Department of Pulmonary and Critical Care Medicine, Shengjing Hospital of China Medical University, Shenyang, China.
Department of Intensive Care Unit, The Fourth People's Hospital of Shenyang, Shenyang, China.
BMC Pulm Med. 2022 Aug 9;22(1):305. doi: 10.1186/s12890-022-02094-9.
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a systemic disease that involves the infiltration of IgG4-positive plasma cells in multiple organs. Kimura disease (KD) presents as subcutaneous masses on the head and neck, frequently accompanied by eosinophilia and high immunoglobulin E (IgE) levels. Here, we report a rare case of concurrence of IgG4-RD and KD with manifestations of asthma, pulmonary embolism, and central diabetes insipidus accompanied by lung carcinoma.
A 65-year-old Chinese male with an eight-year history of KD was admitted to our hospital with complaints of dyspnea and expectoration for one month. Laboratory examination showed a considerable elevation in the serum eosinophil count and total IgE and IgG4 levels. Chest enhanced computed tomography showed filling defects in the right pulmonary artery and a nodule in the left inferior lobe. Pancreatic enhanced magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography showed a swollen pancreatic tail and local stricture of the pancreatic duct section of the common bile duct. Enhanced MRI of the pituitary gland showed thickening of the pituitary stalk. Additionally, immunohistochemistry of the specimens collected eight years prior revealed IgG4-positive cells. Following the diagnosis of IgG4-RD with KD, glucocorticoids with immunosuppressants were initiated; there was a prompt improvement in the patient's condition. One-year post-discharge, the patient underwent wedge-shaped resection of the lung due to enlargement of the pulmonary nodule, and the pathology revealed lung squamous carcinoma.
This case presents a rare clinical condition in which the concurrence of IgG4-RD and KD causes various rare manifestations including asthma, pulmonary embolism, central diabetes insipidus, and complicated lung carcinoma. This highlights the importance of monitoring for malignancies in IgG4-RD patients during follow-up.
免疫球蛋白 G4（IgG4）相关疾病（IgG4-RD）是一种全身性疾病，涉及多个器官中 IgG4 阳性浆细胞的浸润。Kimura 病（KD）表现为头颈部皮下肿块，常伴有嗜酸性粒细胞增多和高免疫球蛋白 E（IgE）水平。在这里，我们报告了一例罕见的 IgG4-RD 和 KD 合并病例，表现为哮喘、肺栓塞和中枢性尿崩症，并伴有肺癌。
一名 65 岁的中国男性，有 8 年 KD 病史，因呼吸困难和咳痰 1 个月入院。实验室检查显示血清嗜酸性粒细胞计数、总 IgE 和 IgG4 水平显著升高。胸部增强 CT 显示右肺动脉充盈缺损和左肺下叶结节。胰腺增强 MRI 和磁共振胰胆管成像显示胰尾肿胀和胆总管胰段局部狭窄。垂体增强 MRI 显示垂体柄增粗。此外，8 年前采集的标本免疫组化显示 IgG4 阳性细胞。诊断为 IgG4-RD 合并 KD 后，开始使用糖皮质激素联合免疫抑制剂；患者病情迅速改善。出院后 1 年，因肺结节增大行楔形切除术，病理显示肺鳞癌。
本例罕见的临床表现为 IgG4-RD 和 KD 合并导致各种罕见表现，包括哮喘、肺栓塞、中枢性尿崩症和合并肺癌。这强调了在随访中监测 IgG4-RD 患者恶性肿瘤的重要性。
