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55-11-第十一节 抗磷脂综合征的诊断标准及治疗
一、诊断标准
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APS不是一个特定的疾病,只是一个临床的综合表现。目前还无一个临床表现的明确范围,而且症状逐渐增多,所涉及的病种也有扩大趋势,特别是实验方法、条件和确定正常值标准的差异,这就使得目前尚无一个非常严密的诊断标准。
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6 K [ }; n( `* @. m' E0 r7 X# } 这些诊断标准仅供参考,由于它未能涉及临床一些可能的表现,如心脏损伤,特别是很多学者发现aPL抗体和神经精神异常有关,其临床表现在该标准中也未涉及,尽管多数报道APS与aPL抗体滴度有关,但也有APS与aPL抗体滴度无关的报道。另外,疾病的不同时期也影响检查结果,部分病人在疾病活动期 aPL抗体滴度高,病情稳定时aPL抗体滴度则下降或转阴。在疾病的治疗过程中药物也影响部分病人的检查结果。这些因素可能造成APS漏诊,因此诊断标准还需在临床及基础方面不断深入的研究中逐步完善,应用时要根据临床具体情况决定。9 y9 ^; }% w; W) C$ j1 v1 ~9 t
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对APS,目前尚无令人满意的治疗方案,有些患者虽有抗体,但并无其他临床表现;有些患者在治疗产生抗磷脂抗体的原发病后,病情缓解处于稳定期,而血中aPL抗体仍持续阳性,因此对无临床症状的APS一般不需治疗。但综前所述,aPL抗体的出现大大增加了血栓形成、流产、死胎或血小板减少的危险性。因此,在临床上当出现上述3种情况时则应予以治疗。一般来讲,如果仅有轻度血小板减少,可不予治疗。对于严重的血小板减少,通常采用糖皮质激素和免疫抑制剂进行治疗,近来有人采用静脉注射免疫球蛋白来治疗血小板减少症。血栓形成的治疗可分为急性期治疗及预防再栓塞治疗两种治疗方案,动脉血栓形成与静脉血栓形成的治疗也不相同。由于并不是所有aPL抗体阳性的患者都会有血栓形成,而目前也不能预测哪些病人会有血栓形成,因此,不主张进行预防性治疗。如果一旦发生了血栓,急,t期治疗一般采用常规抗栓治疗,但应根据年龄、血栓发生部位及并存的其他疾病来调整每个患者的用药剂量。由于aPL抗体阳性并发血栓形成者大多为青年患者,血管通常是正常的,因此,可采用积极的溶栓治疗。对于急性期后的预防再栓塞治疗的时间长短一直存在争议,但多倾向于认为,只要 aPL抗体存在就需进行抗栓预防治疗。华法令治疗静脉血栓形成很有效,预防动脉血栓形成则抗血小板聚集药物如阿司匹林或潘生丁是理想的选择。单用阿司匹林对预防与aPL抗体相关的冠状动脉搭桥血管再阻塞十分有效。肝素、低分子肝素也是常用药物,免疫抑制剂仅用于经抗栓治疗未能防止血管阻塞的患者,但疗效尚待确定。
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1983年Labble和Walker报道了1例曾有流产史的妇女经泼尼松和阿司匹林联合治疗后妊娠成功的病例,此后其他学者也采用泼尼松和阿司匹林联合治疗使妊娠成功率明显提高。但Lockshin认为,泼尼松对流产治疗无效。有人认为,单用阿司匹林对与aPL抗体阳性伴流产的治疗有效。Gatenby等人发现,泼尼松联合阿司匹林治疗可以提高妊娠成功率,而单用泼尼松则无效。有两组病例报告表明,抗血小板治疗也有效。荷兰学者对37例有流产史的患者采用阿司匹林和潘生丁治疗后,胎儿成活率从18%升高到93 ,英国学者对42位妇女经单用阿司匹林治疗后,胎儿成活率从 18%提高到88%。因此,aPL抗体合并流产的治疗与aPL抗体相关的血栓形成的治疗相似,均可采用抗栓治疗。Rosove等人在妊娠全程中皮下注射肝素将胎儿成活率从1/28提高到13/14.由于糖皮质激素的治疗有一定副作用,有人采用静脉注射免疫球蛋白成功地防止了流产的发生。近来,Lockshin提议具有非其他原因的流产史,当伴有高滴度的IgG型aCL或LA时,是需进行治疗的肯定指征。
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2 |8 L, _( y6 }- F* o3 F 总结APS的特点为:① aPL抗体是一组值得进一步研究的抗体,随着研究的深人将对其产生的机制、致病机制及预防有更深刻的认识;OO aPL抗体在临床上主要与血栓、习惯性流产、血小板减少相关,但与aPL抗体相关的临床表现和所涉及的疾病范围均有扩大趋势;③ aPL抗体,尤其是LA和aCL抗体在SLE中存在时提示可能出现血栓、习惯性流产、血小板减少和神经精神病变,特别是抗体滴度高时更应予以重视;④对aPL抗体的治疗,现尚无肯定的方法,一般以治疗原发病为主,糖皮质激素、免疫抑制剂和抗凝治疗有一定作用。6 o- K* M- @/ d0 \4 ^
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