Ann Rheum Dis:系统性硬化症患者的进展性间质性肺病

2020-10-15 xiangting MedSci原创

SSc-ILD存在异质性,并且病程多变,因此对所有患者进行密切随访非常重要。在FVC下降之前就开始治疗的新观念可以避免不可逆的器官损害。

系统性硬化症(SSc)是以局限性或弥漫性皮肤变硬、增厚,同时可伴有肺脏、心脏、肾脏、消化道等内脏器官损伤为特征的少见结缔组织疾病,肺部表现为间质性肺病(ILD),ILD是SSc患者死亡的主要原因,SSc-ILD预后差,死亡率高。这项研究使用欧洲硬皮病试验与研究(EUSTAR)数据库中的数据,确定了SSc-ILD患者出现进展性间质性肺病(ILD)的总体病程、进展模式和危险因素。

 

EUSTAR数据库对符合条件的SSc-ILD患者进行了登记,在基线和12±3个月后测量用力肺活量(FVC)。对多次测量FVC的患者评估长期进展性ILD和进展模式。使用多因素混合效应模型分析ILD进展的潜在预测因素。

共纳入826例SSc-ILD患者。在12±3个月内,219例患者(27%)表现为进展性ILD:FVC中度下降(降低5%-10%)或显著下降(降低>10%)。平均5年的随访期内,共有535例患者(65%)进行了多次FVC测量。每12个月的周期内有23%-27%的SSc-ILD患者表现为进展性ILD,但只有少数患者呈连续性进展。大多数进展性ILD患者(58%)肺功能下降缓慢,其稳定/改善时间多于下降,而只有8%的患者FVC持续快速下降。178例患者(33%)FVC没有下降。5年内FVC下降的最强预测因素是男性、改良Rodnan皮肤评分高和反流/吞咽困难症状。

SSc-ILD存在异质性,并且病程多变,因此对所有患者进行密切随访非常重要。在FVC下降之前就开始治疗的新观念可以避免不可逆的器官损害。

原始出处:

Anna-Maria Hoffmann-Vold. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis. October 2020.

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    2021-01-05 zhaojie88
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    2021-01-03 14700befm06暂无昵称

    学习了

    0

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    2021-03-22 amy0559
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