Azathioprine for Connective Tissue Disease-Associated Interstitial Lung Disease
Azathioprine for Connective Tissue Disease-Associated Interstitial Lung Disease
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DOI：
10.1159/000508540
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发表时间：
2020-09-01
期刊：
影响因子：
3.7
通讯作者：
Bonella, Francesco
中科院分区：
文献类型：
--
作者：
Boerner, Eda B.;Cuyas, Marta;Bonella, Francesco
背景：免疫抑制治疗仍然是结缔组织病相关性间质性肺疾病（CTD-ILD）患者的标准治疗方法。Objectives：本回顾性研究旨在提供硫唑嘌呤治疗进展性CTD-ILD的耐受性和疗效数据。结果：平均治疗时间为34个月，范围为3-105个月。15名患者（27%）在前3个月内因副作用而停止治疗，主要是由于肝酶升高。41例患者治疗时间超过3个月，其中27例（66%）在治疗期间肺功能稳定或改善。在保持稳定或改善的患者中，治疗开始时的平均FVC为62 +/- 17%预测值（% pred），末次随访访视时为65 +/- 17%预测值（p= 0.036），治疗开始时平均DLCO为38 +/-16%pred，末次随访时为39 +/-17%pred（p= 0.06）。结论：硫唑嘌呤可稳定或改善CTD-ILD。虽然早期药物不耐受是常见的，但大多数对药物耐受良好的患者实现了肺功能的长期稳定或改善。
Background:Immunosuppressive therapy still is the standard treatment for patients with connective tissue disease-associated interstitial lung disease (CTD-ILD).Objectives:This retrospective study aimed to provide data on the tolerability and efficacy of azathioprine in progressive CTD-ILDs.Methods:A total of 56 patients with CTD-ILD treated with azathioprine between 2003 and 2014 were included in the study. The patients were assessed every 3 months during follow-up.Results:The mean treatment duration was 34 months, with a range of 3-105 months. Fifteen patients (27%) discontinued treatment due to side effects, mostly due to elevated liver enzymes, within the first 3 months. Forty-one patients were treated for longer than 3 months, and 27 of those (66%) had stabilization or improvement of pulmonary function during treatment. In patients who remained stable or improved, the mean FVC was 62 +/- 17% predicted (% pred) at initiation of treatment and 65 +/- 17% pred at the last follow-up visit (p= 0.036), and the mean DLCO was 38 +/- 16% pred at initiation of treatment and 39 +/- 17% pred at the last follow-up visit (p= 0.06).Conclusions:Azathioprine can stabilize or improve CTD-ILD. While early drug intolerance is frequent, most patients who have tolerated the drug well achieve long-term stabilization or improvement of lung function.