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Semin Arthritis Rheu:成人Still病中的肺动脉高压

2018-12-18 xiangting MedSci原创

PAH是一种未被认识的AOSD并发症,在对运动时有呼吸困难或运动耐力下降的患者进行鉴别诊断时,应牢记这一点。

这项研究旨在调查成人Still病(AOSD)中肺动脉高压(PAH)的患病率、临床特征和预后。

研究人员回顾性审查了2个三级转诊医院33年间诊断的所有AOSD患者,选择那些通过右心导管检查确诊为PAH的患者进行分析。还进行了系统的文献综述(PubMed 1990至2018年7月),以确定该并发症的预后和最佳治疗策略。

该AOSD人群中PAH的总患病率为4.8%(2/41)。已报道了20例记录良好的患者,其中包括该人群中的2例。PAH可能在病程的任何时间段使AOSD复杂化,并通常发生在患持续性严重疾病的患者中,既往或伴随严重临床并发症的频率很高(35%)。

根据2015 ESC/ERS指南,所有的病例肺动脉高压的病因是1组PAH。该系列中大多数患者在PAH诊断时的WHO功能分级为III-IV级,反映了明显的诊断延迟。

尽管接受了治疗,33%的患者预后不良,死亡率达到了22%。获得最佳结果的治疗策略是使用糖皮质激素、免疫抑制剂和PAH特异性血管扩张治疗。

PAH是一种未被认识的AOSD并发症,在对运动时有呼吸困难或运动耐力下降的患者进行鉴别诊断时,应牢记这一点。

原始出处:

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    2018-12-20 zhaojie88
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    2018-12-18 明天jing

    肺动脉高压表面是罕见病,事实上临床上并不少见,治疗药物虽然有一些,但是整体仍然不理解,可能未来需要采用综合治疗措施。

    0

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淋巴瘤VS成人still病——长期高热,如何鉴别?

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Ann Rheum Dis :托珠单抗对糖皮质激素难治性成人still病的疗效

该研究表明,尽管未达到主要终点,并且没有得出可靠的结论,托珠单抗治疗成人Still病是有效的。

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