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Abstract

CLC Number:

R593.2

Figures/Tables1

	建议综合管理	RG
联合疗法	抗MDA5抗体阳性DM(或CADM)合并RPILD患者应首选联合疗法	D
1	糖皮质激素（激素）加钙调磷酸酶抑制剂（环孢素A或他克莫司）的联合疗法,或在此基础上加用静脉滴注环磷酰胺^a) 的三联疗法,均被认为是较好的初始选择	D
	a环孢素A和他克莫司疗效均好,临床选择基于其药物安全性及患者特征	√
	b建议监测钙调磷酸酶抑制剂的血药浓度以调整剂量,减少毒副作用	√
2	当钙调磷酸酶抑制剂不可行时,应考虑糖皮质激素联合其他免疫抑制剂[如环磷酰胺^b)和（或）吗替麦考酚酯],或将利妥昔单抗^b)加到任一先前的治疗方案中	D
	a这些药物的选择基于患者的个体特征及医生的临床经验	√
难治性DM(或CADM)合并RPILD患者的治疗
1	对激素联合免疫抑制剂治疗无效的患者,应考虑以下替代方案
	a在当前治疗方案中加用以下任一免疫抑制剂：环磷酰胺、吗替麦考酚酯、利妥昔单抗、巴利昔单抗或托法替布	D
	b换用另一种免疫抑制剂	√
2	对激素联合免疫抑制剂治疗无反应的患者,可单独或依次采用以下替代疗法
	a多黏菌素B血液灌流	D
	b 血浆置换	D
	c 静脉注射免疫球蛋白	√
3	对出现危及生命的严重和（或）难治性呼吸功能不全的患者,应考虑使用ECMO来维持患者的生命,等待患者对强化联合免疫抑制疗法的临床反应或作为肺移植前的桥梁阶段	√
4	对难治性患者应考虑肺移植,在诊断RPILD后应尽早转诊,进行移植资格评估	√
其他治疗选择
1	不建议将硫唑嘌呤、氨甲蝶呤和来氟米特用于治疗此类患者	√
2	不建议在治疗中使用英夫利昔单抗	√
3	尽管在肺纤维化的数据中,吡非尼酮已经被添加到CADM相关的亚急性ILD的常规免疫抑制治疗中,但专家组可能并不建议	√

References34

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