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:

本文系梅斯医学(MedSci)原创编译整理,转载需授权!

版权声明:
本网站所有内容来源注明为“梅斯医学”或“MedSci原创”的文字、图片和音视频资料,版权均属于梅斯医学所有。非经授权,任何媒体、网站或个人不得转载,授权转载时须注明来源为“梅斯医学”。其它来源的文章系转载文章,或“梅斯号”自媒体发布的文章,仅系出于传递更多信息之目的,本站仅负责审核内容合规,其内容不代表本站立场,本站不负责内容的准确性和版权。如果存在侵权、或不希望被转载的媒体或个人可与我们联系,我们将立即进行删除处理。
在此留言
评论区 (5)
#插入话题
  1. [GetPortalCommentsPageByObjectIdResponse(id=1925265, encodeId=e6e21925265bf, content=<a href='/topic/show?id=26d633354c9' target=_blank style='color:#2F92EE;'>#动脉高压#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=33354, encryptionId=26d633354c9, topicName=动脉高压)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=44a360, createdName=jklm09, createdTime=Fri Apr 26 16:15:00 CST 2019, time=2019-04-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1992569, encodeId=3024199256911, content=<a href='/topic/show?id=46885382321' target=_blank style='color:#2F92EE;'>#成人still病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=53823, encryptionId=46885382321, topicName=成人still病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=951d2500061, createdName=12498717m66(暂无昵称), createdTime=Fri Jun 21 06:15:00 CST 2019, time=2019-06-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1972575, encodeId=b4da19e257534, content=<a href='/topic/show?id=8a6a2e8839' target=_blank style='color:#2F92EE;'>#Arthritis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2788, encryptionId=8a6a2e8839, topicName=Arthritis)], attachment=null, authenticateStatus=null, createdAvatar=https://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTIibticIBly5jCSic5ZElW4He3rO4DMpUHUFaFX57OicNMnzckWaicBNeStwugWHFCuOdicoakApM4HGmtw/132, createdBy=f8672500006, createdName=ms6390871039024434, createdTime=Mon May 13 00:15:00 CST 2019, time=2019-05-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1357028, encodeId=c692135e028d9, content=<a href='/topic/show?id=b0702e84b9' target=_blank style='color:#2F92EE;'>#ART#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2784, encryptionId=b0702e84b9, topicName=ART)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=4c37265, createdName=zhaojie88, createdTime=Thu Dec 20 07:15:00 CST 2018, time=2018-12-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1050828, encodeId=17e81050828c3, content=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。, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f0620, createdName=明天jing, createdTime=Tue Dec 18 19:15:00 CST 2018, time=2018-12-18, status=1, ipAttribution=)]
  2. [GetPortalCommentsPageByObjectIdResponse(id=1925265, encodeId=e6e21925265bf, content=<a href='/topic/show?id=26d633354c9' target=_blank style='color:#2F92EE;'>#动脉高压#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=33354, encryptionId=26d633354c9, topicName=动脉高压)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=44a360, createdName=jklm09, createdTime=Fri Apr 26 16:15:00 CST 2019, time=2019-04-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1992569, encodeId=3024199256911, content=<a href='/topic/show?id=46885382321' target=_blank style='color:#2F92EE;'>#成人still病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=53823, encryptionId=46885382321, topicName=成人still病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=951d2500061, createdName=12498717m66(暂无昵称), createdTime=Fri Jun 21 06:15:00 CST 2019, time=2019-06-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1972575, encodeId=b4da19e257534, content=<a href='/topic/show?id=8a6a2e8839' target=_blank style='color:#2F92EE;'>#Arthritis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2788, encryptionId=8a6a2e8839, topicName=Arthritis)], attachment=null, authenticateStatus=null, createdAvatar=https://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTIibticIBly5jCSic5ZElW4He3rO4DMpUHUFaFX57OicNMnzckWaicBNeStwugWHFCuOdicoakApM4HGmtw/132, createdBy=f8672500006, createdName=ms6390871039024434, createdTime=Mon May 13 00:15:00 CST 2019, time=2019-05-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1357028, encodeId=c692135e028d9, content=<a href='/topic/show?id=b0702e84b9' target=_blank style='color:#2F92EE;'>#ART#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2784, encryptionId=b0702e84b9, topicName=ART)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=4c37265, createdName=zhaojie88, createdTime=Thu Dec 20 07:15:00 CST 2018, time=2018-12-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1050828, encodeId=17e81050828c3, content=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。, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f0620, createdName=明天jing, createdTime=Tue Dec 18 19:15:00 CST 2018, time=2018-12-18, status=1, ipAttribution=)]
  3. [GetPortalCommentsPageByObjectIdResponse(id=1925265, encodeId=e6e21925265bf, content=<a href='/topic/show?id=26d633354c9' target=_blank style='color:#2F92EE;'>#动脉高压#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=33354, encryptionId=26d633354c9, topicName=动脉高压)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=44a360, createdName=jklm09, createdTime=Fri Apr 26 16:15:00 CST 2019, time=2019-04-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1992569, encodeId=3024199256911, content=<a href='/topic/show?id=46885382321' target=_blank style='color:#2F92EE;'>#成人still病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=53823, encryptionId=46885382321, topicName=成人still病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=951d2500061, createdName=12498717m66(暂无昵称), createdTime=Fri Jun 21 06:15:00 CST 2019, time=2019-06-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1972575, encodeId=b4da19e257534, content=<a href='/topic/show?id=8a6a2e8839' target=_blank style='color:#2F92EE;'>#Arthritis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2788, encryptionId=8a6a2e8839, topicName=Arthritis)], attachment=null, authenticateStatus=null, createdAvatar=https://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTIibticIBly5jCSic5ZElW4He3rO4DMpUHUFaFX57OicNMnzckWaicBNeStwugWHFCuOdicoakApM4HGmtw/132, createdBy=f8672500006, createdName=ms6390871039024434, createdTime=Mon May 13 00:15:00 CST 2019, time=2019-05-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1357028, encodeId=c692135e028d9, content=<a href='/topic/show?id=b0702e84b9' target=_blank style='color:#2F92EE;'>#ART#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2784, encryptionId=b0702e84b9, topicName=ART)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=4c37265, createdName=zhaojie88, createdTime=Thu Dec 20 07:15:00 CST 2018, time=2018-12-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1050828, encodeId=17e81050828c3, content=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。, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f0620, createdName=明天jing, createdTime=Tue Dec 18 19:15:00 CST 2018, time=2018-12-18, status=1, ipAttribution=)]
  4. [GetPortalCommentsPageByObjectIdResponse(id=1925265, encodeId=e6e21925265bf, content=<a href='/topic/show?id=26d633354c9' target=_blank style='color:#2F92EE;'>#动脉高压#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=33354, encryptionId=26d633354c9, topicName=动脉高压)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=44a360, createdName=jklm09, createdTime=Fri Apr 26 16:15:00 CST 2019, time=2019-04-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1992569, encodeId=3024199256911, content=<a href='/topic/show?id=46885382321' target=_blank style='color:#2F92EE;'>#成人still病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=53823, encryptionId=46885382321, topicName=成人still病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=951d2500061, createdName=12498717m66(暂无昵称), createdTime=Fri Jun 21 06:15:00 CST 2019, time=2019-06-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1972575, encodeId=b4da19e257534, content=<a href='/topic/show?id=8a6a2e8839' target=_blank style='color:#2F92EE;'>#Arthritis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2788, encryptionId=8a6a2e8839, topicName=Arthritis)], attachment=null, authenticateStatus=null, createdAvatar=https://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTIibticIBly5jCSic5ZElW4He3rO4DMpUHUFaFX57OicNMnzckWaicBNeStwugWHFCuOdicoakApM4HGmtw/132, createdBy=f8672500006, createdName=ms6390871039024434, createdTime=Mon May 13 00:15:00 CST 2019, time=2019-05-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1357028, encodeId=c692135e028d9, content=<a href='/topic/show?id=b0702e84b9' target=_blank style='color:#2F92EE;'>#ART#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2784, encryptionId=b0702e84b9, topicName=ART)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=4c37265, createdName=zhaojie88, createdTime=Thu Dec 20 07:15:00 CST 2018, time=2018-12-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1050828, encodeId=17e81050828c3, content=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。, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f0620, createdName=明天jing, createdTime=Tue Dec 18 19:15:00 CST 2018, time=2018-12-18, status=1, ipAttribution=)]
    2018-12-20 zhaojie88
  5. [GetPortalCommentsPageByObjectIdResponse(id=1925265, encodeId=e6e21925265bf, content=<a href='/topic/show?id=26d633354c9' target=_blank style='color:#2F92EE;'>#动脉高压#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=38, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=33354, encryptionId=26d633354c9, topicName=动脉高压)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=44a360, createdName=jklm09, createdTime=Fri Apr 26 16:15:00 CST 2019, time=2019-04-26, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1992569, encodeId=3024199256911, content=<a href='/topic/show?id=46885382321' target=_blank style='color:#2F92EE;'>#成人still病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=53823, encryptionId=46885382321, topicName=成人still病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=951d2500061, createdName=12498717m66(暂无昵称), createdTime=Fri Jun 21 06:15:00 CST 2019, time=2019-06-21, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1972575, encodeId=b4da19e257534, content=<a href='/topic/show?id=8a6a2e8839' target=_blank style='color:#2F92EE;'>#Arthritis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2788, encryptionId=8a6a2e8839, topicName=Arthritis)], attachment=null, authenticateStatus=null, createdAvatar=https://thirdwx.qlogo.cn/mmopen/vi_32/Q0j4TwGTfTIibticIBly5jCSic5ZElW4He3rO4DMpUHUFaFX57OicNMnzckWaicBNeStwugWHFCuOdicoakApM4HGmtw/132, createdBy=f8672500006, createdName=ms6390871039024434, createdTime=Mon May 13 00:15:00 CST 2019, time=2019-05-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1357028, encodeId=c692135e028d9, content=<a href='/topic/show?id=b0702e84b9' target=_blank style='color:#2F92EE;'>#ART#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=2784, encryptionId=b0702e84b9, topicName=ART)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=4c37265, createdName=zhaojie88, createdTime=Thu Dec 20 07:15:00 CST 2018, time=2018-12-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1050828, encodeId=17e81050828c3, content=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。, beContent=null, objectType=article, channel=null, level=null, likeNumber=31, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f0620, createdName=明天jing, createdTime=Tue Dec 18 19:15:00 CST 2018, time=2018-12-18, status=1, ipAttribution=)]
    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

相关资讯

淋巴瘤VS成人still病——长期高热,如何鉴别?

成人Still病和淋巴瘤均可引起长期不明原因高热(体温高于38 ℃且持续2周以上),且它们的临床表现和化验检查有许多相似之处,如发病年龄均以青壮年为主,均可出现咽痛,肝、脾、淋巴结肿大,皮疹,胸腔积液,肝功异常,血白细胞升高,类风湿因子和抗核抗体阴性等,临床上很容易混淆。但因它们的治疗方法和预后不同,因此对这两种疾病进行鉴别非常重要。

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。