Chirila Silviu Mihail
Universitätsspital Basel Klinik für Pneumologie Petersgraben 4 4031 Basel.
Ther Umsch. 2024 Feb;81(1):16-20.
Diffuse cystic lung disease (DCLD) represents a heterogeneous group of conditions, typically characterized by the presence of multiple thin-walled, predominantly round parenchymal lucencies. The increased accessibility of computed tomography (CT) underscores the growing relevance of a relatively rare group of diseases as more clinicians are confronted with the presence of multiple lung cysts on the chest CT scan. Although the etiology of these conditions is very diverse, the focus of the differential diagnosis revolves around four primary causative factors - Lymphangioleiomyomatosis (LAM), Pulmonary Langerhanscell histiocytosis (PLCH), Birt-Hogg-Dubé (BHD) and lymphoid interstitial pneumonia (LIP). Achieving an accurate diagnosis poses a challenge and typically necessitates lung biopsies; however, it is crucial for ensuring proper management.
弥漫性囊性肺疾病（DCLD）是一组异质性疾病，其典型特征是存在多个薄壁、主要为圆形的实质透亮区。计算机断层扫描（CT）检查的普及使得一组相对罕见的疾病越来越受到关注，因为越来越多的临床医生在胸部CT扫描中发现多个肺囊肿。尽管这些疾病的病因非常多样，但鉴别诊断的重点围绕四个主要致病因素——淋巴管平滑肌瘤病（LAM）、肺朗格汉斯细胞组织细胞增多症（PLCH）、Birt-Hogg-Dubé综合征（BHD）和淋巴样间质性肺炎（LIP）。实现准确诊断具有挑战性，通常需要进行肺活检；然而，这对于确保正确的治疗至关重要。
