Tubulointerstitial nephritis with IgM-positive plasma cells complicated by liver failure.

IF 1 Q4 UROLOGY & NEPHROLOGY CEN Case Reports Pub Date : 2024-10-23 DOI:10.1007/s13730-024-00932-9
Takashi Kudo, Daigo Nakazawa, Saori Nishio, Fumihiko Hattanda, Yusho Ueda, Junpei Yoshikawa, Satoka Shiratori-Aso, Sari Iwasaki, Takahiro Tsuji, Yasuni Nakanuma, Goki Suda, Koji Ogawa, Naoya Sakamoto, Tatsuya Atsumi
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Abstract

Tubulointerstitial nephritis (TIN) is characterized by inflammation of the renal interstitium with the infiltration of immune cells, mainly consisting of T cells. Recently, patients with TIN with the predominant infiltration of immunoglobulin M (IgM)-positive plasma cells were reported, coined IgMPC-TIN. Here we report the case of a 70-year-old woman diagnosed with Fanconi syndrome and renal tubular acidosis. Renal biopsy revealed IgMPC-TIN. Her renal dysfunction and clinical findings improved after corticosteroid therapy. However, the patient died of progressive liver failure and spontaneous bacterial peritonitis. In laboratory tests, viral hepatitis was excluded, and autoantibodies associated with liver diseases were negative. Generally, IgMPC-TIN is often complicated by primary biliary cholangitis (PBC), whereas her autopsy revealed the local infiltration of IgM-positive plasma cells, obliterative portal venopathy, and nodular regenerative hyperplasia in liver. This case is the first demonstration that IgMPC-TIN is also seen in liver disease with nodular regenerative hyperplasia, although IgMPC-TIN is more common in anti-M2 antibody-positive disease.

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IgM阳性浆细胞并发肝衰竭的肾小管间质性肾炎。
肾小管间质性肾炎(TIN)的特点是肾间质炎症,免疫细胞浸润,主要由 T 细胞组成。最近,有报道称 TIN 患者主要伴有免疫球蛋白 M(IgM)阳性浆细胞的浸润,被称为 IgMPC-TIN。在此,我们报告了一例被诊断为范可尼综合征和肾小管酸中毒的 70 岁女性病例。肾活检发现了 IgMPC-TIN。经过皮质类固醇治疗后,她的肾功能障碍和临床症状有所改善。然而,患者死于进行性肝功能衰竭和自发性细菌性腹膜炎。实验室检查排除了病毒性肝炎,与肝病相关的自身抗体呈阴性。一般来说,IgMPC-TIN 通常会并发原发性胆汁性胆管炎(PBC),而她的尸检结果显示,局部存在 IgM 阳性浆细胞浸润、闭塞性门静脉病变和肝脏结节性再生增生。该病例首次证明,IgMPC-TIN 也可见于伴有结节性再生增生的肝病,尽管 IgMPC-TIN 在抗 M2 抗体阳性的疾病中更为常见。
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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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