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免疫球蛋白G4相关疾病的组织病理学标志物:分析诊断模式与免疫组化结果

Histopathological Markers of Immunoglobulin G4-related Disease: Analyzing Diagnostic Patterns and Immunohistochemical Findings.

作者信息

Shanmugasundaram Sakthisankari, Michael Anita

机构信息

Department of Pathology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India.

PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India.

出版信息

Int J Surg Pathol. 2025 Sep;33(6):1393-1400. doi: 10.1177/10668969251316911. Epub 2025 Feb 23.

Abstract

Immunoglobulin G4 (IgG4) related disease is a localized or multisystem chronic inflammatory disease with characteristic pathological features. Several consensus criteria are available to aid in the diagnosis and its management. These criteria are highly specific, owing to their dependency on major histopathological criteria and laboratory investigations to diagnose IgG4-related diseases (IgG4-RD). We evaluated the lesions diagnosed as IgG4-RDs with histopathological scoring criteria based on histopathology, immunohistochemistry, and serology in the screening of IgG4-RDs. The lesions diagnosed as IgG4-RD during the study period were evaluated using the histopathological scoring criteria which include storiform fibrosis, obliterative phlebitis, plasmacytic infiltrates, and lymphoid follicles. Immunohistochemistry with IgG4 was performed on all these lesions. There were twelve patients with IgG4-RD involving the retroperitoneum, pancreas, lymph node, and nasal sinuses. Lesions involving the retroperitoneum showed all the characteristic histopathological findings of IgG4-RD. Storiform fibrosis and obliterative vasculitis were seen in 67% and 58% of the lesions respectively. Two patients (17%) lacked both storiform fibrosis and obliterative phlebitis. Additionally, lymphoid follicles were present in 58%, perineural infiltration in 33%, and tissue eosinophilia in 58% of individuals. Two patients with eosinophilic angiocentric fibrosis and a single patient with IgG4-RD associated with retroperitoneal mucinous cystadenoma were identified. IgG4-RD is a group of rare diseases that needs a multidisciplinary team approach for diagnosis. In our study, we report a rare association of IgG4-RD and retroperitoneal mucinous cystadenoma as well as rare lesions of IgG4-related eosinophilic angiocentric fibrosis. Clinicopathological and radiological correlation is crucial in the accurate diagnosis of IgG4-RD and to avoid overdiagnosis.

摘要

免疫球蛋白G4(IgG4)相关疾病是一种具有特征性病理表现的局限性或多系统慢性炎症性疾病。有多种共识标准可用于辅助其诊断和管理。这些标准具有高度特异性,因为它们依赖主要组织病理学标准和实验室检查来诊断IgG4相关疾病(IgG4-RD)。我们在IgG4-RD的筛查中,使用基于组织病理学、免疫组织化学和血清学的组织病理学评分标准评估诊断为IgG4-RD的病变。在研究期间诊断为IgG4-RD的病变,使用包括席纹状纤维化、闭塞性静脉炎、浆细胞浸润和淋巴滤泡的组织病理学评分标准进行评估。对所有这些病变进行IgG4免疫组织化学检测。有12例IgG4-RD患者累及腹膜后、胰腺、淋巴结和鼻窦。累及腹膜后的病变表现出IgG4-RD所有特征性组织病理学表现。分别在67%和58%的病变中见到席纹状纤维化和闭塞性血管炎。2例患者(17%)既没有席纹状纤维化也没有闭塞性静脉炎。此外,58%的个体存在淋巴滤泡,33%存在神经周围浸润,58%存在组织嗜酸性粒细胞增多。鉴定出2例嗜酸性血管中心性纤维化患者和1例与腹膜后黏液性囊腺瘤相关的IgG4-RD患者。IgG4-RD是一组罕见疾病,需要多学科团队方法进行诊断。在我们的研究中,我们报告了IgG4-RD与腹膜后黏液性囊腺瘤的罕见关联以及IgG4相关嗜酸性血管中心性纤维化的罕见病变。临床病理和影像学相关性对于IgG4-RD的准确诊断及避免过度诊断至关重要。

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