Lymphangioleiomyomatosis: a clinical review.
Lymphangioleiomyomatosis: a clinical review.
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DOI：
10.1183/20734735.0007-2020
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发表时间：
2020-06
期刊：
影响因子：
--
通讯作者：
McCarthy C
中科院分区：
文献类型：
--
作者：
O'Mahony AM;Lynn E;Murphy DJ;Fabre A;McCarthy C
淋巴管平滑肌瘤病是一种弥漫性囊性肺疾病。有两种主要类型的LAM：散发性和与结节性硬化症（TSC）相关的LAM，这是由TSC 1和TSC 2基因突变引起的。LAM的特征是囊性肺病，导致进行性呼吸困难、肾血管平滑肌脂肪瘤和淋巴并发症。气胸经常发生（70%），由于复发风险高，建议采用胸膜固定术进行明确治疗。计算机断层扫描可见特征性薄壁囊肿，血清血管内皮生长因子D水平升高具有良好的诊断特异性。目前，没有单一的临床或血清学因素已被证明可以预测预后。然而，在过去的十年中，我们对LAM病理生理学的理解取得了重大进展，从而提高了对这种罕见疾病的认识并确定了治疗方案。雷帕霉素抑制剂的机制靶点可以减缓肺功能下降的速度，并可以解决乳糜渗出和血管平滑肌脂肪瘤消退。LAM患者的预期寿命是有利的，从诊断时起平均无移植生存期>20年。 对LAM分子基础理解的持续进展将导致治疗靶点的改善和更稳健的预后指标的发展。阐明LAM的临床特征、常见表现和放射学特征概述LAM的诊断方法，包括VEGF-D的作用回顾LAM的当前预后指标，并概述肺功能、激素状态、VEGF-D和结局的临床表现为临床医生提供药物和非药物性淋巴管平滑肌瘤病（LAM）的管理选择信息是一种罕见的弥漫性囊性肺疾病，具有强大的诊断生物标志物和有效的治疗https://bit.ly/3dEbiEe
Lymphangioleiomyomatosis (LAM) is a diffuse cystic lung disease. There are two main types of LAM: sporadic, and LAM associated with the tuberous sclerosis complex (TSC), which is caused by mutations in the TSC1 and TSC2 genes. LAM is characterised by cystic lung disease resulting in progressive dyspnoea, renal angiomyolipomas and lymphatic complications. Pneumothorax occurs frequently (70%) and definitive management with pleurodesis is recommended as the risk of recurrence is high. Characteristic thin-walled cysts are seen on computed tomography and the presence of elevated serum levels of a vascular endothelial growth factor-D has good diagnostic specificity. Currently, no single clinical or serological factor has been shown to predict prognosis. However, over the past decade, significant advances in our understanding of the pathophysiology of LAM has led to improved recognition of this rare disease and identification of treatment options. Mechanistic target of rapamycin inhibitors slow the rate of lung function decline and can resolve chylous effusion and regress angiomyolipomas. Life expectancy in patients with LAM is favourable, with a mean transplant-free survival >20 years from the time of diagnosis. Continued advances in understanding the molecular basis of LAM will lead to improved therapeutic targets and the development of more robust prognostic indicators. To illustrate the clinical features, common presentations and radiological features of LAM To outline the diagnostic approach to LAM, including the role of VEGF-D To review the current prognostic indicators in LAM, and outline the impact of lung function, hormonal status, VEGF-D and clinical presentation on outcome To inform clinicians on the management options for LAM both pharmacological and nonpharmacological Lymphangioleiomyomatosis (LAM) is a rare diffuse cystic lung disease with robust diagnostic biomarkers and effective therapy https://bit.ly/3dEbiEe
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影响因子：
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