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一例PLA2R阳性膜性病,随后发生IgG4相关疾病

Fumiaki Tanemoto¹, Imari Mimura², Hiroyuki Abe³

¹Division of Nephrology and Endocrinology, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.

CEN case reports

|November 8, 2024

概括

本案例研究描述了一名患有脂酶A2受体 (PLA2R) 阳性膜性病 (MN) 的患者,该患者后来患上与免疫球蛋白G4相关的疾病 (IgG4-RD). 它强调IgG4-RD甚至可以发生在PLA2R阳性的MN中.

科学领域:

腎臟病學 (nephrology) 是一種醫學專業.
免疫学免疫学免疫学
胃肠病学胃肠病学

背景情况:

膜性病 (MN) 是成年人性综合征的常见原因.
MN是免疫球蛋白G4相关疾病 (IgG4-RD) 的已知的体现.
以前,与IgG4-RD相关的MN通常是脂酶A2受体 (PLA2R) 负的.

研究的目的:

报告第一个PLA2R阳性MN病例,随后发生IgG4-RD.
在IgG4-RD的背景下,讨论区分初级MN和二级MN的诊断挑战.
强调在PLA2R阳性MN病例中考虑IgG4-RD的重要性.

主要方法:

一个60岁的男性被诊断出患有性综合征的病例报告.
最初的脏活检显示PLA2R阳性MN.
随访调查,包括成像和活检,揭示了自身免疫性胰腺炎和管管间炎,导致IgG4-RD诊断.

主要成果:

这名患者出现了PLA2R阳性MN,后来发生了IgG4-RD的胰腺和脏表现.
用葡萄糖皮质激素治疗IgG4-RD导致自身免疫性胰腺炎,质和MN的缓解.
这是PLA2R阳性MN在IgG4-RD发育之前首次被记录的病例.

结论:

在患有PLA2R阳性MN的患者中,应考虑IgG4-RD的可能性.
当IgG4-RD存在时,区分初级和二级MN可能具有挑战性.
这一案例扩大了对IgG4-RD参与的范围的理解.

关键词:

自身免疫性胰腺炎是一种自身免疫性胰腺炎与IgG4相关的疾病膜性脏病是一种膜性脏病.脏病综合征 - 脏病综合征脂酶A2受体 (PLA2R) 是一种一级膜性脏病的主要脏病.

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