Sawahata Michiru, Yamaguchi Tetsuo
Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Tochigi, Japan.
Department of Respiratory Medicine, Shinjuku Tsurukame Clinic, Tokyo, Japan.
Sarcoidosis Vasc Diffuse Lung Dis. 2022;39(2):e2022018. doi: 10.36141/svdld.v39i2.12995. Epub 2022 Jun 29.
In pulmonary sarcoidosis, respiratory tract lesions almost always appear, and residual lung shadows require treatment in about 20% of cases. Pulmonary fibrosis is among the three leading causes of death. Treatment strategies are urgently needed to inhibit the progression of pulmonary fibrosis by combining antifibrotic drugs and immunosuppressive drugs such as corticosteroids. Establishing consensus on the process of pulmonary fibrosis progression is important for determining the most effective treatment.
Among more than 2500 cases of sarcoidosis treated at our hospital, cases that led to chronic respiratory failure were analyzed for CT findings of pulmonary fibrosis. Early in sarcoidosis, granulomatous lesions appeared along the bronchovascular bundle. As pulmonary fibrosis progressed, a central consolidation developed on the central side in the direction of lymph flow, a peripheral consolidation developed on the pleural side, and a central-peripheral band developed connecting the two. Infiltrative or wedge-shaped shadows sometimes formed in the immediate subpleural area, appearing as a pleuroparenchymal fibroelastosis-like lesion. Traction bronchiectasis may form cysts at the periphery or may congregate to form a honeycomb lung-like structure. Combination of these lesions led to shrinkage of the upper lobe. Patients with multiple peripheral cysts/bullae had a unique disease course characterized by wheezing and concomitant pulmonary hypertension and pulmonary aspergillosis.
Further understanding of the process of pulmonary fibrosis progression is needed. Summarizing imaging findings and understanding their contribution to respiratory impairment will contribute to comprehensively evaluating the stages of pulmonary fibrosis progression and establishing an optimal treatment strategy.
在肺结节病中,呼吸道病变几乎总会出现,约20%的病例残留肺部阴影需要治疗。肺纤维化是主要死因之一。迫切需要通过联合抗纤维化药物和免疫抑制药物(如皮质类固醇)来抑制肺纤维化进展的治疗策略。就肺纤维化进展过程达成共识对于确定最有效的治疗方法很重要。
在我院治疗的2500多例结节病病例中,对导致慢性呼吸衰竭的病例进行了肺纤维化的CT表现分析。在结节病早期,沿支气管血管束出现肉芽肿性病变。随着肺纤维化进展,在淋巴引流方向的中央侧出现中央实变,在胸膜侧出现外周实变,并且形成连接两者的中央 - 外周带。有时在紧邻胸膜下区域形成浸润性或楔形阴影,表现为胸膜实质纤维弹性组织增生样病变。牵拉性支气管扩张可能在周边形成囊肿,或聚集形成蜂窝肺样结构。这些病变的组合导致上叶缩小。有多个外周囊肿/肺大疱的患者有独特的病程,其特征为喘息并伴有肺动脉高压和肺曲霉菌病。
需要进一步了解肺纤维化进展过程。总结影像学表现并了解它们对呼吸功能损害的作用将有助于全面评估肺纤维化进展阶段并制定最佳治疗策略。