To explore the clinical manifestations, features, diagnosis, differential diagnosis and treatment methods of pulmonary lymphangioleiomyomatosis (PLAM), high-resolution tomography (HRCT).

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.

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.

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.