Department of Respiratory and Critical Care Medicine, the first College of Clinical Medicine science, China Three Gorges University, Yichang, People's Republic of China.
Department of Respiratory and Critical Care Medicine, Yichang Central People's Hospital., Wuhan, Hubei, China.
Medicine (Baltimore). 2022 Dec 30;101(52):e32492. doi: 10.1097/MD.0000000000032492.
Lymphangioleiomyomatosis (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.
We 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).
36 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.
Physicians 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.
淋巴管平滑肌瘤病（LAM）是一种累及多系统的罕见疾病，分为散发性 LAM（S-LAM）和结节性硬化症相关的 LAM，主要影响育龄期女性。男性患者的数据有限且分散。因此，有必要进行系统评价，以研究男性 LAM 的临床特征、诊断、治疗和结局。
我们通过检索过去 35 年（1986 年 4 月至 2021 年 10 月）发表的所有男性 LAM 病例报告，进行了文献回顾。
本文纳入了 26 篇参考文献中描述的 36 例男性患者。发病中位年龄为 34 岁（四分位距：1-79）。最常见的首发症状为咳嗽、呼吸困难、喘息和咯血，伴有气胸和乳糜胸等肺部并发症。5 例（13.9%）患者入院时无症状。36 例男性患者中有近一半存在薄壁充气性囊肿，可见于双肺。考虑到上述非典型临床特征，8 例（22.2%）患者误诊。此外，结节性硬化症相关的淋巴管平滑肌瘤病患者常无肺部表现，而有多种肺外表现，肾血管平滑肌脂肪瘤的发生率高于 S-LAM 患者（P < 0.01）。最终，4 例 S-LAM 患者死亡。
医生应提高对男性 LAM 的认识。应建议早期监测各个系统，以确保早期管理和积极随访。结节性硬化症患者应立即跟踪 LAM 疾病的发病情况，以改善预后。
