Sener Coskun Ardan, Alpaydın Aylin Ozgen, Kılınc Oguz
Department of Pulmonary Diseases Dokuz Eylül University Faculty of Medicine Hospital Izmir Turkey.
Department of Pulmonary Diseases Economy University Faculty of Medicine Hospital Izmir Turkey.
Respirol Case Rep. 2024 Oct 10;12(10):e70039. doi: 10.1002/rcr2.70039. eCollection 2024 Oct.
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.
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。该疾病通常表现为类似肿瘤的器官肿大,可累及泪腺、大唾液腺、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。该疾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细胞和浆细胞浸润、席纹状纤维化和闭塞性静脉炎。免疫球蛋白 G4(IgG4-RD)是一种最近发现的罕见全身性纤维炎症性疾病,全球估计发病率不到十万分之一。我们报告了我们的病例,该病例通过肺细针穿刺活检被诊断为 IgG4 相关血管炎,这在文献中非常罕见。