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"142 1","pages":""},"PeriodicalIF":0.8000,"publicationDate":"2024-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Iranian journal of kidney diseases","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.5254/dt6zs176","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
IgG4-related disease (IgG4-RD) is a chronic systemic inflammatory disease, characterized by tissue infiltration of lymphocytes and IgG4-secreting plasma cells, presenting by fibrosis of different tissues, which is usually responsive only to oral steroids therapy. Kidneys are the most commonly involved organs, exhibiting renal insufficiency, tubulointerstitial nephritis, and glomerulonephritis. Here, we describe a patient with acute renal insufficiency who was presented with edema, weakness, anemia and multiple lymphadenopathies. Kidney and lymph node biopsy showed crescentic glomerulonephritis in kidneys and lymphoplasmacytic infiltration in lymph nodes. After a course of treatment with an intravenous pulse of corticosteroid and cyclophosphamide, the patient's symptoms subsided, and kidney function improved. DOI: 10.52547/ijkd.7788.
期刊介绍：
The Iranian Journal of Kidney Diseases (IJKD), a peer-reviewed journal in English, is the official publication of the Iranian Society of Nephrology. The aim of the IJKD is the worldwide reflection of the knowledge produced by the scientists and clinicians in nephrology. Published quarterly, the IJKD provides a new platform for advancement of the field. The journal’s objective is to serve as a focal point for debates and exchange of knowledge and experience among researchers in a global context. Original papers, case reports, and invited reviews on all aspects of the kidney diseases, hypertension, dialysis, and transplantation will be covered by the IJKD. Research on the basic science, clinical practice, and socio-economics of renal health are all welcomed by the editors of the journal.
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