Hypertrophic pachymeningitis, IgG4-related disease: case report

Q4 Medicine
G. Gerson, C. E. L. Soares, A. R. Rangel, G. Chagas, Daniel R. F. Távora, K. P. Fermon
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引用次数: 1

Abstract

The IgG4-related disease (IgG4-RD) is a systemic disease recently characterized as an inflammatory condition generally related to the increase in serum IgG4 levels, a subclass of immunoglobulins (IgG) which corresponds to less than 6% of the total serum IgG, with singular histopathological features. The involvement of the central nervous system is rare and may be isolated or associated with other organs, mimicking tumors. Commonly, it involves the hypophysis, presenting hypophysitis as the main manifestation, but it can also affect the dura mater, presenting as IgG4-related hypertrophic pachymeningitis (IgG4-RHP). Neurological manifestations occur as a result of mass effect, typically due to vascular or nervous structures compression, resulting in functional deficits according to the anatomical site of the lesion. The main histopathological features are dense lymphoplasmacytic infiltrate, fibrosis arranged, at least focally, in a storiform pattern, and obliterative phlebitis, associated with increased numbers of IgG4+ plasma cells or an increased IgG4/IgG ratio in tissue. In this disease, the serum IgG4 levels are usually increased. The objective of this article is to report the case of a 37-year-old male patient who presented a pulsatile headache associated with diplopia and blurred vision. After radiological, histopathological and immunohistochemical studies, the diagnosis of IgG4-RHP was confirmed, besides presenting a literature review about IgG4-RD and IgG4-RHP.
肥厚性厚性脑膜炎,igg4相关疾病1例报告
IgG4相关疾病(IgG4- rd)是一种全身性疾病,最近的特征是一种炎症性疾病,通常与血清IgG4水平升高有关,IgG4是免疫球蛋白(IgG)的一个亚类,对应于血清总IgG的不足6%,具有独特的组织病理学特征。中枢神经系统的受累是罕见的,可能是孤立的或与其他器官联合,模拟肿瘤。通常累及脑垂体,以脑垂体炎为主要表现,但也可累及硬脑膜,表现为igg4相关性肥厚性厚膜脑膜炎(IgG4-RHP)。神经系统表现是肿块效应的结果,通常是由于血管或神经结构受到压迫,根据病变的解剖部位导致功能缺陷。主要的组织病理学特征为密集的淋巴浆细胞浸润,纤维化排列,至少是局部的,呈层状,闭塞性静脉炎,与组织中IgG4+浆细胞数量增加或IgG4/IgG比值增加有关。在这种疾病中,血清IgG4水平通常升高。这篇文章的目的是报告一个37岁的男性病人谁提出搏动性头痛与复视和视力模糊。经过影像学、组织病理学和免疫组化检查,确认了IgG4-RHP的诊断,并对有关IgG4-RD和IgG4-RHP的文献进行了综述。
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来源期刊
Jornal Brasileiro de Patologia e Medicina Laboratorial
Jornal Brasileiro de Patologia e Medicina Laboratorial Health Professions-Medical Laboratory Technology
CiteScore
1.30
自引率
0.00%
发文量
0
审稿时长
20 weeks
期刊介绍: The Jornal Brasileiro de Patologia e Medicina Laboratorial (Brazilian Journal of Pathology and Laboratory Medicine), a continuation of Jornal Brasileiro de Patologia (Brazilian Journal of Pathology), and published quarterly (March, June, September and December) is directed towards the publication of scientific articles that contribute to the development of the area of Laboratory Medicine (Clinical Pathology, Pathology, Cytopathology). It accepts the following categories of articles: original articles, review articles, case reports, short communications, updating articles, letters to editors and reviews.
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