Iijima Yoshihito, Iwai Shun, Motono Nozomu, Usuda Katsuo, Shioya Akihiro, Takeuchi Shingo, Yamagishi Shigeki, Koizumi Kiyoshi, Yamada Sohsuke, Uramoto Hidetaka
Department of Thoracic Surgery, Kanazwa Medical University, 1-1 Daigaku, Uchinada-machi, Kahoku-gun, 920-0293, Ishikawa, Japan.
Department of Pathology and Laboratory Medicine, Kanazwa Medical University, Ishikawa, Japan.
Surg Case Rep. 2021 Apr 7;7(1):84. doi: 10.1186/s40792-021-01166-y.
Immunoglobulin G4 (IgG4)-related diseases are characterized by abnormal IgG4 levels, swelling, and marked infiltration and fibrosis of the lymphocytes and IgG4-positive plasma cells, causing hypertrophic lesions or nodules. The cause is currently not well understood. IgG4-related diseases involving lesions limited to the pleura are extremely rare. Herein, we report an IgG4-related disease presenting with multiple pleural nodules confirmed by thoracoscopic surgical biopsy.
A 74 year-old man was referred to our department for definitive diagnosis of multiple pleural nodules after 1 year of follow-up. Computed tomography of the chest revealed multiple pleural nodules, while 2-deoxy-2-( F)-fluorodeoxyglucose positron emission tomography imaging exhibited tracer accumulation in the nodules. A thoracoscopic surgical biopsy was performed. Histopathological examination revealed hyalinized fibrous tissue with a high degree of plasma cell-based inflammatory cell infiltration. Immunohistochemically, IgG4-positive cells were conspicuous, accounting for 70.5% of the plasma cells. The postoperative serum IgG4 concentration was 289 mg/dL. We diagnosed the patient with an IgG4-related disease with multiple pleural nodules. The postoperative course was good, and the patient is currently being followed up.
IgG4-related disease should be considered in cases presenting with multiple pleural nodules.
免疫球蛋白G4（IgG4）相关疾病的特征是IgG4水平异常、肿胀以及淋巴细胞和IgG4阳性浆细胞的显著浸润和纤维化，导致肥厚性病变或结节。目前其病因尚不清楚。IgG4相关疾病累及仅限于胸膜的病变极为罕见。在此，我们报告一例经胸腔镜手术活检确诊为IgG4相关疾病并伴有多个胸膜结节的病例。
一名74岁男性在随访1年后因多个胸膜结节的明确诊断被转诊至我科。胸部计算机断层扫描显示多个胸膜结节，而2-脱氧-2-（F）-氟脱氧葡萄糖正电子发射断层扫描成像显示结节中有示踪剂积聚。进行了胸腔镜手术活检。组织病理学检查显示透明变性的纤维组织，伴有高度的以浆细胞为主的炎性细胞浸润。免疫组织化学检查显示IgG4阳性细胞明显，占浆细胞的70.5%。术后血清IgG4浓度为289mg/dL。我们诊断该患者为伴有多个胸膜结节的IgG4相关疾病。术后病程良好，患者目前正在接受随访。
对于出现多个胸膜结节的病例应考虑IgG4相关疾病。
