Clinical analysis of 48 cases with pulmonary lymphangioleiomyomatosis:a report of three cases and review of domestic literatures published in the past ten years / 中华全科医师杂志

ABSTRACT

Objective To improve the understanding and diagnosis of pulmonary lymphangioleiomyomatosis (PLAM) by the comprehensive review of domestic literatures in the past ten years.Methods Three new cases with PLAM were reported and integraed with other 45 cases reported domestically in the past ten years for analysis of their clinical features.Results The newly reported three cases of PLAM were all women at child-bearing age, with initial symptom of dyspnea after activity. Two of them complicated with extra-pulmonary PLAM. All the three cases were free of chylous effusion. Forty-seven of 48 cases with PLAM were pathologically diagnozed, with ages of onset of 5~69 (mean?s of 34?10) years. Their clinical manifestations were mainly respiratory, including dyspnoea (95.8%), haemoptysis (52.1%), pneumothorax (45.8%), chylous effusion (33.3%),cough (31.3%) and chest pain (12.5%). Abnormal manifestations in abdomen, including renal mass, retroperitoneal mass and retroperitoneal lymphadenopathy, were detected in 16 cases. Thirty-nine cases had their high-resolution CT (HRCT) examined and appearance of multiple cysts distributed throughout the bilateral lung fields could be discerned in 38 of them. Obstructive ventilation disturbance could be observed in 23 of 30 cases with the data or conclusions on pulmonary function tests, and mixed ventilation disturbance in seven cases. Respiratory failure was complicated in 17 of 28 cases with the data of arterial blood gas analyses.Conclusions HRCT had confirmative value for diagnosis of PLAM. In practice, HRCT, as well as other routine abdominal and pelvic imaging examinations, should be performed in time for child-bearing-age women with progressive dyspnoea, haemoptysis, or spontaneous pneumothorax, to detect if they complicate with PLAM.