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结缔组织疾病相关肺动脉高压

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摘要 结缔组织疾病是指一大类因淋巴细胞对自身组织出现免疫反应并导致组织损伤的非器官特异性自身免疫病,该类疾病以血管和结缔组织慢性炎症的病理改变为基础,可累及多个系统和器官.肺部是结缔组织疾病最常见的受累器官之一,肺内所有结构包括肺实质、气道、肺血管、胸膜、肌肉和骨骼等均可受到累及而出现形态各异的病变,如间质性肺疾病、肺内结节、气道阻塞、胸膜炎或胸腔积液、肺血管炎和肺动脉高压等.肺动脉高压是结缔组织疾病的严重并发症之一,患者一旦出现肺动脉高压,则提示预后不良.可合并肺动脉高压的结缔组织疾病包括系统性硬化病、系统性红斑狼疮、混合性结缔组织病、原发性干燥综合征、类风湿关节炎等等,尽管在临床研究中常常将这些患者归为一组,但事实上不同类型结缔组织疾病患者合并肺动脉高压的情况有各自不同的特点.
作者 林小玲 江山平
机构地区 中山大学孙逸仙纪念医院呼吸内科
出处 《临床内科杂志》 CAS 2014年第7期448-451,共4页 Journal of Clinical Internal Medicine
关键词 结缔组织疾病 肺动脉高压
分类号 R593.2 [医药卫生—内科学]
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参考文献26

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二级参考文献12

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临床内科杂志
2014年 第7期

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