Institute of Radiology, Department of Medicine, University of Udine, "S. Maria della Misericordia" University Hospital, p.le S. Maria della Misericordia, 15 - 33100 Udine, Italy.
Institute of Radiology, Department of Medicine, University of Udine, "S. Maria della Misericordia" University Hospital, p.le S. Maria della Misericordia, 15 - 33100 Udine, Italy.
Curr Probl Diagn Radiol. 2022 Sep-Oct;51(5):759-767. doi: 10.1067/j.cpradiol.2021.07.007. Epub 2021 Aug 29.
The term "connective tissue diseases" (CTDs) refers to a heterogeneous group of autoimmune disorders, including systemic sclerosis, rheumatoid arthritis, Sjögren's syndrome, systemic lupus erythematosus, polymyositis, dermatomyositis, antisynthetase syndrome, and mixed connective tissue disease. Chest high-resolution computed tomography (HRCT) is the imaging method of choice for evaluating patients with known or suspected CTD-related interstitial lung disease (CTD-ILD), a complication accounting for substantial morbidity and mortality. While specific HRCT patterns and signs of CTD-ILD have been extensively described (hence the designation "the usual suspects"), the knowledge of various, less frequent conditions involving the lungs in patients with CTD would help the radiologist produce a clinically valuable report, thus potentially influencing patient management. This paper aims to provide an up-to-date review of various unusual pulmonary CTD-related conditions the radiologist should be aware of; namely, acute exacerbation of CTD-ILD, CTD-related interstitial lung abnormalities, lung amyloidosis, MALT lymphoma, antisynthetase syndrome, pleuroparenchymal fibroelastosis-like lesion, drug-induced ILD, combined pulmonary fibrosis and emphysema, and pulmonary hypertension. For each condition, the chest HRCT appearance and the key histopathological and clinical features are resumed, helping the radiologist participate actively in the multidisciplinary discussion of complex clinical cases.
“结缔组织疾病”(CTD)这一术语是指一组异质性自身免疫性疾病,包括系统性硬化症、类风湿关节炎、干燥综合征、系统性红斑狼疮、多发性肌炎、皮肌炎、抗合成酶综合征和混合性结缔组织病。胸部高分辨率计算机断层扫描(HRCT)是评估已知或疑似 CTD 相关间质性肺疾病(CTD-ILD)患者的首选影像学方法,这是一种发病率和死亡率都很高的并发症。虽然 CTD-ILD 的特定 HRCT 模式和特征已被广泛描述(因此被称为“常见嫌疑者”),但了解 CTD 患者肺部的各种罕见情况,将有助于放射科医生提供有临床价值的报告,从而可能影响患者的管理。本文旨在提供一份关于放射科医生应了解的各种不常见的与 CTD 相关的肺部疾病的最新综述,即 CTD-ILD 的急性加重、CTD 相关的间质性肺异常、肺淀粉样变性、MALT 淋巴瘤、抗合成酶综合征、胸膜实质纤维弹性样病变、药物诱导的ILD、合并性肺纤维化和肺气肿以及肺动脉高压。对于每种情况,总结了胸部 HRCT 表现和关键的组织病理学和临床特征,有助于放射科医生积极参与复杂临床病例的多学科讨论。