• Haoyu Zhang, Zhigang Hu, Sufei Wang, Kanhao Wu, Qiaoyu Yang, and Xinyu Song.
• Department of Respiratory and Critical Care Medicine, the first College of Clinical Medicine science, China Three Gorges University, Yichang, People's Republic of China.
• Medicine (Baltimore). 2022 Dec 30; 101 (52): e32492e32492.

BackgroundLymphangioleiomyomatosis (LAM) is a rare disease involving multiple systems, which is divided into sporadic LAM (S-LAM) and tuberous sclerosis complex-LAM, mostly affecting women who are in childbearing age stage. Data on male patients are limited and scattered. Therefore, it is necessary to conduct a systematic review to investigate the clinical features, diagnosis, treatment, and outcomes of LAM in male.MethodsWe performed a literature review by searching for all the published reported cases of LAM in male during the past 35 years (April 1986-October 2021).Results36 male patients described in 26 references were included in this article. The median age of onset was 34 years (interquartile range: 1-79). The most common initial manifestations were cough, dyspnea, respite, and hemoptysis, with pulmonary complications such as pneumothorax and chylothorax. Five patients (13.9%) were asymptomatic at admission. Nearly half of the 36 male patients had thin-walled air-filled cysts that were visible throughout both lungs. Considering the abovementioned atypical clinical features, misdiagnosis was committed in 8 patients (22.2%). In addition, patients with tuberous sclerosis complex lymphangioleiomyomatosis often have no pulmonary manifestations at onset but present multiple extrapulmonary manifestations and have higher rates of renal angiomyolipomas than patients with S-LAM (P < 0.01). Eventually, 4 patients with S-LAM eventually died.ConclusionPhysicians should increase the awareness of LAM in male. Early monitoring of various systems should be recommended to ensure early management and active follow-up. Tuberous sclerosis complex patients should immediately be tracked for the onset of LAM disease to improve prognosis.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

18 keywords...

hide…