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以快速进展性肾衰竭和腹股沟淋巴结病为表现的IgG4相关性疾病

IGg4-related Disease Presenting as Rapidly Progressive Renal Failure and Inguinal Iymphadenopathy.

作者信息

Anvar Maniyar Iqbal, Iyenger Jayaram N, Mehta Shweta S, Jagdeeshkumar Biradar

机构信息

Associate Professor Department of Nephrology, VIMS, Bellary, Karnataka, India.

Director at Anand Diagnostic Laboratory- A Neuberg Associate, Bengaluru, Karnataka, India.

出版信息

Indian J Nephrol. 2021 May-Jun;31(3):286-289. doi: 10.4103/ijn.IJN_377_19. Epub 2021 Apr 2.

DOI:10.4103/ijn.IJN_377_19
PMID:34376945
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8330657/
Abstract

A 58-year-old man presented with swelling in the left inguinal region and loss of appetite, nausea and azotemia with a serum creatinine of 5.2 mg/dL and urine albumin of 1+ and normal size kidneys with no hematuria. His serum creatinine increased to 9 mg/dL over 1 month, his total proteins were 10.8 and serum albumin was 3.3 g/dL, and lymph node excision biopsy showed fragments of lymphoid tissue with interfollicular areas containing sheets of plasma cells and atretic germinal centers. In view of unexplained renal failure, he underwent renal biopsy, which showed interstitial inflammatory infiltrate composed of lymphocytes and rich in plasma cells with storiform fibrosis and a possibility of IgG4-related renal disease was reported. On further evaluation, serum electrophoresis showed no M band. Serum IgG4 levels were 18.2 g/L (0.03-2 g/L). A diagnosis of IgG4-related renal disease was confirmed.

摘要

一名58岁男性,因左腹股沟区肿胀、食欲不振、恶心及氮质血症就诊,血清肌酐为5.2mg/dL,尿白蛋白为1+,双肾大小正常,无血尿。1个月内其血清肌酐升至9mg/dL,总蛋白为10.8,血清白蛋白为3.3g/dL,淋巴结切除活检显示淋巴组织碎片,滤泡间区域可见成片浆细胞及闭锁生发中心。鉴于不明原因的肾衰竭,患者接受了肾活检,结果显示间质炎性浸润由淋巴细胞组成,富含浆细胞,并伴有席纹状纤维化,报告怀疑为IgG4相关性肾病。进一步检查发现,血清电泳未显示M带。血清IgG4水平为18.2g/L(0.03 - 2g/L)。确诊为IgG4相关性肾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2422/8330657/2cf4cedb2531/IJN-31-286-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2422/8330657/979e32d0b2cd/IJN-31-286-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2422/8330657/2792372eba5c/IJN-31-286-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2422/8330657/2cf4cedb2531/IJN-31-286-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2422/8330657/979e32d0b2cd/IJN-31-286-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2422/8330657/2792372eba5c/IJN-31-286-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2422/8330657/2cf4cedb2531/IJN-31-286-g003.jpg

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