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SAPHO综合征特征，影像学表现

2016-05-08 MedSci MedSci原创

SAPHO综合征
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   SAPHO综合征作为一种主要累及皮肤、骨和关节的慢性疾病，直到最近二十年才逐渐被人们所认识，其发病率较低，国内至今报道尚不足百例。SAPHO为下列5个英文单词的缩写，即滑膜炎(synovitis)、痤疮(acne)、脓疱病(pustulosis)、骨肥厚(hyperostosis)和骨髓炎(osteomyelitis)。 SAPHO综合征患者掌跖脓疱病表现 198

SAPHO综合征作为一种主要累及皮肤、骨和关节的慢性疾病，直到最近二十年才逐渐被人们所认识，其发病率较低，国内至今报道尚不足百例。SAPHO为下列5个英文单词的缩写，即滑膜炎(synovitis)、痤疮(acne)、脓疱病(pustulosis)、骨肥厚(hyperostosis)和骨髓炎(osteomyelitis)。

SAPHO综合征患者掌跖脓疱病表现

1987年法国医师雅尔玛(Charmot)在分析了85例患者的临床资料后，首次提出以SAPHO综合征来命名一组特殊的症候群，包括掌跖脓疱病、化脓性汗腺炎或重症痤疮伴发胸肋锁骨肥厚、慢性复发性多灶性关节炎、骶髂关节炎等损害。

SAPHO综合征好发于中青年，其临床表现主要包括皮肤和骨骼关节病变。典型的皮肤病变为脓疱病和痤疮。骨骼关节特征性病变是骨炎和骨肥厚，临床上主要表现为受累处的疼痛和活动受限。以胸骨、锁骨、胸锁关节、肋骨受累最为常见，脊柱、骶髂关节、长骨和扁骨也可受累。因为该病的皮肤改变和骨骼关节改变并不平行，二者可同时发生，也可先后发生，间隔数月至数年不等，甚至有些患者始终都无皮肤受累的表现，从而为诊断本病造成困难，导致误诊和漏诊。

影像学检查是发现该病骨骼损害的主要方法，主要表现为骨质增生和骨炎，主要特点是由慢性骨膜反应和皮质增生引起的骨肥厚。实验室检查多无特异性，可出现急性时相反应物如血沉、C反应蛋白的升高，约有30%的患者人白细胞抗原(HLA)-B27阳性。

胸部CT可见SAPHO综合征患者锁骨肥厚表现

SAPHO综合征的一线治疗方案以非类固醇类抗炎药(NSAID)对症治疗为主，部分炎症反应重且一线药物疗效不佳者，可短期使用中小剂量糖皮质激素。如外周关节滑膜炎明显或皮损明显者，可联用慢作用抗风湿药物如甲氨蝶呤及柳氮磺吡啶等。有文献证实肿瘤坏死因子抑制剂对难治性SAPHO患者有效。最近的研究表明，双磷酸盐类药物如帕米磷酸二钠等对本病有确切疗效。

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    2016-10-28 zz70

    #SAP#

    0

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    2016-06-03 忠诚向上

    好好学习

    0

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    2016-05-10 卡莲

    值得学习

    0

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    2016-05-10 1dd8a7c5m95(暂无匿称)

    值得阅读，学习！

    0

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    2016-05-10 zhouqu_8

    #综合征#

    0

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    2016-05-09 jaysppr

    皮肤病种类和分类最头痛了

    0

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    2016-05-09 憨豆医生

    sapho综合征

    0

  8. [GetPortalCommentsPageByObjectIdResponse(id=1898174, encodeId=4a5618981e434, content=<a href='/topic/show?id=8904158e9e7' target=_blank style='color:#2F92EE;'>#SAP#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=95, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=15879, encryptionId=8904158e9e7, topicName=SAP)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=30f5146, createdName=zz70, createdTime=Fri Oct 28 13:39:00 CST 2016, time=2016-10-28, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=88322, encodeId=94df8832284, content=好好学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=159, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=45101626056, createdName=忠诚向上, createdTime=Fri Jun 03 07:50:00 CST 2016, time=2016-06-03, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=85389, encodeId=9daa8538968, content=值得学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=172, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=e4e21675717, createdName=卡莲, createdTime=Tue May 10 21:12:00 CST 2016, time=2016-05-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=85342, encodeId=4ac18534248, content=值得阅读，学习！, beContent=null, objectType=article, channel=null, level=null, likeNumber=176, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/mStl88fu4NfNLvzZhgPxRuUXgacXpPeW0x8Z3Qr9c3I2kTic4ZIpR29iaftYgkjRcbv982icYMtqANBPYBgPnOia6mVtX76qMXuc/0, createdBy=ebe61630855, createdName=1dd8a7c5m95(暂无匿称), createdTime=Tue May 10 12:53:00 CST 2016, time=2016-05-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1430615, encodeId=f1c814306150f, content=<a href='/topic/show?id=f9b9e9124a8' target=_blank style='color:#2F92EE;'>#综合征#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=100, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=79124, encryptionId=f9b9e9124a8, topicName=综合征)], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/aLGWoFXAyMbIu3qymFOyheQLjPSX3OUs5GmkyBlcCOwTPIeq3why9NGibxxUqYo6hcx8qZLHZFgNPnBK1yzWeOFpyg2OnWOt0/0, createdBy=fa4716, createdName=zhouqu_8, createdTime=Tue May 10 09:39:00 CST 2016, time=2016-05-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=84928, encodeId=9d7d84928a5, content=皮肤病种类和分类最头痛了, beContent=null, objectType=article, channel=null, level=null, likeNumber=147, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/PiajxSqBRaEIsrUxHnicsiaqwzJibCks60HOicbEAgbNzUiciboMJDzsWg16OHTeNHhyYvTg6TDXTlOx7icnhttCYYe2ujYt0yND7dYusOsibl9XnQCE/0, createdBy=7ad21722200, createdName=jaysppr, createdTime=Mon May 09 10:22:00 CST 2016, time=2016-05-09, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=84862, encodeId=ed088486244, content=sapho综合征, beContent=null, objectType=article, channel=null, level=null, likeNumber=173, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160509/IMG572F717D95C5F3379.jpg, createdBy=f29c1665748, createdName=憨豆医生, createdTime=Mon May 09 01:05:00 CST 2016, time=2016-05-09, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=84828, encodeId=87068482845, content=收藏, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=http://cacheapi.medsci.cn/resource/upload/20160307/IMG56DD346053F0D5538.jpg, createdBy=7bed1697174, createdName=那夏花开, createdTime=Sun May 08 23:21:00 CST 2016, time=2016-05-08, status=1, ipAttribution=)]
    2016-05-08 那夏花开

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