肺淋巴管平滑肌瘤5例报道及文献复习

5 cases of pulmonary lymphangileiomyomatosis and literature review

目的分析肺淋巴管平滑肌瘤病(pulmonary lymphangioleiomyomatosis,PLAM)的临床表现、高分辨率体层摄影(high-resolution tomography,HRCT)的特点、诊断、鉴别诊断及治疗方法。方法选择2017年12月至2022年2月青海大学附属医院收治的5例肺淋巴管平滑肌瘤病患者,分析相关资料及文献。结果5例肺淋巴管平滑肌瘤病患者中,平均年龄40.6岁,女性,育龄期占60%(3/5),绝经期占40%(2/5)。HRCT表现为双肺弥漫分布的多发囊状影。主要临床症状为活动后气促、呼吸困难、咳嗽等。5例PLAM患者中,散发型淋巴管肌瘤病(S-LAM)占80%(4/5),其中1例仅肺脏受累,3例为多系统受累,表现为肺、肾、肝、腹盆腔等病变。结节硬化症相关淋巴管肌瘤病(TSC-LAM)占20%(1/5),合并肺外表现:肝、肾、骨、皮肤等病变,为多系统受累。结论PLAM是一种罕见的肺部疾病,HRCT具有特异性,为本病的诊断和鉴别提供重要的影像学依据,本文5例患者胸部CT有共同点,表现为双肺弥漫分布的含气囊腔,边缘可见周围血管征,目前尚无有效治疗方法,免疫抑制剂可以使一些患者的病情改善,病例1患者服用依维莫司,可抑制由于mTOR(哺乳动物雷帕霉素靶蛋白)被激活后引起的LAM细胞过度增值,延缓病情进展,终末期时可考虑肺移植。

Objective To explore the clinical manifestations,features,diagnosis,differential diagnosis and treatment methods of pulmonary lymphangioleiomyomatosis(PLAM),high-resolution tomography(HRCT).Methods The relevant data and literature of 5 patients with pulmonary lymphangioleiomyomatosis admitted by the Affiliated Hospital of Qinghai University from December 2017 to February 2022 were collected for case analysis.Results In this study,the average age of 5 patients with pulmonary lymphangioleiomyomatosis was 40.6 years,all of whom were female,accounting for 60%(3/5)of childbearing age and 40%(2/5)of menopausal women.HRCT all showed multiple cystic opacities with diffuse distribution in both lungs.The main clinical symptoms are shortness of breath after activity,dyspnea,cough,etc.Among the 5 patients with PLAM,sporadic lymphangiomyomatosis(S-LAM)accounted for 80%(4/5),of which 1 case was only lung involvement,and 3 cases were multisystem involvement,showing lung,kidney,liver,abdominal-pelvic cavity and other lesions.Tuberrosclerosis-associated lymphangiomyopathy(TSC-LAM)accounts for 20%(1/5),with extrapulmonary manifestations:liver,kidney,bone,skin and other lesions,which are multisystem involvement.Conclusions PLAM is a rare lung disease,HRCT has specificity,providing an important imaging basis for the diagnosis and identification of this disease,Chest CT in all 5 patients in this study had common features,all manifested as diffuse distribution of both lungs containing air cyst cavity,peripheral vascular signs can be seen at the edge,there is currently no effective treatment,immunosuppressants can improve the condition of some patients,case 1 patients take everolimus,It can inhibit the hyperplasia of LAM cells caused by activation of mTOR(mammalian target protein of rapamycin),delay disease progression,and consider lung transplantation in the terminal stage.