Clinical analysis of patients with pulmonary lymphangioleiomyomatosis (PLAM) in mainland China

Ye L, Jin M, Bai C.

The Respiratory Dept., Zhongshan Hospital, Fudan University, Shanghai, China.

Abstract

BACKGROUND AND OBJECTIVE: There have been no clinical reports on pulmonary lymphangioleiomyomatosis (PLAM) based on large studies or epidemiological surveys in mainland China. The purpose of this study was to provide a retrospective analysis of PLAM patients in mainland China by reviewing the clinical data of PLAM cases reported.

METHODS: The China Academic Journals Full-text Database search engine was used to collect related cases in mainland China through the end of 2008. 120 cases met the study's inclusion criteria and were reviewed for this analysis.

RESULTS: The average age of the 120 patients upon confirmed diagnosis was 37.3+/-6.4 years. The average duration from onset of symptoms to a confirmed diagnosis was 29.6+/-35.8 months, with 80 person-time patients having experienced misdiagnosis before the confirmed diagnosis. The major clinical manifestations of PLAM included progressive dyspnea, recurrent pneumatothorax, refractory chylothorax. Pulmonary function abnormalities included obstructive pulmonary ventilation disorders and degenerated diffusing capacity. Ten patients were found to be complicated with renal angiomyolipoma and 17 with abdominal or pelvic lymphangioleiomyoma. Half of the patients had undergone antiestrogen therapies such as progesterone, and four patients received pulmonary transplantation. The average duration from the confirmed diagnosis to death was 36.4+/-48.9 months among the 28 cases of death.

CONCLUSIONS: Doctors in mainland China are becoming increasingly vigilant to PLAM, although misdiagnosis or missed diagnosis still exists. Provider attention to the correlation between PLAM and tuberous sclerosis complex, as well as to the possible involvement of multiple organs, is insufficient.