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至20世纪中后期，大量的临床研究与实验研究发现，在很多结缔组织病病人体内能检出针对自身抗原的自身抗体和与自身细胞反应的致敏淋巴细胞以及病变组织中有各种抗体，补体与免疫复合物沉积及免疫活性细胞浸润，因而主张将结缔组织病更名为“自身免疫性疾病”。 | [
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20世纪末众多临床学家认为“结缔组织病”与“自身免疫性疾病”的命名强调了病理学与免疫学研究结果，但都不能全部涵盖风湿性疾病的特征，故主张仍使用风湿性疾病的名称。 | [
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但现代风湿病概念已特指自身免疫耐受破坏后，发生自身免疫损伤的一类风湿性疾病。 | [
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它不包含外伤、代谢异常以及退行性变等许多经典风湿性疾病。 | [
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风湿性疾病的病因并不十分清楚，遗传因素、感染因素以及免疫异常是公认的值得深入研究的方向。 | [
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风湿性疾病往往因累及的器官不同而表现出由各种症状和体征组合的临床综合征。 | [
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“胶原性疾病”、“结缔组织病”以及“自身免疫性疾病”的命名均从不同角度反映了风湿性疾病的基本特征，这些命名有助于深入理解和研究风湿性疾病。 | [
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目前还缺乏针对各种风湿性疾病有特异性诊断价值的实验室检查项目，诊断主要依靠临床症状与实验室检查的综合分析，在除外其他疾病基础上，风湿性疾病的诊断才能成立。 | [
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一、免疫学检查（一）免疫活化现象免疫活化现象有关的实验室检查结果是引导临床诊断风湿性疾病的简易途径：1.血清免疫球蛋白（immunoglobulin，Ig）Ig水平升高是风湿性疾病最常见的免疫活化现象，系统性红斑狼疮（SLE）、幼年特发性关节炎（JIA）、皮肌炎以及各种血管炎综合征均常有一种或几种免疫球蛋白显著升高B细胞增殖及分泌功能过度活化淋巴细胞表型分析免疫活化现象还可以在淋巴细胞表型分析检测发现CD3、CD4、CD8、CD16、CD19、CD20阳性细胞绝对数与相对数增加得到证实。 | [
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3.免疫病理各脏器组织免疫病理学检测可见多种Ig、补体、抗原抗体复合物沉积病变区域或血管内外以及大量淋巴细胞的组织浸润补体检测测定补体活性及其中某些成分有助于判断几种风湿性疾病的活动性，尤其是对SLE有诊断意义。 | [
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C3、C4显著降低是补体消耗现象，也是免疫活化的标志。 | [
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一定要注意极少数原发性补体缺陷病以及与补体缺陷有关的风湿性疾病（表17-17）以上免疫活化现象仅是诊断各种风湿性疾病的提示指标。 | [
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表17-13补体缺陷与风湿性疾病（二）自身抗体与抗核抗体谱理论上讲，检出自身抗体是自身免疫性疾病的直接证据。 | [
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最早人们发现了抗细胞核自身抗体（ANA），并成为诊断SLE的有力工具，随后发现ANA并非SLE特异性诊断抗体，干燥综合征、皮肌炎、关节炎以及混合性结缔组织病均见ANA阳性。 | [
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无任何临床症状的“健康人”也见低滴度ANA阳性（也可能是各种风湿病亚临床病人）。 | [
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近年研究成果发现依据针对不同细胞核成分可将抗核抗体分成多种抗核抗体亚类谱系，抗核抗体谱能更好地帮助临床鉴别诊断风湿性疾病。 | [
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但并不是所有抗核抗体亚类都具备非常高的特异性和敏感性。 | [
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1.类风湿因子（rheumatoidfactor，RF）属一种抗Ig稳定区（Fc）的自身抗体。 | [
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虽然最先在类风湿关节炎病人血清中发现RF，但它在疾病中确切的病理及生理意义并未弄清。 | [
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健康人血清中的RF为天然自身抗体，往往亲和力低，效价低，属IgM抗体，可能来自胚胎性免疫球蛋白基因。 | [
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由RF构成的免疫复合体还可以在滑膜液、滑膜组织中及皮肤溃疡、病变血管和吞噬细胞中检出。 | [
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"end_offset": 23,
"label": "bod"
},
{
"id": 4,
"entity": "皮肤溃疡",
"start_offset": 25,
"end_offset": 29,
"label": "dis"
},
{
"id": 5,
"entity": "病变血管",
"start_offset": 30,
"end_offset": 34,
"label": "bod"
},
{
"id": 6,
"entity": "吞噬细胞",
"start_offset": 35,
"end_offset": 39,
"label": "bod"
}
] |
急、慢性感染，恶性肿瘤病人RF常呈阳性，甚至正常人也有低效价RF被检出，年龄越大阳性结果可能性越大。 | [
{
"id": 0,
"entity": "慢性感染",
"start_offset": 2,
"end_offset": 6,
"label": "dis"
},
{
"id": 1,
"entity": "恶性肿瘤",
"start_offset": 7,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "RF",
"start_offset": 13,
"end_offset": 15,
"label": "bod"
},
{
"id": 3,
"entity": "RF",
"start_offset": 30,
"end_offset": 32,
"label": "bod"
}
] |
虽然检测RF对JIA诊断并非关键，但RF阳性JIA患儿常伴有严重、顽固的关节炎及血管炎，值得重视。 | [
{
"id": 0,
"entity": "RF",
"start_offset": 4,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "JIA",
"start_offset": 7,
"end_offset": 10,
"label": "dis"
},
{
"id": 2,
"entity": "RF阳性JIA",
"start_offset": 18,
"end_offset": 25,
"label": "dis"
},
{
"id": 3,
"entity": "关节炎",
"start_offset": 36,
"end_offset": 39,
"label": "dis"
},
{
"id": 4,
"entity": "血管炎",
"start_offset": 40,
"end_offset": 43,
"label": "dis"
}
] |
推测RF阳性JIA病人的发病机制可能与成人类风湿关节炎发病机制相同。 | [
{
"id": 0,
"entity": "RF阳性JIA",
"start_offset": 2,
"end_offset": 9,
"label": "dis"
},
{
"id": 1,
"entity": "类风湿关节炎",
"start_offset": 21,
"end_offset": 27,
"label": "dis"
}
] |
2.隐匿性RF（hiddenrheumatoidfactor）沉降系数为19S，常规方法检测不出来，在血清中它与IgG结合在一起，用酸性凝胶滤过处理血清之后才能将其分离后再用ELISA方法测定。 | [
{
"id": 0,
"entity": "隐匿性RF",
"start_offset": 2,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "hiddenrheumatoidfactor",
"start_offset": 8,
"end_offset": 30,
"label": "bod"
},
{
"id": 2,
"entity": "沉降系数",
"start_offset": 31,
"end_offset": 35,
"label": "ite"
},
{
"id": 3,
"entity": "血清",
"start_offset": 51,
"end_offset": 53,
"label": "bod"
},
{
"id": 4,
"entity": "IgG",
"start_offset": 56,
"end_offset": 59,
"label": "bod"
},
{
"id": 5,
"entity": "酸性凝胶",
"start_offset": 66,
"end_offset": 70,
"label": "dru"
},
{
"id": 6,
"entity": "血清",
"start_offset": 74,
"end_offset": 76,
"label": "bod"
},
{
"id": 7,
"entity": "ELISA",
"start_offset": 87,
"end_offset": 92,
"label": "pro"
}
] |
有研究者发现65%～75%用经典方法检测RF阴性的JIA患儿隐匿性RF检测可能阳性。 | [
{
"id": 0,
"entity": "RF阴性的JIA",
"start_offset": 20,
"end_offset": 28,
"label": "dis"
},
{
"id": 1,
"entity": "隐匿性RF检测",
"start_offset": 30,
"end_offset": 37,
"label": "pro"
}
] |
Moore等人认为这种隐匿性RF与JIA活动有关，因此有助于诊断和治疗观察。 | [
{
"id": 0,
"entity": "隐匿性RF",
"start_offset": 11,
"end_offset": 16,
"label": "pro"
},
{
"id": 1,
"entity": "JIA",
"start_offset": 17,
"end_offset": 20,
"label": "dis"
}
] |
3.抗核抗体（antinuclearantibodies，ANAs）是与各种细胞核成分反应的自身抗体的总称，包括针对脱氧核糖核酸（DNA）、可提取的核蛋白（snENP）、核糖核酸（RNA）、组蛋白、酶及核仁等细胞核成分的抗体。 | [
{
"id": 0,
"entity": "抗核抗体",
"start_offset": 2,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "antinuclearantibodies",
"start_offset": 7,
"end_offset": 28,
"label": "bod"
},
{
"id": 2,
"entity": "ANAs",
"start_offset": 29,
"end_offset": 33,
"label": "bod"
},
{
"id": 3,
"entity": "细胞核",
"start_offset": 38,
"end_offset": 41,
"label": "bod"
},
{
"id": 4,
"entity": "自身抗体",
"start_offset": 46,
"end_offset": 50,
"label": "bod"
},
{
"id": 5,
"entity": "脱氧核糖核酸",
"start_offset": 58,
"end_offset": 64,
"label": "bod"
},
{
"id": 6,
"entity": "DNA",
"start_offset": 65,
"end_offset": 68,
"label": "bod"
},
{
"id": 7,
"entity": "核蛋白",
"start_offset": 74,
"end_offset": 77,
"label": "bod"
},
{
"id": 8,
"entity": "snENP",
"start_offset": 78,
"end_offset": 83,
"label": "bod"
},
{
"id": 9,
"entity": "核糖核酸",
"start_offset": 85,
"end_offset": 89,
"label": "bod"
},
{
"id": 10,
"entity": "RNA",
"start_offset": 90,
"end_offset": 93,
"label": "bod"
},
{
"id": 11,
"entity": "组蛋白",
"start_offset": 95,
"end_offset": 98,
"label": "bod"
},
{
"id": 12,
"entity": "酶",
"start_offset": 99,
"end_offset": 100,
"label": "bod"
},
{
"id": 13,
"entity": "核仁",
"start_offset": 101,
"end_offset": 103,
"label": "bod"
},
{
"id": 14,
"entity": "细胞核",
"start_offset": 104,
"end_offset": 107,
"label": "bod"
}
] |
研究证明抗核抗体见于多种风湿性疾病，是筛查诊断SLE的指引性项目。 | [
{
"id": 0,
"entity": "抗核抗体",
"start_offset": 4,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "风湿性疾病",
"start_offset": 12,
"end_offset": 17,
"label": "dis"
},
{
"id": 2,
"entity": "SLE",
"start_offset": 23,
"end_offset": 26,
"label": "dis"
}
] |
几乎所有SLE病人，50%硬皮病，25%皮肌炎和超过50%小年龄JIA，如少关节型女性患儿及RF阳性多关节型患儿会有ANA阳性结果。 | [
{
"id": 0,
"entity": "SLE",
"start_offset": 4,
"end_offset": 7,
"label": "dis"
},
{
"id": 1,
"entity": "硬皮病",
"start_offset": 13,
"end_offset": 16,
"label": "dis"
},
{
"id": 2,
"entity": "皮肌炎",
"start_offset": 20,
"end_offset": 23,
"label": "dis"
},
{
"id": 3,
"entity": "JIA",
"start_offset": 32,
"end_offset": 35,
"label": "dis"
},
{
"id": 4,
"entity": "RF",
"start_offset": 46,
"end_offset": 48,
"label": "bod"
},
{
"id": 5,
"entity": "ANA",
"start_offset": 58,
"end_offset": 61,
"label": "bod"
}
] |
ANA阳性JIA常伴虹膜睫状体炎。 | [
{
"id": 0,
"entity": "ANA阳性JIA",
"start_offset": 0,
"end_offset": 8,
"label": "dis"
},
{
"id": 1,
"entity": "虹膜睫状体炎",
"start_offset": 10,
"end_offset": 16,
"label": "dis"
}
] |
健康儿童也有2%～9%的人ANA阳性，有人通过5年随访也未见这些ANA阳性健康儿童发展成为风湿性疾病。 | [
{
"id": 0,
"entity": "ANA",
"start_offset": 13,
"end_offset": 16,
"label": "bod"
},
{
"id": 1,
"entity": "ANA",
"start_offset": 32,
"end_offset": 35,
"label": "bod"
},
{
"id": 2,
"entity": "风湿性疾病",
"start_offset": 45,
"end_offset": 50,
"label": "dis"
}
] |
Miles和Isenberg（1993）发现在风湿性疾病病人的一、二级亲属中ANA阳性检出率较高。 | [
{
"id": 0,
"entity": "风湿性疾病",
"start_offset": 23,
"end_offset": 28,
"label": "dis"
},
{
"id": 1,
"entity": "ANA",
"start_offset": 38,
"end_offset": 41,
"label": "bod"
}
] |
ANA滴度与临床症状间并无必然联系，但它有助于疾病随访。 | [
{
"id": 0,
"entity": "ANA",
"start_offset": 0,
"end_offset": 3,
"label": "bod"
}
] |
在细菌、病毒及真菌感染，甚至患恶性肿瘤时ANA也见阳性。 | [
{
"id": 0,
"entity": "细菌",
"start_offset": 1,
"end_offset": 3,
"label": "mic"
},
{
"id": 1,
"entity": "病毒",
"start_offset": 4,
"end_offset": 6,
"label": "mic"
},
{
"id": 2,
"entity": "真菌",
"start_offset": 7,
"end_offset": 9,
"label": "mic"
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{
"id": 3,
"entity": "恶性肿瘤",
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"label": "dis"
},
{
"id": 4,
"entity": "ANA",
"start_offset": 20,
"end_offset": 23,
"label": "bod"
}
] |
4.抗核抗体谱包括抗DNA抗体、抗组蛋白抗体、抗DNA组蛋白复合物抗体以及抗非组蛋白4种大类的自身抗体。 | [
{
"id": 0,
"entity": "抗核抗体",
"start_offset": 2,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "抗DNA抗体",
"start_offset": 9,
"end_offset": 15,
"label": "bod"
},
{
"id": 2,
"entity": "抗组蛋白抗体",
"start_offset": 16,
"end_offset": 22,
"label": "bod"
},
{
"id": 3,
"entity": "抗DNA组蛋白复合物抗体",
"start_offset": 23,
"end_offset": 35,
"label": "bod"
},
{
"id": 4,
"entity": "抗非组蛋白",
"start_offset": 37,
"end_offset": 42,
"label": "bod"
},
{
"id": 5,
"entity": "自身抗体",
"start_offset": 47,
"end_offset": 51,
"label": "bod"
}
] |
目前国内普遍采用德国欧蒙试剂的抗核抗体谱检测，包括抗SS-A、抗SS-B、抗-Sm、抗Scl-70、抗Jo-1、抗Ro-52抗核小体抗体、抗组蛋白抗体、抗CNEPB、核糖体P蛋白抗体和抗nRNP/Sm等12种抗原的IgG抗体。 | [
{
"id": 0,
"entity": "德国欧蒙试剂",
"start_offset": 8,
"end_offset": 14,
"label": "pro"
},
{
"id": 1,
"entity": "抗核抗体谱检测",
"start_offset": 15,
"end_offset": 22,
"label": "pro"
},
{
"id": 2,
"entity": "抗SS-A",
"start_offset": 25,
"end_offset": 30,
"label": "bod"
},
{
"id": 3,
"entity": "抗SS-B",
"start_offset": 31,
"end_offset": 36,
"label": "bod"
},
{
"id": 4,
"entity": "抗-Sm",
"start_offset": 37,
"end_offset": 41,
"label": "bod"
},
{
"id": 5,
"entity": "抗Scl-70",
"start_offset": 42,
"end_offset": 49,
"label": "bod"
},
{
"id": 6,
"entity": "抗Jo-1",
"start_offset": 50,
"end_offset": 55,
"label": "bod"
},
{
"id": 7,
"entity": "抗Ro-52抗核小体抗体",
"start_offset": 56,
"end_offset": 68,
"label": "bod"
},
{
"id": 8,
"entity": "抗组蛋白抗体",
"start_offset": 69,
"end_offset": 75,
"label": "bod"
},
{
"id": 9,
"entity": "抗CNEPB",
"start_offset": 76,
"end_offset": 82,
"label": "bod"
},
{
"id": 10,
"entity": "核糖体P蛋白抗体",
"start_offset": 83,
"end_offset": 91,
"label": "bod"
},
{
"id": 11,
"entity": "抗nRNP/Sm",
"start_offset": 92,
"end_offset": 100,
"label": "bod"
},
{
"id": 12,
"entity": "IgG抗体",
"start_offset": 107,
"end_offset": 112,
"label": "bod"
}
] |
除抗dsDNA外，可提取核抗原（ENA）抗体中诊断意义较大的有抗-Sm、抗nRNP/Sm、抗SS-A、抗SS-B、抗Scl-70以及抗Jo-1六种自身抗体：①抗dsDNA：抗dsDNA抗体检测对诊断SLE具极高特异性（＞95%），且对SLE预后判断也有重要价值，但其敏感性只有30%～40%。 | [
{
"id": 0,
"entity": "抗dsDNA",
"start_offset": 1,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "核抗原（ENA）抗体",
"start_offset": 12,
"end_offset": 22,
"label": "bod"
},
{
"id": 2,
"entity": "抗-Sm",
"start_offset": 31,
"end_offset": 35,
"label": "bod"
},
{
"id": 3,
"entity": "抗nRNP/Sm",
"start_offset": 36,
"end_offset": 44,
"label": "bod"
},
{
"id": 4,
"entity": "抗SS-A",
"start_offset": 45,
"end_offset": 50,
"label": "bod"
},
{
"id": 5,
"entity": "抗SS-B",
"start_offset": 51,
"end_offset": 56,
"label": "bod"
},
{
"id": 6,
"entity": "抗Scl-70",
"start_offset": 57,
"end_offset": 64,
"label": "bod"
},
{
"id": 7,
"entity": "抗Jo-1",
"start_offset": 66,
"end_offset": 71,
"label": "bod"
},
{
"id": 8,
"entity": "自身抗体",
"start_offset": 73,
"end_offset": 77,
"label": "bod"
},
{
"id": 9,
"entity": "抗dsDNA",
"start_offset": 79,
"end_offset": 85,
"label": "bod"
},
{
"id": 10,
"entity": "抗dsDNA抗体检测",
"start_offset": 86,
"end_offset": 96,
"label": "pro"
},
{
"id": 11,
"entity": "SLE",
"start_offset": 99,
"end_offset": 102,
"label": "dis"
},
{
"id": 12,
"entity": "SLE",
"start_offset": 117,
"end_offset": 120,
"label": "dis"
}
] |
高滴度抗dsDNA抗体不仅表明SLE的活动性，而且提示病变累及肾脏的可能性极大。 | [
{
"id": 0,
"entity": "高滴度抗dsDNA抗体",
"start_offset": 0,
"end_offset": 11,
"label": "bod"
},
{
"id": 1,
"entity": "SLE",
"start_offset": 15,
"end_offset": 18,
"label": "dis"
},
{
"id": 2,
"entity": "肾脏",
"start_offset": 31,
"end_offset": 33,
"label": "bod"
}
] |
dsDNA抗体阳性率易受皮质激素和免疫抑制剂使用影响。 | [
{
"id": 0,
"entity": "dsDNA抗体",
"start_offset": 0,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "皮质激素",
"start_offset": 12,
"end_offset": 16,
"label": "dru"
},
{
"id": 2,
"entity": "免疫抑制剂",
"start_offset": 17,
"end_offset": 22,
"label": "dru"
}
] |
抗ssDNA抗体阳性率在SLE中高于抗dsDNA抗体，但其特异性不如后者；在其他AID，甚至病毒感染性疾病中也可见到抗ssDNA抗体阳性。 | [
{
"id": 0,
"entity": "抗ssDNA抗体",
"start_offset": 0,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "SLE",
"start_offset": 12,
"end_offset": 15,
"label": "dis"
},
{
"id": 2,
"entity": "抗dsDNA抗体",
"start_offset": 18,
"end_offset": 26,
"label": "bod"
},
{
"id": 3,
"entity": "AID",
"start_offset": 40,
"end_offset": 43,
"label": "dis"
},
{
"id": 4,
"entity": "病毒感染性疾病",
"start_offset": 46,
"end_offset": 53,
"label": "dis"
},
{
"id": 5,
"entity": "抗ssDNA抗体",
"start_offset": 58,
"end_offset": 66,
"label": "bod"
}
] |
②抗-Sm抗体：抗-Sm抗体也是SLE的标志性抗体，诊断特异性也超过95%，敏感性在40%左右。 | [
{
"id": 0,
"entity": "抗-Sm抗体",
"start_offset": 1,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "抗-Sm抗体",
"start_offset": 8,
"end_offset": 14,
"label": "bod"
},
{
"id": 2,
"entity": "SLE",
"start_offset": 16,
"end_offset": 19,
"label": "dis"
}
] |
③抗nRNP/Sm：抗nRNP/Sm对混合性结缔组织病阳性率可达100%，但SLE及干燥综合征也有抗nRNP/Sm阳性检出。 | [
{
"id": 0,
"entity": "抗nRNP/Sm",
"start_offset": 1,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "抗nRNP/Sm",
"start_offset": 10,
"end_offset": 18,
"label": "bod"
},
{
"id": 2,
"entity": "混合性结缔组织病",
"start_offset": 19,
"end_offset": 27,
"label": "dis"
},
{
"id": 3,
"entity": "SLE",
"start_offset": 38,
"end_offset": 41,
"label": "dis"
},
{
"id": 4,
"entity": "干燥综合征",
"start_offset": 42,
"end_offset": 47,
"label": "dis"
},
{
"id": 5,
"entity": "抗nRNP/Sm",
"start_offset": 49,
"end_offset": 57,
"label": "bod"
}
] |
④抗SS-A及抗SS-B：抗SS-A和抗SS-B在干燥综合征中阳性检出率分别为75%和60%，但也见于SLE、硬皮病、皮肌炎以及关节炎等患者。 | [
{
"id": 0,
"entity": "抗SS-A",
"start_offset": 1,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "抗SS-B",
"start_offset": 7,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "抗SS-A",
"start_offset": 13,
"end_offset": 18,
"label": "bod"
},
{
"id": 3,
"entity": "抗SS-B",
"start_offset": 19,
"end_offset": 24,
"label": "bod"
},
{
"id": 4,
"entity": "干燥综合征",
"start_offset": 25,
"end_offset": 30,
"label": "dis"
},
{
"id": 5,
"entity": "SLE",
"start_offset": 51,
"end_offset": 54,
"label": "dis"
},
{
"id": 6,
"entity": "硬皮病",
"start_offset": 55,
"end_offset": 58,
"label": "dis"
},
{
"id": 7,
"entity": "皮肌炎",
"start_offset": 59,
"end_offset": 62,
"label": "dis"
},
{
"id": 8,
"entity": "关节炎",
"start_offset": 64,
"end_offset": 67,
"label": "dis"
}
] |
⑤抗Scl-70：抗Scl-70为硬皮病标志性抗体，但阳性率不足50%。 | [
{
"id": 0,
"entity": "抗Scl-70",
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{
"id": 1,
"entity": "抗Scl-70",
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"end_offset": 16,
"label": "bod"
},
{
"id": 2,
"entity": "硬皮病",
"start_offset": 17,
"end_offset": 20,
"label": "dis"
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] |
⑥抗Jo-1：抗Jo-1见于多肌炎患者，阳性率仅25%～35%，常与合并肺间质纤维化有关。 | [
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"id": 0,
"entity": "抗Jo-1",
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{
"id": 1,
"entity": "抗Jo-1",
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{
"id": 2,
"entity": "多肌炎",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
},
{
"id": 3,
"entity": "肺间质纤维化",
"start_offset": 36,
"end_offset": 42,
"label": "dis"
}
] |
5.其他细胞成分自身抗体这类抗体仅与专一的细胞成分反应，引起相应的细胞病变也较为局限，如：（1）抗细胞抗体：如抗淋巴细胞、红细胞、血小板及精子细胞抗体，引起临床所见淋巴细胞坏死性、增生性疾病及自身免疫性溶血症、血小板减少症和不孕症等疾病。 | [
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"entity": "细胞",
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"id": 1,
"entity": "自身抗体",
"start_offset": 8,
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"label": "bod"
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"entity": "抗体",
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"entity": "细胞",
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"entity": "细胞",
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{
"id": 5,
"entity": "抗细胞抗体",
"start_offset": 48,
"end_offset": 53,
"label": "bod"
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{
"id": 6,
"entity": "抗淋巴细胞",
"start_offset": 55,
"end_offset": 60,
"label": "bod"
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{
"id": 7,
"entity": "红细胞",
"start_offset": 61,
"end_offset": 64,
"label": "bod"
},
{
"id": 8,
"entity": "血小板",
"start_offset": 65,
"end_offset": 68,
"label": "bod"
},
{
"id": 9,
"entity": "精子细胞抗体",
"start_offset": 69,
"end_offset": 75,
"label": "bod"
},
{
"id": 10,
"entity": "淋巴细胞坏死",
"start_offset": 82,
"end_offset": 88,
"label": "dis"
},
{
"id": 11,
"entity": "增生性疾病",
"start_offset": 90,
"end_offset": 95,
"label": "dis"
},
{
"id": 12,
"entity": "自身免疫性溶血症",
"start_offset": 96,
"end_offset": 104,
"label": "dis"
},
{
"id": 13,
"entity": "血小板减少症",
"start_offset": 105,
"end_offset": 111,
"label": "dis"
},
{
"id": 14,
"entity": "不孕症",
"start_offset": 112,
"end_offset": 115,
"label": "dis"
}
] |
（2）抗细胞质抗体：如抗线粒体抗体、核糖体抗体及溶酶体抗体等，引起相应的病变。 | [
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"id": 0,
"entity": "抗细胞质抗体",
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{
"id": 1,
"entity": "抗线粒体抗体",
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{
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"entity": "核糖体抗体",
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"label": "bod"
},
{
"id": 3,
"entity": "溶酶体抗体",
"start_offset": 24,
"end_offset": 29,
"label": "bod"
}
] |
（3）抗受体抗体：如抗乙酰胆碱受体及抗胰岛素受体抗体等所引起的自身免疫性神经传导性病变（自身免疫性重症肌无力）和胰腺等神经内分泌相关疾病。 | [
{
"id": 0,
"entity": "抗受体抗体",
"start_offset": 3,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "抗乙酰胆碱受体",
"start_offset": 10,
"end_offset": 17,
"label": "bod"
},
{
"id": 2,
"entity": "抗胰岛素受体抗体",
"start_offset": 18,
"end_offset": 26,
"label": "bod"
},
{
"id": 3,
"entity": "自身免疫性神经传导性病变",
"start_offset": 31,
"end_offset": 43,
"label": "dis"
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{
"id": 4,
"entity": "自身免疫性重症肌无力",
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"end_offset": 54,
"label": "dis"
},
{
"id": 5,
"entity": "胰腺",
"start_offset": 56,
"end_offset": 58,
"label": "bod"
}
] |
（4）抗中性粒细胞胞浆抗体（ANCA）：ANCA是近年研究较多的自身抗体之一，ANCA主要有两个亚型，即抗蛋白酶3（PR3）抗体，称为胞浆型ANCA（C-ANCA）和抗髓过氧化物酶（MPO）抗体，称为核周型ANCA（P-ANCA）。 | [
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"entity": "抗中性粒细胞胞浆抗体",
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{
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{
"id": 5,
"entity": "抗蛋白酶3（PR3）抗体",
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{
"id": 6,
"entity": "胞浆型ANCA",
"start_offset": 67,
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{
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"entity": "C-ANCA",
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{
"id": 8,
"entity": "抗髓过氧化物酶",
"start_offset": 83,
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"label": "bod"
},
{
"id": 9,
"entity": "MPO",
"start_offset": 91,
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{
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"entity": "核周型ANCA",
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},
{
"id": 11,
"entity": "P-ANCA",
"start_offset": 108,
"end_offset": 114,
"label": "bod"
}
] |
ANCA主要出现在以系统性小血管炎为基础病变的AID患者血中，如Wegener肉芽肿、镜下多小动脉炎、系统性血管炎、川崎病、SLE以及过敏性紫癜等。 | [
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"id": 1,
"entity": "系统性小血管炎",
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"entity": "AID",
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{
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"entity": "Wegener肉芽肿",
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"entity": "镜下多小动脉炎",
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"entity": "系统性血管炎",
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{
"id": 6,
"entity": "川崎病",
"start_offset": 58,
"end_offset": 61,
"label": "dis"
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{
"id": 7,
"entity": "SLE",
"start_offset": 62,
"end_offset": 65,
"label": "dis"
},
{
"id": 8,
"entity": "过敏性紫癜",
"start_offset": 67,
"end_offset": 72,
"label": "dis"
}
] |
临床上把ANCA视为小血管炎并肾炎的特殊血清学标志。 | [
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"entity": "ANCA",
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{
"id": 2,
"entity": "肾炎",
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] |
（5）抗磷脂抗体（aPL抗体）：aPL抗体是一组非均一性抗体，磷脂是从牛心组织中提取的一种抗原，命名为心磷脂。 | [
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"entity": "抗磷脂抗体",
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"id": 1,
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"id": 2,
"entity": "aPL抗体",
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"label": "bod"
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{
"id": 3,
"entity": "磷脂",
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"end_offset": 33,
"label": "bod"
},
{
"id": 4,
"entity": "心磷脂",
"start_offset": 51,
"end_offset": 54,
"label": "bod"
}
] |
临床上aPL分为两种，即狼疮凝集物（LAC）和抗心磷脂抗体（aCL）。 | [
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"id": 0,
"entity": "aPL",
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{
"id": 1,
"entity": "狼疮凝集物",
"start_offset": 12,
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"label": "bod"
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{
"id": 2,
"entity": "LAC",
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{
"id": 3,
"entity": "抗心磷脂抗体",
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"label": "bod"
},
{
"id": 4,
"entity": "aCL",
"start_offset": 30,
"end_offset": 33,
"label": "bod"
}
] |
LAC能与凝血酶原复合物中磷脂成分结合而抑制凝血作用，可能与SLE病人出血及凝血机制异常有关，实验发现aCL抗体有laG与IgM两种，IgG型与AID并流产、血栓形成及血小板减少有关；IgM型与AID并溶血性贫血、粒细胞减少、网状青斑及心内膜病变有关。 | [
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"id": 0,
"entity": "LAC",
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},
{
"id": 1,
"entity": "凝血酶原复合物",
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"label": "bod"
},
{
"id": 2,
"entity": "磷脂",
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"label": "bod"
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{
"id": 3,
"entity": "SLE",
"start_offset": 30,
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"label": "dis"
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{
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"entity": "aCL抗体",
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{
"id": 5,
"entity": "laG",
"start_offset": 57,
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{
"id": 6,
"entity": "IgM",
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"end_offset": 64,
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{
"id": 7,
"entity": "IgG",
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"label": "bod"
},
{
"id": 8,
"entity": "AID",
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"end_offset": 75,
"label": "dis"
},
{
"id": 9,
"entity": "流产",
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"end_offset": 78,
"label": "dis"
},
{
"id": 10,
"entity": "血栓",
"start_offset": 79,
"end_offset": 81,
"label": "bod"
},
{
"id": 11,
"entity": "血小板",
"start_offset": 84,
"end_offset": 87,
"label": "bod"
},
{
"id": 12,
"entity": "IgM",
"start_offset": 92,
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"label": "bod"
},
{
"id": 13,
"entity": "AID",
"start_offset": 97,
"end_offset": 100,
"label": "dis"
},
{
"id": 14,
"entity": "溶血性贫血",
"start_offset": 101,
"end_offset": 106,
"label": "dis"
},
{
"id": 15,
"entity": "粒细胞减少",
"start_offset": 107,
"end_offset": 112,
"label": "dis"
},
{
"id": 16,
"entity": "网状青斑",
"start_offset": 113,
"end_offset": 117,
"label": "dis"
},
{
"id": 17,
"entity": "心内膜病变",
"start_offset": 118,
"end_offset": 123,
"label": "dis"
}
] |
（6）抗神经节苷脂抗体（GM抗体）：抗GM抗体针对髓鞘脂类成分，AID中抗GM抗体主要引起神经及精神病变，IgM型抗GM抗体与SLE脑病高度相关。 | [
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"entity": "抗神经节苷脂抗体",
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"label": "bod"
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{
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"entity": "GM抗体",
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"label": "bod"
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{
"id": 2,
"entity": "抗GM抗体",
"start_offset": 18,
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"label": "bod"
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{
"id": 3,
"entity": "髓鞘脂类",
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"label": "bod"
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{
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"entity": "AID",
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"label": "dis"
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{
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"entity": "抗GM抗体",
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{
"id": 6,
"entity": "神经",
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},
{
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"entity": "IgM型抗GM抗体",
"start_offset": 53,
"end_offset": 62,
"label": "bod"
},
{
"id": 8,
"entity": "SLE脑病",
"start_offset": 63,
"end_offset": 68,
"label": "dis"
}
] |
检测抗GM抗体可能有预测SLE神经系统并发症的价值，尤其是脑脊液中抗GM抗体检测。 | [
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{
"id": 3,
"entity": "抗GM抗体检测",
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"label": "pro"
}
] |
6.器官特异性自身抗体同细胞专一成分的自身抗体一样，器官特异性自身抗体主要针对某些器官组织并引起器官特异性AID。 | [
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{
"id": 1,
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{
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"entity": "自身抗体",
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"label": "bod"
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{
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"entity": "器官特异性自身抗体",
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{
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"entity": "器官组织",
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{
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"entity": "器官特异性AID",
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] |
（1）抗脑组织抗体：有41.5%的精神分裂症患者、37%脑血管意外患者血清中可检出抗脑组织抗体，而正常人中该抗体阳性率仅1.4%，先天性脑发育不全者该抗体阳性率为9.1%。 | [
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"entity": "抗脑组织抗体",
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"label": "bod"
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{
"id": 1,
"entity": "精神分裂症",
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{
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{
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"entity": "血清",
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{
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"entity": "抗脑组织抗体",
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},
{
"id": 5,
"entity": "先天性脑发育不全",
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"label": "dis"
}
] |
37例癫痫患者中抗脑组织抗体阳性率也皆高于正常对照组，在风湿性疾病脑损害中的诊断价值有待研究。 | [
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"id": 0,
"entity": "癫痫",
"start_offset": 3,
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"label": "dis"
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{
"id": 1,
"entity": "抗脑组织抗体",
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{
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},
{
"id": 3,
"entity": "脑损害",
"start_offset": 33,
"end_offset": 36,
"label": "dis"
}
] |
（2）抗心肌M受体抗体：抗心肌抗体的靶抗原十分复杂，可针对肌纤维、肌动蛋白、肌凝蛋白及Z带等抗原成分，因此在心肌梗死、风湿性心肌炎、病毒性心肌炎、心肌病、冠状动脉硬化性心脏病，甚至心脏手术之后，均可检出抗心肌抗体。 | [
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"entity": "抗心肌M受体抗体",
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{
"id": 1,
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"label": "bod"
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{
"id": 2,
"entity": "靶抗原",
"start_offset": 18,
"end_offset": 21,
"label": "mic"
},
{
"id": 3,
"entity": "肌纤维",
"start_offset": 29,
"end_offset": 32,
"label": "bod"
},
{
"id": 4,
"entity": "肌动蛋白",
"start_offset": 33,
"end_offset": 37,
"label": "bod"
},
{
"id": 5,
"entity": "肌凝蛋白",
"start_offset": 38,
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"label": "bod"
},
{
"id": 6,
"entity": "Z带",
"start_offset": 43,
"end_offset": 45,
"label": "bod"
},
{
"id": 7,
"entity": "心肌梗死",
"start_offset": 54,
"end_offset": 58,
"label": "dis"
},
{
"id": 8,
"entity": "风湿性心肌炎",
"start_offset": 59,
"end_offset": 65,
"label": "dis"
},
{
"id": 9,
"entity": "病毒性心肌炎",
"start_offset": 66,
"end_offset": 72,
"label": "dis"
},
{
"id": 10,
"entity": "心肌病",
"start_offset": 73,
"end_offset": 76,
"label": "dis"
},
{
"id": 11,
"entity": "冠状动脉硬化性心脏病",
"start_offset": 77,
"end_offset": 87,
"label": "dis"
},
{
"id": 12,
"entity": "心脏手术",
"start_offset": 90,
"end_offset": 94,
"label": "pro"
},
{
"id": 13,
"entity": "抗心肌抗体",
"start_offset": 101,
"end_offset": 106,
"label": "bod"
}
] |
目前难以确定是组织损伤，自身抗原改变刺激产生自身抗体，还是感染、药物等诱生的自身抗体。 | [
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"id": 0,
"entity": "自身抗体",
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"end_offset": 26,
"label": "bod"
},
{
"id": 1,
"entity": "自身抗体",
"start_offset": 38,
"end_offset": 42,
"label": "bod"
}
] |
（3）抗甲状腺球蛋白及抗甲状腺微粒体抗体（TGA与TPO）：在桥本甲状腺炎患者血清中高滴度TGA90%～95%，TPO检测也有相应诊断价值。 | [
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"id": 0,
"entity": "抗甲状腺球蛋白",
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"end_offset": 10,
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{
"id": 1,
"entity": "抗甲状腺微粒体抗体",
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{
"id": 2,
"entity": "TGA",
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{
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{
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"entity": "桥本甲状腺炎",
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{
"id": 5,
"entity": "血清",
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{
"id": 6,
"entity": "高滴度TG",
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},
{
"id": 7,
"entity": "高滴度TG",
"start_offset": 42,
"end_offset": 47,
"label": "bod"
}
] |
（4）类风湿关节炎特异性抗体：多年来在RA患者中（尤其是儿童RA）难以检出特异性抗体，一直是令医师头痛的问题。 | [
{
"id": 0,
"entity": "类风湿关节炎特异性抗体",
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"end_offset": 14,
"label": "bod"
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{
"id": 1,
"entity": "RA",
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{
"id": 2,
"entity": "儿童RA",
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"label": "dis"
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{
"id": 3,
"entity": "特异性抗体",
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"label": "bod"
}
] |
最近发现一种被称为Sa的抗原物质，其相对分子质量为50000或55000，属于人体组织的正常成分。 | [
{
"id": 0,
"entity": "Sa",
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] |
抗Sa抗体在成人RA中阳性率为31.9%，在SLE为4.3%，干燥综合征为3%。 | [
{
"id": 0,
"entity": "抗Sa抗体",
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{
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"id": 2,
"entity": "SLE",
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{
"id": 3,
"entity": "干燥综合征",
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"label": "dis"
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] |
抗Sa抗体对RA诊断特异性为98.6%。 | [
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"entity": "抗Sa抗体",
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{
"id": 1,
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] |
研究发现抗Sa抗体与RF、RA3、SSA、SSB、RNP、Sm、Jo-1及Scl-70等多种自身抗体无交叉反应性，遗憾的是Sa抗体对JIA的诊断价值罕见报告。 | [
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"entity": "抗Sa抗体",
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{
"id": 1,
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"label": "bod"
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{
"id": 2,
"entity": "RA3",
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"entity": "SSA",
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{
"id": 4,
"entity": "SSB",
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{
"id": 5,
"entity": "RNP",
"start_offset": 25,
"end_offset": 28,
"label": "bod"
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{
"id": 6,
"entity": "Sm",
"start_offset": 29,
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{
"id": 7,
"entity": "Jo-1",
"start_offset": 32,
"end_offset": 36,
"label": "bod"
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{
"id": 8,
"entity": "自身抗体",
"start_offset": 46,
"end_offset": 50,
"label": "bod"
},
{
"id": 9,
"entity": "Sa抗体",
"start_offset": 61,
"end_offset": 65,
"label": "bod"
},
{
"id": 10,
"entity": "JIA",
"start_offset": 66,
"end_offset": 69,
"label": "dis"
}
] |
抗环瓜氨酸抗体（ACCP）：研究表明，JIA患儿ACCP为阳性率不足2%，远低于成年（63%）。 | [
{
"id": 0,
"entity": "抗环瓜氨酸抗体",
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"end_offset": 7,
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{
"id": 1,
"entity": "ACCP",
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{
"id": 2,
"entity": "JIA",
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{
"id": 3,
"entity": "ACCP",
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"end_offset": 28,
"label": "bod"
}
] |
因此，ACCP难以作为JIA诊断的筛选手段。 | [
{
"id": 0,
"entity": "ACCP",
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"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "JIA",
"start_offset": 11,
"end_offset": 14,
"label": "dis"
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] |
但有报告13%的多关节型和2%的其他类型JIA血清中ACCP抗体为阳性，健康对照仅0.6%阳性，其中RF阳性多关节型JIA患儿中57%ACCP抗体为阳性。 | [
{
"id": 0,
"entity": "JIA",
"start_offset": 20,
"end_offset": 23,
"label": "dis"
},
{
"id": 1,
"entity": "血清",
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"label": "bod"
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{
"id": 2,
"entity": "ACCP抗体",
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"end_offset": 32,
"label": "bod"
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{
"id": 3,
"entity": "RF阳性多关节型JIA",
"start_offset": 50,
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"label": "dis"
},
{
"id": 4,
"entity": "ACCP抗体",
"start_offset": 67,
"end_offset": 73,
"label": "bod"
}
] |
HLA-DR4阳性多关节型患儿ACCP抗体阳性率高于HLA-DR4阴性的患儿，且与多关节型发病、病程和关节破坏程度有关。 | [
{
"id": 0,
"entity": "HLA-DR4",
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{
"id": 1,
"entity": "ACCP抗体",
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"label": "bod"
},
{
"id": 2,
"entity": "HLA-DR4",
"start_offset": 26,
"end_offset": 33,
"label": "bod"
}
] |
抗核周因子抗体（APF）：NesherG检测64名JIA患儿（28名多关节型，26名少关节型，10名全身型），结果多关节型中APF10名阳性，少关节型中5名阳性，全身型中1名阳性。 | [
{
"id": 0,
"entity": "抗核周因子抗体",
"start_offset": 0,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "APF",
"start_offset": 8,
"end_offset": 11,
"label": "bod"
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{
"id": 2,
"entity": "NesherG检测",
"start_offset": 13,
"end_offset": 22,
"label": "pro"
},
{
"id": 3,
"entity": "JIA",
"start_offset": 25,
"end_offset": 28,
"label": "dis"
},
{
"id": 4,
"entity": "APF",
"start_offset": 62,
"end_offset": 65,
"label": "bod"
}
] |
遗憾的是，以上自身抗体能提示某种器官损害，其特异性及阳性率报告差异极大，以器官疾病命名的自身抗体不一定具有诊断该病的高度特异性，临床医师绝不能僵化地看待自身抗体的报告。 | [
{
"id": 0,
"entity": "自身抗",
"start_offset": 7,
"end_offset": 10,
"label": "ite"
},
{
"id": 1,
"entity": "自身抗",
"start_offset": 7,
"end_offset": 10,
"label": "bod"
},
{
"id": 2,
"entity": "提示",
"start_offset": 12,
"end_offset": 14,
"label": "bod"
},
{
"id": 3,
"entity": "极大",
"start_offset": 33,
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"label": "bod"
},
{
"id": 4,
"entity": "病命名的",
"start_offset": 40,
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"label": "bod"
},
{
"id": 5,
"entity": "化地看待",
"start_offset": 72,
"end_offset": 76,
"label": "bod"
}
] |
临床检测自身抗体要注意3点：①自身抗体有生理性与病理性之分，生理性自身抗体不但效价低，产生机制也可能不同；②多数自身抗体特异性欠佳，要特别强调临床综合分析；③自身抗体仅用于风湿性疾病诊断，在一些非风湿性疾病中也通过自身抗体检测获取临床信息，如肺癌诊断，淋巴细胞白血病分型等。 | [
{
"id": 0,
"entity": "自身抗体",
"start_offset": 4,
"end_offset": 8,
"label": "bod"
},
{
"id": 1,
"entity": "自身抗体",
"start_offset": 15,
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"label": "bod"
},
{
"id": 2,
"entity": "自身抗体",
"start_offset": 33,
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{
"id": 3,
"entity": "自身抗体",
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{
"id": 4,
"entity": "自身抗",
"start_offset": 79,
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},
{
"id": 5,
"entity": "自身抗",
"start_offset": 79,
"end_offset": 82,
"label": "bod"
},
{
"id": 6,
"entity": "体仅用于风",
"start_offset": 82,
"end_offset": 87,
"label": "dis"
},
{
"id": 7,
"entity": "在一些非风",
"start_offset": 94,
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},
{
"id": 8,
"entity": "中也通过自身",
"start_offset": 103,
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"label": "pro"
},
{
"id": 9,
"entity": "信息",
"start_offset": 117,
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"label": "dis"
},
{
"id": 10,
"entity": "癌诊断，淋巴细",
"start_offset": 122,
"end_offset": 129,
"label": "dis"
}
] |
第七章心肌病心肌病（cardiomyopathy）为发生于心肌的疾病。 | [
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"entity": "心肌病",
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{
"id": 1,
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{
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"entity": "心肌",
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}
] |
该术语最初出现于1957年，当时指一组不能归因于冠状动脉病变的心肌病变。 | [
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"entity": "冠状动脉",
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{
"id": 1,
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"start_offset": 31,
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] |
此后，心肌病的定义发生了变化。 | [
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"id": 0,
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"label": "dis"
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] |
目前，心肌病的定义为心肌的结构或功能异常，且无高血压或肺动脉高压、无心脏瓣膜病变、无先天性心脏病而言。 | [
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"id": 0,
"entity": "心肌病",
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{
"id": 1,
"entity": "心肌",
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{
"id": 2,
"entity": "高血压",
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"label": "dis"
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{
"id": 3,
"entity": "肺动脉高压",
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"label": "dis"
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{
"id": 4,
"entity": "心脏瓣膜病变",
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"label": "dis"
},
{
"id": 5,
"entity": "心脏病",
"start_offset": 45,
"end_offset": 48,
"label": "dis"
}
] |
以解剖与生理改变为依据，可将心肌病分为以下三型：①扩张（充血）型心肌病：此型左心室或双心室扩大，心肌收缩功能不同程度降低充血性心力衰竭的症状与体征。 | [
{
"id": 0,
"entity": "心肌病",
"start_offset": 14,
"end_offset": 17,
"label": "dis"
},
{
"id": 1,
"entity": "扩张（充血）型心肌病",
"start_offset": 25,
"end_offset": 35,
"label": "dis"
},
{
"id": 2,
"entity": "心肌",
"start_offset": 48,
"end_offset": 50,
"label": "bod"
},
{
"id": 3,
"entity": "心肌收缩功能不同程度降低",
"start_offset": 48,
"end_offset": 60,
"label": "sym"
},
{
"id": 4,
"entity": "充血性心力衰竭",
"start_offset": 60,
"end_offset": 67,
"label": "sym"
}
] |
②肥厚性心肌病：先前称之为特发性肥厚性心肌病，以左心室肥厚左心室流出道梗阻舒张功能障碍或心律失常引起猝死。 | [
{
"id": 0,
"entity": "肥厚性心肌病",
"start_offset": 1,
"end_offset": 7,
"label": "dis"
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{
"id": 1,
"entity": "左心室",
"start_offset": 24,
"end_offset": 27,
"label": "bod"
},
{
"id": 2,
"entity": "左心室肥厚",
"start_offset": 24,
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"label": "sym"
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{
"id": 3,
"entity": "左心室",
"start_offset": 29,
"end_offset": 32,
"label": "bod"
},
{
"id": 4,
"entity": "左心室流出道梗阻",
"start_offset": 29,
"end_offset": 37,
"label": "sym"
},
{
"id": 5,
"entity": "舒张功能障碍",
"start_offset": 37,
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"label": "sym"
},
{
"id": 6,
"entity": "心律",
"start_offset": 44,
"end_offset": 46,
"label": "ite"
},
{
"id": 7,
"entity": "心律失常引起猝死",
"start_offset": 44,
"end_offset": 52,
"label": "sym"
}
] |
③限制型心肌病（restrictivecardiomyopathy）：心房显著扩大，一般心室大小及收缩功能正常，舒张功能损害，症状由肺及体循环静脉充血引起，也可出现晕厥。 | [
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"id": 0,
"entity": "心室",
"start_offset": 44,
"end_offset": 46,
"label": "bod"
},
{
"id": 1,
"entity": "肺",
"start_offset": 66,
"end_offset": 67,
"label": "bod"
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{
"id": 2,
"entity": "体循环",
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"end_offset": 71,
"label": "bod"
},
{
"id": 3,
"entity": "晕厥",
"start_offset": 82,
"end_offset": 84,
"label": "sym"
}
] |
第一节扩张性心肌病【病因】【扩张性心肌病扩张性心肌病（dilatedcardiomyopathy，DCM）在各种类型心肌病中最为常见，在美国及欧洲，其年发病率约为2/10万～8/10万人口，据估计每10万人口中约有36人患有DCM。 | [
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"entity": "扩张性心肌病",
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{
"id": 1,
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{
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"label": "dis"
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{
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"entity": "心肌病",
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"label": "dis"
},
{
"id": 4,
"entity": "DCM",
"start_offset": 112,
"end_offset": 115,
"label": "dis"
}
] |
此外，20%～30%的DCM患者为家族性的。 | [
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"id": 0,
"entity": "DCM",
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] |
表9-10扩张性心肌病的病因【病理】扩张性心肌病病变以心肌纤维化为主，心肌肥厚不显著心腔扩大明显二尖瓣环和三尖瓣环增大，乳头肌伸长心腔内附壁血栓心肌节律点及传导系统而引起心律失常。 | [
{
"id": 0,
"entity": "扩张性心肌病",
"start_offset": 18,
"end_offset": 24,
"label": "dis"
},
{
"id": 1,
"entity": "心肌纤维化",
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"label": "sym"
},
{
"id": 2,
"entity": "心肌",
"start_offset": 35,
"end_offset": 37,
"label": "bod"
},
{
"id": 3,
"entity": "心肌肥厚不显著",
"start_offset": 35,
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"label": "sym"
},
{
"id": 4,
"entity": "心腔",
"start_offset": 42,
"end_offset": 44,
"label": "bod"
},
{
"id": 5,
"entity": "心腔扩大明显",
"start_offset": 42,
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"label": "sym"
},
{
"id": 6,
"entity": "二尖瓣环和三尖瓣环增大",
"start_offset": 48,
"end_offset": 59,
"label": "sym"
},
{
"id": 7,
"entity": "乳头肌",
"start_offset": 60,
"end_offset": 63,
"label": "bod"
},
{
"id": 8,
"entity": "乳头肌伸长",
"start_offset": 60,
"end_offset": 65,
"label": "sym"
},
{
"id": 9,
"entity": "心腔",
"start_offset": 65,
"end_offset": 67,
"label": "bod"
},
{
"id": 10,
"entity": "心腔内附壁血栓",
"start_offset": 65,
"end_offset": 72,
"label": "sym"
},
{
"id": 11,
"entity": "心肌",
"start_offset": 72,
"end_offset": 74,
"label": "bod"
},
{
"id": 12,
"entity": "心律失常",
"start_offset": 85,
"end_offset": 89,
"label": "sym"
}
] |
由于心肌纤维化，心肌收缩功能心力衰竭。 | [
{
"id": 0,
"entity": "心肌",
"start_offset": 8,
"end_offset": 10,
"label": "bod"
},
{
"id": 1,
"entity": "心肌收缩功能",
"start_offset": 8,
"end_offset": 14,
"label": "sym"
},
{
"id": 2,
"entity": "心力衰竭",
"start_offset": 14,
"end_offset": 18,
"label": "sym"
}
] |
主要表现为心脏增大心力衰竭，心律失常小动脉栓塞心脏增大气喘、乏力、心悸、咳嗽、胸闷等症状，有的可有偏瘫。 | [
{
"id": 0,
"entity": "心脏",
"start_offset": 5,
"end_offset": 7,
"label": "bod"
},
{
"id": 1,
"entity": "心脏增大",
"start_offset": 5,
"end_offset": 9,
"label": "sym"
},
{
"id": 2,
"entity": "心力衰竭",
"start_offset": 9,
"end_offset": 13,
"label": "sym"
},
{
"id": 3,
"entity": "心律",
"start_offset": 14,
"end_offset": 16,
"label": "ite"
},
{
"id": 4,
"entity": "心律失常",
"start_offset": 14,
"end_offset": 18,
"label": "sym"
},
{
"id": 5,
"entity": "动脉",
"start_offset": 19,
"end_offset": 21,
"label": "bod"
},
{
"id": 6,
"entity": "小动脉栓塞",
"start_offset": 18,
"end_offset": 23,
"label": "sym"
},
{
"id": 7,
"entity": "心脏",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
},
{
"id": 8,
"entity": "心脏增大",
"start_offset": 23,
"end_offset": 27,
"label": "sym"
},
{
"id": 9,
"entity": "气喘",
"start_offset": 27,
"end_offset": 29,
"label": "sym"
},
{
"id": 10,
"entity": "乏力",
"start_offset": 30,
"end_offset": 32,
"label": "sym"
},
{
"id": 11,
"entity": "心悸",
"start_offset": 33,
"end_offset": 35,
"label": "sym"
},
{
"id": 12,
"entity": "咳嗽",
"start_offset": 36,
"end_offset": 38,
"label": "sym"
},
{
"id": 13,
"entity": "胸闷",
"start_offset": 39,
"end_offset": 41,
"label": "sym"
},
{
"id": 14,
"entity": "偏瘫",
"start_offset": 49,
"end_offset": 51,
"label": "sym"
}
] |
体格检查可见心尖搏动弥散或抬举心浊音界向左扩大，心率增快可闻及Ⅱ/Ⅵ～Ⅲ/Ⅵ级收缩期杂音（心力衰竭控制后杂音减轻或消失），肝脏增大下肢水肿。 | [
{
"id": 0,
"entity": "体格检查",
"start_offset": 0,
"end_offset": 4,
"label": "ite"
},
{
"id": 1,
"entity": "心尖",
"start_offset": 6,
"end_offset": 8,
"label": "bod"
},
{
"id": 2,
"entity": "心尖搏动弥散或抬举",
"start_offset": 6,
"end_offset": 15,
"label": "sym"
},
{
"id": 3,
"entity": "心浊音界向左扩大",
"start_offset": 15,
"end_offset": 23,
"label": "sym"
},
{
"id": 4,
"entity": "心率",
"start_offset": 24,
"end_offset": 26,
"label": "ite"
},
{
"id": 5,
"entity": "心率增快",
"start_offset": 24,
"end_offset": 28,
"label": "sym"
},
{
"id": 6,
"entity": "可闻及Ⅱ/Ⅵ～Ⅲ/Ⅵ级收缩期杂音",
"start_offset": 28,
"end_offset": 44,
"label": "sym"
},
{
"id": 7,
"entity": "肝脏",
"start_offset": 61,
"end_offset": 63,
"label": "bod"
},
{
"id": 8,
"entity": "肝脏增大",
"start_offset": 61,
"end_offset": 65,
"label": "sym"
},
{
"id": 9,
"entity": "下肢",
"start_offset": 65,
"end_offset": 67,
"label": "bod"
},
{
"id": 10,
"entity": "下肢水肿",
"start_offset": 65,
"end_offset": 69,
"label": "sym"
}
] |
【实验室检查】（一）胸部X线检查心影扩大，由左心室、左心房扩大引起。 | [
{
"id": 0,
"entity": "胸部X线检查",
"start_offset": 10,
"end_offset": 16,
"label": "ite"
},
{
"id": 1,
"entity": "心影",
"start_offset": 16,
"end_offset": 18,
"label": "ite"
},
{
"id": 2,
"entity": "左心室",
"start_offset": 22,
"end_offset": 25,
"label": "bod"
},
{
"id": 3,
"entity": "左心房",
"start_offset": 26,
"end_offset": 29,
"label": "bod"
}
] |
常存在肺静脉充血，可发展为肺水肿。 | [
{
"id": 0,
"entity": "肺静脉",
"start_offset": 3,
"end_offset": 6,
"label": "bod"
},
{
"id": 1,
"entity": "肺水肿",
"start_offset": 13,
"end_offset": 16,
"label": "sym"
}
] |
左肺部分区域可因左心房扩大压迫左支气管而致不张胸腔积液。 | [
{
"id": 0,
"entity": "左肺",
"start_offset": 0,
"end_offset": 2,
"label": "bod"
},
{
"id": 1,
"entity": "左心房",
"start_offset": 8,
"end_offset": 11,
"label": "bod"
},
{
"id": 2,
"entity": "左支气管",
"start_offset": 15,
"end_offset": 19,
"label": "bod"
},
{
"id": 3,
"entity": "左肺部分区域可因左心房扩大压迫左支气管而致不张",
"start_offset": 0,
"end_offset": 23,
"label": "sym"
},
{
"id": 4,
"entity": "胸腔",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
},
{
"id": 5,
"entity": "胸腔积液",
"start_offset": 23,
"end_offset": 27,
"label": "sym"
}
] |
（二）心电图及HOLTER大多数患儿心电图上呈窦性心动过速左心室肥大左右心房扩大右心室肥大HOLTER检查可发现心律失常。 | [
{
"id": 0,
"entity": "心电图",
"start_offset": 3,
"end_offset": 6,
"label": "pro"
},
{
"id": 1,
"entity": "HOLTER",
"start_offset": 7,
"end_offset": 13,
"label": "pro"
},
{
"id": 2,
"entity": "心动",
"start_offset": 25,
"end_offset": 27,
"label": "ite"
},
{
"id": 3,
"entity": "窦性心动过速",
"start_offset": 23,
"end_offset": 29,
"label": "sym"
},
{
"id": 4,
"entity": "左心室",
"start_offset": 29,
"end_offset": 32,
"label": "bod"
},
{
"id": 5,
"entity": "左心室肥大",
"start_offset": 29,
"end_offset": 34,
"label": "sym"
},
{
"id": 6,
"entity": "心房",
"start_offset": 36,
"end_offset": 38,
"label": "bod"
},
{
"id": 7,
"entity": "左右心房扩大",
"start_offset": 34,
"end_offset": 40,
"label": "sym"
},
{
"id": 8,
"entity": "右心室",
"start_offset": 40,
"end_offset": 43,
"label": "bod"
},
{
"id": 9,
"entity": "右心室肥大",
"start_offset": 40,
"end_offset": 45,
"label": "sym"
},
{
"id": 10,
"entity": "HOLTER检查",
"start_offset": 45,
"end_offset": 53,
"label": "pro"
}
] |
（三）超声心动图DCM患儿的超声心动图特征包括左心室、左心房扩大，缩短分数及射血分数减低，左心室射血前期与射血期比率增加等。 | [
{
"id": 0,
"entity": "超声心动图",
"start_offset": 3,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "DCM",
"start_offset": 8,
"end_offset": 11,
"label": "dis"
},
{
"id": 2,
"entity": "左心室",
"start_offset": 23,
"end_offset": 26,
"label": "bod"
},
{
"id": 3,
"entity": "左心房",
"start_offset": 27,
"end_offset": 30,
"label": "bod"
},
{
"id": 4,
"entity": "左心室、左心房扩大",
"start_offset": 23,
"end_offset": 32,
"label": "sym"
}
] |
（四）心导管检查与活体组织检查由于DCM可由超声心动图检查确定，心导管检查主要用于排除异常的左冠状动脉起源，因这一情况在超声心动图检查时易于漏诊，必要时活体组织检查帮助确定心肌病的病因。 | [
{
"id": 0,
"entity": "心导管检查",
"start_offset": 3,
"end_offset": 8,
"label": "pro"
},
{
"id": 1,
"entity": "活体组织检查",
"start_offset": 9,
"end_offset": 15,
"label": "pro"
},
{
"id": 2,
"entity": "超声心动图检查",
"start_offset": 22,
"end_offset": 29,
"label": "pro"
},
{
"id": 3,
"entity": "心导管检查",
"start_offset": 32,
"end_offset": 37,
"label": "pro"
},
{
"id": 4,
"entity": "左冠状动脉",
"start_offset": 46,
"end_offset": 51,
"label": "bod"
},
{
"id": 5,
"entity": "超声心动图检查",
"start_offset": 60,
"end_offset": 67,
"label": "pro"
},
{
"id": 6,
"entity": "活体组织检查",
"start_offset": 76,
"end_offset": 82,
"label": "pro"
},
{
"id": 7,
"entity": "心肌病",
"start_offset": 86,
"end_offset": 89,
"label": "dis"
}
] |
【治疗】扩张性心肌病的临床特征为心输出量减少、液体潴留及血管收缩活性增加，后者为神经体液因素作用以维持足够的灌注压。 | [
{
"id": 0,
"entity": "扩张性心肌病",
"start_offset": 4,
"end_offset": 10,
"label": "dis"
},
{
"id": 1,
"entity": "心输出量减少",
"start_offset": 16,
"end_offset": 22,
"label": "sym"
},
{
"id": 2,
"entity": "液体潴留",
"start_offset": 23,
"end_offset": 27,
"label": "sym"
},
{
"id": 3,
"entity": "血管收缩活性增加",
"start_offset": 28,
"end_offset": 36,
"label": "sym"
}
] |
增强心肌收缩力的药物1.第一类为拟交感药物包括多巴胺、多巴酚丁胺及肾上腺素。 | [
{
"id": 0,
"entity": "多巴胺",
"start_offset": 23,
"end_offset": 26,
"label": "dru"
},
{
"id": 1,
"entity": "多巴酚丁胺",
"start_offset": 27,
"end_offset": 32,
"label": "dru"
},
{
"id": 2,
"entity": "肾上腺素",
"start_offset": 33,
"end_offset": 37,
"label": "dru"
}
] |
多巴胺小剂量时可改善肾脏功能，剂量加大可增强对心脏的作用，但也可引起外周血管阻力增加，并有可能致心律失常。 | [
{
"id": 0,
"entity": "多巴胺",
"start_offset": 0,
"end_offset": 3,
"label": "dru"
},
{
"id": 1,
"entity": "肾脏",
"start_offset": 10,
"end_offset": 12,
"label": "bod"
},
{
"id": 2,
"entity": "心脏",
"start_offset": 23,
"end_offset": 25,
"label": "bod"
},
{
"id": 3,
"entity": "血管",
"start_offset": 36,
"end_offset": 38,
"label": "bod"
},
{
"id": 4,
"entity": "心律",
"start_offset": 48,
"end_offset": 50,
"label": "ite"
}
] |
多巴酚丁胺致心律失常作用较弱，但有报道因可引起肺动脉楔压升高而致肺水肿。 | [
{
"id": 0,
"entity": "多巴酚丁胺",
"start_offset": 0,
"end_offset": 5,
"label": "dru"
},
{
"id": 1,
"entity": "心律",
"start_offset": 6,
"end_offset": 8,
"label": "ite"
},
{
"id": 2,
"entity": "肺动脉",
"start_offset": 23,
"end_offset": 26,
"label": "bod"
}
] |
2.第二类增强心肌收缩力的药物为双吡啶衍生剂包括氨力农及米力农，可通过抑制磷酸二酯酶增加细胞内钙的浓度，有强心及扩张外周血管的作用。 | [
{
"id": 0,
"entity": "心肌",
"start_offset": 7,
"end_offset": 9,
"label": "bod"
},
{
"id": 1,
"entity": "双吡啶衍生剂",
"start_offset": 16,
"end_offset": 22,
"label": "dru"
},
{
"id": 2,
"entity": "氨力农",
"start_offset": 24,
"end_offset": 27,
"label": "dru"
},
{
"id": 3,
"entity": "抑制磷酸二酯酶",
"start_offset": 35,
"end_offset": 42,
"label": "dru"
},
{
"id": 4,
"entity": "细胞",
"start_offset": 44,
"end_offset": 46,
"label": "mic"
},
{
"id": 5,
"entity": "血管",
"start_offset": 60,
"end_offset": 62,
"label": "bod"
}
] |
其可能的副作用为血小板减少、肝毒性及胃肠道刺激。 | [
{
"id": 0,
"entity": "血小板",
"start_offset": 8,
"end_offset": 11,
"label": "bod"
}
] |