White Barbara
Baltimore VA Medical Center, Research Service (151), 10 North Greene Street, Room 3A-125, Baltimore, MD 21201, USA.
Curr Rheumatol Rep. 2002 Apr;4(2):108-12. doi: 10.1007/s11926-002-0005-1.
Pulmonary fibrosis causes significant morbidity and mortality in patients with scleroderma. Lung inflammation identifies patients at greater risk for decline in forced vital capacity and diffusing capacity for carbon monoxide. Factors that are increased in patients with scleroderma with lung fibrosis include connective tissue growth factor, KL-6, pulmonary surfactant-D, tissue inhibitor of metalloproteinase 2, monocyte chemotactic protein-1, macrophage inhibitory protein-1 alpha, soluble interleukin-6 receptors, anti-endothelial cell antibodies, and anti-DNA topoisomerase I antibodies. Potential mechanisms of lung damage in scleroderma include increased production of profibrotic type 2 cytokines and abnormal signaling by thrombin of tenascin-C production by lung fibroblasts, with protein kinase C epsilon as an intermediate in the signaling pathway. Treatment of scleroderma lung disease with cyclophosphamide may have a beneficial effect on pulmonary function and survival. Lung transplantation provides a therapeutic option for patients with scleroderma with end-stage lung disease.
肺纤维化在硬皮病患者中会导致显著的发病率和死亡率。肺部炎症可识别出用力肺活量和一氧化碳弥散量下降风险更高的患者。硬皮病合并肺纤维化患者体内升高的因素包括结缔组织生长因子、KL-6、肺表面活性物质-D、金属蛋白酶组织抑制剂2、单核细胞趋化蛋白-1、巨噬细胞抑制蛋白-1α、可溶性白细胞介素-6受体、抗内皮细胞抗体和抗DNA拓扑异构酶I抗体。硬皮病肺损伤的潜在机制包括促纤维化2型细胞因子的产生增加以及凝血酶对肺成纤维细胞产生腱生蛋白-C的异常信号传导，蛋白激酶Cε作为信号通路中的中间体。用环磷酰胺治疗硬皮病肺部疾病可能对肺功能和生存率有有益影响。肺移植为患有终末期肺部疾病的硬皮病患者提供了一种治疗选择。
