Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease.

Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease.
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DOI:
10.3899/jrheum.121043
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发表时间:
2013-05
期刊:
The Journal of rheumatology
影响因子:
--
通讯作者:
Swigris JJ
Swigris JJ
中科院分区:
其他
文献类型:
--
作者:
Fischer A;Brown KK;Du Bois RM;Frankel SK;Cosgrove GP;Fernandez-Perez ER;Huie TJ;Krishnamoorthy M;Meehan RT;Olson AL;Solomon JJ;Swigris JJ

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小系列研究表明霉酚酸酯(MMF)耐受性良好,可能是治疗结缔组织病相关间质性肺疾病(CTD-ILD)的有效方法。我们研究了接受MMF治疗的CTD-ILD患者的耐受性和肺生理学的纵向变化。我们确定了在2008年1月至2011年1月期间在我们中心进行评估的连续患者,并为CTD-ILD开出了MMF。我们评估了MMF的安全性和耐受性,并使用纵向数据分析来检查MMF开始之前和之后随时间的肺生理学变化。我们确定了125名接受MMF治疗的受试者,平均治疗时间为897天。13名受试者停用MMF。预测用力肺活量(FVC%)的估计百分比(FVC%)从MMF开始到52周、104周和156周(分别为4.9%±1.9%,p=0.0008;6.1%±1.8%,p=0.0008;和7.3%±2.6%,p=0.004)以及预计弥散量(DLCO%)从MMF开始到52周和104周(6.3%±2.8%,p=0.02;7.1%±2.8%,p=0.01)。在无普通型间质性肺炎(UIP)型损伤的亚组中,MMF显著改善FVC%和DLCO%,而在UIP型损伤亚组中,MMF与FVC%和DLCO%的稳定性有关。在CTD-ILD的大量不同队列中,MMF耐受性良好,停用率低。在平均2.5年的随访期内,使用MMF的治疗与稳定或改善肺生理学有关。MMF似乎是治疗CTD-ILD谱系疾病的一种有前途的治疗方法。
Small series suggest mycophenolate mofetil (MMF) is well tolerated and may be an effective therapy for connective tissue disease-associated interstitial lung disease (CTD-ILD). We examined the tolerability and longitudinal changes in pulmonary physiology in a large and diverse cohort of patients with CTD-ILD treated with MMF. We identified consecutive patients evaluated at our center between January 2008 and January 2011 and prescribed MMF for CTD-ILD. We assessed safety and tolerability of MMF and used longitudinal data analyses to examine changes in pulmonary physiology over time, before and after initiation of MMF. We identified 125 subjects treated with MMF for a median 897 days. MMF was discontinued in 13 subjects. MMF was associated with significant improvements in estimated percentage of predicted forced vital capacity (FVC%) from MMF initiation to 52, 104, and 156 weeks (4.9% ± 1.9%, p = 0.01; 6.1% ± 1.8%, p = 0.0008; and 7.3% ± 2.6%, p = 0.004, respectively); and in estimated percentage predicted diffusing capacity (DLCO%) from MMF initiation to 52 and 104 weeks (6.3% ± 2.8%, p = 0.02; 7.1% ± 2.8%, p = 0.01). In the subgroup without usual interstitial pneumonia (UIP)-pattern injury, MMF significantly improved FVC% and DLCO%, and in the subgroup with UIP-pattern injury, MMF was associated with stability in FVC% and DLCO%. In a large diverse cohort of CTD-ILD, MMF was well tolerated and had a low rate of discontinuation. Treatment with MMF was associated with either stable or improved pulmonary physiology over a median 2.5 years of followup. MMF appears to be a promising therapy for the spectrum of CTD-ILD.
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