Clinical Analysis Of 7 Cases Of Pulmonary Lymphangioleiomyomatosis

Objective: To investigate the clinical feature, diagnosis and treatment of pulmonary lymphangioleiomyomatosis(PLAM), to improve the diagnosis of PLAM.Method: Seven cases with PLAM demonstrated by lung biopsy or high resolution computerized tomography(HRCT)scanning were analysed and followed-up from 2 to 50 months.Result: The patients were all women of reproductive age. Progressive dyspnea was the major clinical manifestation of PLAM, followed by pneumothorax, chest tightness, cough, chylothorax, hemoptysis, chyloperitoneum and infection. Tow patients received medroxyprogesterone died from respiratory failure; one patient who had undergone singal lung transplantation, died from pneumonia three years later; one patient takeing rapamycin was in stable condition; one case of long-term low-dose erythromycin, disease progress; three patients with pneumothorax who had undergone operation of pleural adhesion fixation ,did not recur after operation.Conclusion: Pulmonary lymphangioleiomyomatosis, which is easily misdiagnosised, occurs almost in women of reproductive age. High resolution computerized tomography(HRCT) is the most accurate non-invasion examination. We suggest taking rapamycin for targeted therapy. Lung transplantation must be considered when chronic respiratory failure occurs. Pleural adhesion fixation is recommendations for patients with recurrent pneumothorax.