肺淋巴管平滑肌瘤病的临床及影像学分析

Clinical and Imaging Manifestations of Patients with Pulmonary Lymphangioleiomyomatosis

目的总结肺淋巴管平滑肌瘤病(PLAM)的临床及影像学表现,提高对PLAM的认识。方法回顾分析2003年至2013年间复旦大学附属中山医院收治的具有较完整资料的18例PLAM确诊病例,并进行相关文献复习。结果 18例均为女性,均有活动后气促,3例有乳糜胸,6例有气胸,5例有咯血。18例高分辨CT(HRCT)均显示肺部弥漫分布薄壁囊腔透光区,符合PLAM的特征性表现。14例经组织病理检查确诊。4例患者的肺功能检查显示阻塞性通气功能障碍,9例(50%)患者被误诊为其他疾病。主要给予对症支持治疗,部分应用抗雌激素治疗。文献显示PLAM是较为少见的一种弥漫性肺部疾病,多见于育龄女性,主要表现为呼吸困难、咯血、气胸和乳糜胸,依靠病史、胸部HRCT和组织病理学检查确诊。HRCT检查对PLAM具有诊断价值,抗雌激素治疗有一定的效果,雷帕霉素给此病的治疗带来了新希望。结论 PLAM有特征性的临床和影像学表现,应早期确诊,有效治疗。

Objective To investigate the clinical and imaging features of patients with pulmonary lymphangioleiomyomatosis （ PLAM）, to improve the knowledge of this disease. Methods Eighteen patients with PLAM admitted into Zhongshan Hospital between 2003 and 2013 were retrospectively analyzed. Relevant literatures were reviewed. Results All the 18 cases were female and had dyspnea on exertion. Three of them suffered from chylothorax,6 from pneumothorax and 5 from hemoptysis. The high resolution CT （HRCT） findings in all the 18 cases showed multiple small cysts with thin wall diffusely distributing in the bilateral lung. PLAM was confirmed by biopsy in 14 cases. Pulmonary function tests in 4 cases showed obstructive ventilatory dysfunction. Nine cases （50%） were misdiagnosed as other diseases. All the patients were given symptomatic and supportive treatment. Literatures review showed that as a rare diffuse lung disease, PLAM is more common in women of childbearing age, mainly presenting with dyspnea, hemoptysis, pneumothorax and chylothorax. Diagnosis of the disease relies on medical history, HRCT features and lung tissue biopsy pathology. HRCT examination has diagnostic value for PLAM. Although anti-estrogen treatment has certain effects, rapamycin may be a promising drug for PLAM therapy. Conclusions Patients with PLAM have characteristic clinical and radiographic manifestations. Early diagnosis and effective treatment are keys to the management of patients with PLAM.