Respiratory Medicine Department, Bedford Hospital, Bedfordshire Hospitals NHS Foundation Trust, Bedford, Bedfordshire, UK
Respiratory Medicine Department, Bedford Hospital, Bedfordshire Hospitals NHS Foundation Trust, Bedford, Bedfordshire, UK.
BMJ Case Rep. 2021 Feb 23;14(2):e239976. doi: 10.1136/bcr-2020-239976.
IgG4-related disease is a rheumatological disorder, affecting multiple organ systems, and displaying dense lymphoplasmacytic inflammatory infiltrate and storiform fibrosis on histology. The pulmonary manifestations of IgG4-related disease are varied. Most commonly, bronchovascular consolidation and perilymphatic parenchymal thickening occur. IgG4-related disease can present as a solitary parenchymal mass and is often mistaken for a primary pulmonary or haematological malignancy. This report presents a case of IgG4-related disease in a patient with 6 months reported haemoptysis symptomatology and CT findings of perihilar lymphadenopathy, multiple pulmonary parenchymal nodules and a single parenchymal mass. Clinician cognisance of the histopathological presentation of IgG4-disease is important. It should be considered as a differential in patients for which investigations have ruled out malignancy, as it is responsive to glucocorticoids in the majority of cases.
IgG4 相关疾病是一种风湿性疾病,影响多个器官系统,组织学表现为密集的淋巴浆细胞炎症浸润和席纹状纤维化。IgG4 相关疾病的肺部表现多种多样。最常见的是支气管血管实变和肺周实质增厚。IgG4 相关疾病可表现为局灶性实质肿块,常被误诊为原发性肺或血液系统恶性肿瘤。本报告介绍了一例 IgG4 相关疾病患者,该患者有 6 个月的咯血症状,CT 表现为肺门周围淋巴结病、多个肺部实质结节和单个实质肿块。临床医生对 IgG4 疾病的组织病理学表现有认识非常重要。在排除恶性肿瘤的情况下,应将其作为鉴别诊断的一种,因为在大多数情况下,它对糖皮质激素有反应。