Kulwiec E L, Lynch D A, Aguayo S M, Schwarz M I, King T E
Department of Radiology, University of Colorado Health Sciences Center, Denver 80262.
Radiographics. 1992 May;12(3):515-26. doi: 10.1148/radiographics.12.3.1609142.
Pulmonary histiocytosis X is an uncommon but important cause of pulmonary fibrosis and honeycombing in young adults. This article reviews the pathologic, clinical, radiographic, and high-resolution computed tomographic (HRCT) features of pulmonary histiocytosis X, focusing on differential diagnosis and disease progression. The main pathologic feature of pulmonary histiocytosis X is peribronchiolar inflammation, leading to fibrosis and cyst formation. Although the diagnosis of pulmonary histiocytosis X may be suspected on the basis of chest radiographic findings, the HRCT findings of predominantly upper lobe nodules and cysts are virtually pathognomonic of this disorder. As pulmonary histiocytosis X progresses, the nodules decrease in number, leaving multiple thin-walled cysts. HRCT can be useful in visually estimating the proportion of lung involved and is also valuable in distinguishing histiocytosis from other disorders that produce nodules or cysts. Chest HRCT helps confirm the pattern seen at radiography and is valuable in establishing the correct diagnosis.
肺组织细胞增多症X是导致年轻成人肺纤维化和蜂窝肺的一个不常见但重要的原因。本文回顾了肺组织细胞增多症X的病理、临床、影像学及高分辨率计算机断层扫描（HRCT）特征，重点关注鉴别诊断和疾病进展。肺组织细胞增多症X的主要病理特征是细支气管周围炎症，导致纤维化和囊肿形成。虽然根据胸部X线表现可能怀疑肺组织细胞增多症X的诊断，但以主要位于上叶的结节和囊肿为特征的HRCT表现实际上对这种疾病具有诊断意义。随着肺组织细胞增多症X的进展，结节数量减少，留下多个薄壁囊肿。HRCT有助于直观评估受累肺的比例，在鉴别组织细胞增多症与其他产生结节或囊肿的疾病方面也很有价值。胸部HRCT有助于确认X线所见的模式，对确立正确诊断很有价值。
