IgG4相关性疾病!该如何辨别?

2022-11-23 邵逸夫医院 邵逸夫医院

 IgG4-RD的治疗分为诱导缓解和维持治疗两个阶段。目前IgG4-RD的治疗药物种类包括糖皮质激素、传统免疫抑制剂和生物制剂。其他辅助治疗包括梗阻部位置管引流、手术等。

50岁的李大叔(化名)一个月前去当地医院体检,体检时B超发现“胆管增厚、胰腺有明显的肿块”。

拿到这个结果,李大叔和家人都有点担心,再次复查腹部CT发现“胰腺占位,肿瘤不能排除”,当地医院的医生看过后也建议尽快去大医院就诊治疗,一家人的情绪跌落谷底。

“这事耽误不得!”李大叔的子女马上带着他来到浙江大学医学院附属邵逸夫医院就诊,医生询问患者近期有无消瘦、黄疸、腹痛、大便是否成型、是否规律等症状,李大叔说自己近一年来身体倍儿棒,完全没有医生所提到的问题。

医生根据情况给他安排了抽血及穿刺检查,最终发现,李大叔的胰腺“肿块”考虑为“IgG4相关性疾病”,是一种免疫系统疾病,不是肿瘤!随后李大叔被转入风湿免疫科治疗。

IgG4相关性疾病是什么?

想必当你看到这个名称时

也跟当时的李大叔一样

满脑子都是问号吧?

IgG4相关性疾病

是什么病呢?

IgG4相关性疾病(immunoglobulin-G4 related disease ,IgG4-RD)是近年来新被定义的一种由免疫介导的慢性炎症伴纤维化的疾病。

这种疾病可累及全身多个部位,绝大多数患者出现血清IgG4水平升高。

IgG4-RD确切的病因和发病机制目前仍不清楚。研究表明,可能相关的因素包括遗传、环境特别是微生物感染和分子模拟、自身抗体、固有免疫和适应性免疫等。

IgG4-RD好发于中老年人,男女比例约为8:3。我国尚无流行病学数据,日本报道的IgG4-RD患病率为(0.28~1.08)/10万。

尽管这种病是一种良性炎症性疾病,少数患者有自愈倾向,但大多数患者病程呈逐渐进展趋势,可导致重要脏器功能障碍,因此不能轻视。IgG4-RD

有什么症状?

IgG4-RD多为慢性隐匿性或亚急性起病,显著升高的血清IgG4水平和肿块样病灶是最常见的临床表现。

本病全身症状不突出,发热罕见,部分患者会出现乏力、体重下降等。合并过敏性疾病较常见,如过敏性鼻炎、哮喘、湿疹、荨麻疹等。

IgG4-RD可累及全身多个器官和组织,起病症状和临床表现也多种多样。主要受累器官的临床特征如下:

01唾液腺

大唾液腺炎在IgG4-RD最常见,典型表现为双侧或单侧颌下腺、腮腺或舌下腺无痛性肿大,触诊质地较硬,可伴有口干症状。初期症状不明显,容易被忽视,器官肿大明显时易被误诊为肿瘤。

02眶部病变

泪腺受累较常见,表现为单侧或双侧无痛性泪腺肿大,患者可有异物感、眼部不适;眼肌病变可表现为眼肌增粗,严重时可出现眼球突出、视物模糊、重影等;眶内炎性假瘤样病变可压迫视神经,可出现视力下降,亦可表现为突眼。

03胰腺

最常受累的内脏器官之一。多以无痛性梗阻性黄疸起病,部分患者出现上腹痛、脂肪泻及体重减轻,少数可表现为新发糖尿病。典型的影像学表现为胰腺弥漫性肿大;也可出现局灶性病变,类似瘤样肿块,容易与胰腺恶性肿瘤混淆。

04胆道

主要临床表现为胆管酶升高为主的肝功能异常、梗阻性黄疸的表现较突出。

05腹膜后组织

临床主要表现为腰腹部疼痛或不适、下肢水肿。

06胸腔器官

患者可无症状或出现咳嗽、哮喘、气短、胸闷或胸痛等。07泌尿系统

肾实质受累主要表现为小管间质性肾炎,少数患者出现以蛋白尿为主的肾小球病变,严重者可引起急性或慢性肾功能不全。影像学表现为肾实质肿块或皮质多发结节、肾脏弥漫性增大、肾盂占位、肾盂或输尿管管壁增厚等。前列腺受累主要表现为前列腺增大,导致排尿困难、尿频等症状。

08内分泌系统

常见受累器官为甲状腺,称为IgG4相关硬化性甲状腺炎,表现为甲状腺弥漫性肿大、变硬,或局部肿块。有疼痛、局部肿胀、吞咽困难、声嘶、气管受压所致呼吸困难等。绝大多数患者甲状腺功能减退,血清中可检测出抗甲状腺抗体,组织病理活检可明确诊断。

09淋巴结

淋巴结肿大在IgG4-RD很常见,可见于半数以上的患者,表现为浅表或深部淋巴结肿大;浅表淋巴结肿大为无痛性,边界清晰。

10心血管系统

少见,但心血管系统受累会影响患者预后。

基于以上特征,是不是觉得这个小小的免疫疾病有些不可思议啊,不仅能把肿瘤模仿得惟妙惟肖,还能把全身多个部位的肿瘤都给模仿了!一不小心就把病人置于恐慌,把医生置于两难。

IgG4相关性疾病

如何诊断?

IgG4-RD临床表现复杂多样,有时与肿瘤、感染和其他免疫性疾病难以鉴别,诊断需要结合临床病史、血清学、影像学和组织病理学特征。

临床上有多种疾病与IgG4-RD的组织病理学表现相类似,需进行鉴别诊断,如慢性炎症、肿瘤、感染、其他自身免疫病(系统性血管炎)、多中心卡斯尔曼病、罗萨伊-多尔夫曼病(Rosai-Dorfman disease)、炎性肌纤维母细胞瘤等,上述疾病可模拟IgG4-RD,造成误诊。

IgG4相关性疾病

该如何治疗?

总体治疗目标是控制病灶炎症,恢复器官功能,并维持疾病缓解。早期治疗可防止炎性和纤维化导致不可逆的脏器损伤。

治疗原则如下:

● 有症状、病情活动进展的患者均需治疗;无症状性内脏器官受累的患者,如评估病情处于发展阶段,也需要及时治疗。

● 重要脏器受累且病变为活动期者,如胰腺、胆道、肾脏、肺部、腹膜后纤维化、主动脉炎、中枢神经系统等,及时治疗可阻止器官损伤,改善预后。

● 少数无症状性淋巴结病或轻度浅表腺体肿大,且疾病进展很缓慢的患者,如IgG4相关性泪腺炎、颌下腺炎、淋巴结肿大,可密切观察随诊。一旦出现症状或病情活动进展加速,应给予积极治疗。

● IgG4-RD的治疗分为诱导缓解和维持治疗两个阶段。目前IgG4-RD的治疗药物种类包括糖皮质激素、传统免疫抑制剂和生物制剂。其他辅助治疗包括梗阻部位置管引流、手术等。

IgG4-RD

预后怎么样?

该病认识时间较短,长期生存率尚无数据。

整体来说激素治疗反应比较好,如果重要脏器没有不可逆损伤的话长期预后好,有重要脏器损伤且发生功能障碍者预后与器官损伤的程度相关,如胰腺、肾、肺、肥厚性硬脑膜炎或垂体炎等。

这种病目前尚不能治愈,且容易复发,因此需要在风湿免疫科医师指导下规律随诊、监测病情,对改善预后尤为重要。

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    2023-04-25 ms2000000943472648 来自广东省

    学习转发

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    2022-11-23 mswert122 来自上海

    学习了

    0

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    2022-11-24 delianjihui

    容易漏误诊

    0

  4. [GetPortalCommentsPageByObjectIdResponse(id=2128265, encodeId=7e242128265b3, content=学习转发, beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=b7578650055, createdName=ms2000000943472648, createdTime=Tue Apr 25 17:41:48 CST 2023, time=2023-04-25, status=1, ipAttribution=广东省), GetPortalCommentsPageByObjectIdResponse(id=2102246, encodeId=ef52210224693, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=44, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2017/02/28/889a1910ded7780f3c049b354f1648b7.jpg, createdBy=2a791737770, createdName=mswert122, createdTime=Wed Nov 23 21:13:22 CST 2022, time=2022-11-23, status=1, ipAttribution=上海), GetPortalCommentsPageByObjectIdResponse(id=2102502, encodeId=a779210250217, content=容易漏误诊, beContent=null, objectType=article, channel=null, level=null, likeNumber=52, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:04:07 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102501, encodeId=8120210250129, content=转发学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:03:11 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102500, encodeId=f879210250006, content=感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:03:06 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102499, encodeId=eee12102499e8, content=学习受益, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:03:01 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102326, encodeId=79c72102326b4, content=学习坚持打卡, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=65724176436, createdName=13d0179fm99暂无昵称, createdTime=Wed Nov 23 23:39:42 CST 2022, time=2022-11-23, status=1, ipAttribution=山西省), GetPortalCommentsPageByObjectIdResponse(id=2102310, encodeId=cca3210231054, content=学习了,谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=7c592194322, createdName=1209e435m98(暂无昵称), createdTime=Wed Nov 23 22:17:19 CST 2022, time=2022-11-23, status=1, ipAttribution=浙江省), GetPortalCommentsPageByObjectIdResponse(id=2102244, encodeId=9822210224408, content=认真学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6EF3hKja8nER8RMzxQdria9eCYFhgLTLsDOeqnvIYdeLDz5eP6EnicLuE5gibiawgno4zUA0jAR7Nbtp2llQ9fs3Mk/0, createdBy=278f1986883, createdName=医鸣惊人, createdTime=Wed Nov 23 21:07:42 CST 2022, time=2022-11-23, status=1, ipAttribution=山西省)]
    2022-11-24 delianjihui

    转发学习

    0

  5. [GetPortalCommentsPageByObjectIdResponse(id=2128265, encodeId=7e242128265b3, content=学习转发, beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=b7578650055, createdName=ms2000000943472648, createdTime=Tue Apr 25 17:41:48 CST 2023, time=2023-04-25, status=1, ipAttribution=广东省), GetPortalCommentsPageByObjectIdResponse(id=2102246, encodeId=ef52210224693, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=44, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2017/02/28/889a1910ded7780f3c049b354f1648b7.jpg, createdBy=2a791737770, createdName=mswert122, createdTime=Wed Nov 23 21:13:22 CST 2022, time=2022-11-23, status=1, ipAttribution=上海), GetPortalCommentsPageByObjectIdResponse(id=2102502, encodeId=a779210250217, content=容易漏误诊, beContent=null, objectType=article, channel=null, level=null, likeNumber=52, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:04:07 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102501, encodeId=8120210250129, content=转发学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:03:11 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102500, encodeId=f879210250006, content=感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:03:06 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102499, encodeId=eee12102499e8, content=学习受益, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:03:01 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102326, encodeId=79c72102326b4, content=学习坚持打卡, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=65724176436, createdName=13d0179fm99暂无昵称, createdTime=Wed Nov 23 23:39:42 CST 2022, time=2022-11-23, status=1, ipAttribution=山西省), GetPortalCommentsPageByObjectIdResponse(id=2102310, encodeId=cca3210231054, content=学习了,谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=7c592194322, createdName=1209e435m98(暂无昵称), createdTime=Wed Nov 23 22:17:19 CST 2022, time=2022-11-23, status=1, ipAttribution=浙江省), GetPortalCommentsPageByObjectIdResponse(id=2102244, encodeId=9822210224408, content=认真学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6EF3hKja8nER8RMzxQdria9eCYFhgLTLsDOeqnvIYdeLDz5eP6EnicLuE5gibiawgno4zUA0jAR7Nbtp2llQ9fs3Mk/0, createdBy=278f1986883, createdName=医鸣惊人, createdTime=Wed Nov 23 21:07:42 CST 2022, time=2022-11-23, status=1, ipAttribution=山西省)]
    2022-11-24 delianjihui

    感谢分享

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=2128265, encodeId=7e242128265b3, content=学习转发, beContent=null, objectType=article, channel=null, level=null, likeNumber=25, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=b7578650055, createdName=ms2000000943472648, createdTime=Tue Apr 25 17:41:48 CST 2023, time=2023-04-25, status=1, ipAttribution=广东省), GetPortalCommentsPageByObjectIdResponse(id=2102246, encodeId=ef52210224693, content=学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=44, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://cdnapi.center.medsci.cn/medsci/head/2017/02/28/889a1910ded7780f3c049b354f1648b7.jpg, createdBy=2a791737770, createdName=mswert122, createdTime=Wed Nov 23 21:13:22 CST 2022, time=2022-11-23, status=1, ipAttribution=上海), GetPortalCommentsPageByObjectIdResponse(id=2102502, encodeId=a779210250217, content=容易漏误诊, beContent=null, objectType=article, channel=null, level=null, likeNumber=52, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:04:07 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102501, encodeId=8120210250129, content=转发学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:03:11 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102500, encodeId=f879210250006, content=感谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:03:06 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102499, encodeId=eee12102499e8, content=学习受益, beContent=null, objectType=article, channel=null, level=null, likeNumber=48, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a5281475204, createdName=delianjihui, createdTime=Thu Nov 24 22:03:01 CST 2022, time=2022-11-24, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2102326, encodeId=79c72102326b4, content=学习坚持打卡, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=65724176436, createdName=13d0179fm99暂无昵称, createdTime=Wed Nov 23 23:39:42 CST 2022, time=2022-11-23, status=1, ipAttribution=山西省), GetPortalCommentsPageByObjectIdResponse(id=2102310, encodeId=cca3210231054, content=学习了,谢谢分享, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=7c592194322, createdName=1209e435m98(暂无昵称), createdTime=Wed Nov 23 22:17:19 CST 2022, time=2022-11-23, status=1, ipAttribution=浙江省), GetPortalCommentsPageByObjectIdResponse(id=2102244, encodeId=9822210224408, content=认真学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=0, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6EF3hKja8nER8RMzxQdria9eCYFhgLTLsDOeqnvIYdeLDz5eP6EnicLuE5gibiawgno4zUA0jAR7Nbtp2llQ9fs3Mk/0, createdBy=278f1986883, createdName=医鸣惊人, createdTime=Wed Nov 23 21:07:42 CST 2022, time=2022-11-23, status=1, ipAttribution=山西省)]
    2022-11-24 delianjihui

    学习受益

    0

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    2022-11-23 13d0179fm99暂无昵称 来自山西省

    学习坚持打卡

    0

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    2022-11-23 1209e435m98(暂无昵称) 来自浙江省

    学习了,谢谢分享

    0

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    2022-11-23 医鸣惊人 来自山西省

    认真学习了

    0

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