Lymphatics in lymphangioleiomyomatosis and idiopathic pulmonary fibrosis.
Lymphatics in lymphangioleiomyomatosis and idiopathic pulmonary fibrosis.
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DOI：
10.1183/09059180.00009311
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发表时间：
2012-09-01
期刊：
影响因子：
--
通讯作者：
Moss J
中科院分区：
文献类型：
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作者：
Glasgow CG;El-Chemaly S;Moss J
淋巴系统的主要功能是吸收和运输大分子和免疫细胞到一般循环，从而调节液体、营养吸收和免疫细胞的运输。淋巴管生成在组织炎症和肿瘤细胞扩散中起重要作用。淋巴管受累见于淋巴管肌瘤病(LAM)和特发性肺纤维化(IPF)。LAM是一种主要影响女性的疾病，涉及肺(囊性破坏)、肾脏(血管肌脂瘤)和轴淋巴管(腺病和淋巴管肌瘤)。LAM为散发性或与结节性硬化症(TSC)相关。囊性肺破坏是由于LAM细胞的增殖造成的，LAM细胞是一种带有TSC1或TSC2基因突变的异常平滑肌样细胞。淋巴管异常是由于LAM细胞渗入淋巴壁，导致淋巴管受损或阻塞。良性的LAM细胞具有转移特性，存在于血液和其他体液中。IPF是一种进行性肺部疾病，由成纤维细胞增殖和胶原沉积引起。淋巴管生成与肺破坏和疾病严重程度有关。从IPF支气管肺泡灌洗液(BALF)中分离的巨噬细胞亚群在体外表达淋巴管内皮细胞标志，与正常BALF中相同的巨噬细胞亚群形成对比。在这里，我们回顾淋巴参与LAM和IPF。
The primary function of the lymphatic system is absorbing and transporting macromolecules and immune cells to the general circulation, thereby regulating fluid, nutrient absorption and immune cell trafficking. Lymphangiogenesis plays an important role in tissue inflammation and tumour cell dissemination. Lymphatic involvement is seen in lymphangioleiomyomatosis (LAM) and idiopathic pulmonary fibrosis (IPF). LAM, a disease primarily affecting females, involves the lung (cystic destruction), kidney (angiomyolipoma) and axial lymphatics (adenopathy and lymphangioleiomyoma). LAM occurs sporadically or in association with tuberous sclerosis complex (TSC). Cystic lung destruction results from proliferation of LAM cells, which are abnormal smooth muscle-like cells with mutations in the TSC1 or TSC2 gene. Lymphatic abnormalities arise from infiltration of LAM cells into the lymphatic wall, leading to damage or obstruction of lymphatic vessels. Benign appearing LAM cells possess metastatic properties and are found in the blood and other body fluids. IPF is a progressive lung disease resulting from fibroblast proliferation and collagen deposition. Lymphangiogenesis is associated with pulmonary destruction and disease severity. A macrophage subset isolated from IPF bronchoalveolar lavage fluid (BALF) express lymphatic endothelial cell markers in vitro, in contrast to the same macrophage subset from normal BALF. Herein, we review lymphatic involvement in LAM and IPF.