Department of General Internal Medicine 1, Kawasaki Medical School, Okayama, Japan.
Department of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Kurashiki, Japan.
Medicine (Baltimore). 2022 Aug 26;101(34):e30285. doi: 10.1097/MD.0000000000030285.
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a systemic immune-mediated condition that can cause fibroinflammatory lesions in multiple organs. Approximately 35% of IgG4-RD patients have some symptoms in the chest and IgG4-related lung disease (IgG4-RLD) is observed in about 10% of IgG4-RD cases. In addition, it is thought that glucocorticoid therapy is effective for IgG4-RD and IgG4-RLD. It is difficult to diagnose IgG4-RLD complicated with another lung disease.
An 85-year-old Japanese man was hospitalized due to pulmonary consolidations just below the pleura in chest computed tomography while being treated with antibiotics. Previously, an upper lobectomy of the right lung was performed for an upper lung mucinous adenocarcinoma, and he was diagnosed with chronic obstructive pulmonary disease. Although he took antibiotics before admission, C-reactive protein levels were elevated.
IgG4 levels were also elevated (IgG4; 733 mg/dL), and lung biopsy histology showed an abundance of IgG4-positive plasma cell infiltration; about 40% of the affected area was occupied by such infiltration. Based on such findings, we finally diagnosed him as IgG4-RLD.
We administered 20 mg/d prednisolone.
About 2 weeks after administration of prednisolone by intravenous injection, his multifocal pulmonary consolidations just below the pleura were markedly improved and his pulmonary symptoms disappeared. Four weeks after glucocorticoid therapy, IgG4 levels decreased from 831 mg/dL (peak) to 547 mg/dL.
We should consider IgG4-RLD, a rare disease, when lesions are detected as pulmonary consolidations near the pleura and are unresponsive to antibiotic therapy. Glucocorticoid therapy, however, is very effective for such IgG4-RLD.
免疫球蛋白 G4（IgG4）相关疾病（IgG4-RD）是一种系统性免疫介导的疾病，可导致多个器官发生纤维炎症性病变。大约 35%的 IgG4-RD 患者有一些胸部症状，约 10%的 IgG4-RD 病例观察到 IgG4 相关肺部疾病（IgG4-RLD）。此外，人们认为糖皮质激素治疗对 IgG4-RD 和 IgG4-RLD 有效。诊断 IgG4-RLD 合并另一种肺部疾病具有一定难度。
一名 85 岁的日本男性因胸部计算机断层扫描显示胸膜下肺部实变而住院，此前他因右上肺粘液性腺癌接受了右上肺叶切除术，同时患有慢性阻塞性肺病。尽管他在入院前使用了抗生素，但 C 反应蛋白水平仍升高。
IgG4 水平也升高（IgG4；733mg/dL），肺活检组织学显示大量 IgG4 阳性浆细胞浸润；受影响区域的约 40%被这种浸润所占据。基于这些发现，我们最终诊断为 IgG4-RLD。
我们给予 20mg/d 泼尼松龙。
静脉注射泼尼松龙约 2 周后，他的胸膜下多灶性肺部实变明显改善，肺部症状消失。糖皮质激素治疗 4 周后，IgG4 水平从 831mg/dL（峰值）降至 547mg/dL。
当在胸膜附近发现肺部实变且对抗生素治疗无反应时，我们应考虑罕见疾病 IgG4-RLD。然而，糖皮质激素治疗对这种 IgG4-RLD 非常有效。
