一种模仿肺癌的罕见病理:IgG4 相关性血管炎

IF 0.8 Q4 RESPIRATORY SYSTEM Respirology Case Reports Pub Date : 2024-10-10 eCollection Date: 2024-10-01 DOI:10.1002/rcr2.70039
Coskun Ardan Sener, Aylin Ozgen Alpaydın, Oguz Kılınc
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引用次数: 0

摘要

免疫球蛋白-G4(IgG4)相关疾病本质上是一种纤维炎症性疾病,可同时或在不同时间影响任何器官。这种疾病通常表现为模仿肿瘤的器官增生,可累及泪腺、主要唾液腺、胰腺、胆管、腹膜后区域、肺、肾、主动脉、脑膜和甲状腺。这种新疾病背后的免疫发病机制尚未阐明。该病的组织病理学特征包括以 IgG4 阳性浆细胞为主的密集淋巴浆细胞浸润、柱状纤维化和闭塞性静脉炎。免疫球蛋白 G4 病(IgG4-RD)是最近发现的一种罕见的全身性纤维炎性疾病,估计全球发病率不到十万分之一。我们的病例是通过肺细针穿刺活检确诊为 IGG4 相关性血管炎,这在文献中非常罕见。
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A rare pathology that mimics lung cancer: IgG4-related vasculitis.

Immunoglobulin-G4 (IgG4)-related disease is essentially a fibro-inflammatory disease that can affect any organ simultaneously or at different times. The disease usually presents with organ growth that mimics a tumour and can affect the lacrimal glands, major salivary glands, pancreas, bile ducts, retroperitoneal area, lungs, kidneys, aorta, meninges and thyroid gland. The immunopathogenesis behind this new disease has not yet been elucidated. Histopathological distinguishing features of the disease include dense lymphoplasmocytic infiltrates dominated by IgG4 positive plasma cells, storiform fibrosis and obliterative phlebitis. The likelihood of developing with immunoglobulin G4 (IgG4-RD) is a recently identified rare systemic fibroinflammatory disease with an estimated incidence of less than 1 in 100,000 people worldwide. We present our case, which was diagnosed with IGG4-related vasculitis by lung fine needle aspiration biopsy, which is very rare in the literature.

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来源期刊
Respirology Case Reports
Respirology Case Reports RESPIRATORY SYSTEM-
CiteScore
1.40
自引率
0.00%
发文量
178
审稿时长
8 weeks
期刊介绍: Respirology Case Reports is an open-access online journal dedicated to the publication of original clinical case reports, case series, clinical images and clinical videos in all fields of respiratory medicine. The Journal encourages the international exchange between clinicians and researchers of experiences in diagnosing and treating uncommon diseases or diseases with unusual presentations. All manuscripts are peer-reviewed through a streamlined process that aims at providing a rapid turnaround time from submission to publication.
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