Servicio de Radiodiagnóstico, Hospital Clínico San Carlos, Madrid, Spain.
Servicio de Radiodiagnóstico, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Radiologia (Engl Ed). 2022 Dec;64 Suppl 3:265-276. doi: 10.1016/j.rxeng.2022.09.005.
The term cystic lung disease encompasses a heterogeneous group of entities characterised by round lung lesions that correspond to cysts with fine walls, which usually contain air. The differential diagnosis of these lesions can be challenging, requiring both clinical and radiological perspectives. Entities such as pulmonary emphysema and cystic bronchiectasis can simulate cystic disease. High-resolution computed tomography (HRCT) is the imaging technique of choice for the evaluation and diagnosis of cystic lung disease, because it confirms the presence of lung disease and establishes the correct diagnosis of the associated complications. In many cases, the diagnosis can be established based on the HRCT findings, thus making histologic confirmation unnecessary. For these reasons, radiologists need to be familiar with the different presentations of these entities. A wide variety of diseases are characterised by the presence of diffuse pulmonary cysts. Among these, the most common are lymphangioleiomyomatosis, which may or may not be associated with tuberous sclerosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. Other, less common entities include Birt-Hogg-Dubé syndrome, amyloidosis, and light-chain deposit disease. This article describes the characteristics and presentations of some of these entities, emphasizing the details that can help differentiate among them.
囊性肺病涵盖一组异质性实体,其特征为圆形肺病变,对应薄壁囊肿,通常包含空气。这些病变的鉴别诊断具有挑战性,需要临床和影像学观点。肺气肿和囊性支气管扩张等实体可以模拟囊性疾病。高分辨率计算机断层扫描(HRCT)是评估和诊断囊性肺病的首选成像技术,因为它确认存在肺病并确立相关并发症的正确诊断。在许多情况下,根据 HRCT 结果即可诊断,因此无需组织学确认。出于这些原因,放射科医生需要熟悉这些实体的不同表现。许多疾病的特征是弥漫性肺囊肿。其中最常见的是淋巴管平滑肌瘤病,可能与结节性硬化症有关,也可能无关,朗格汉斯细胞组织细胞增生症和淋巴细胞性间质性肺炎。其他不太常见的实体包括 Birt-Hogg-Dubé 综合征、淀粉样变性和轻链沉积病。本文描述了其中一些实体的特征和表现,强调了有助于区分它们的细节。