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霉酚酸酯治疗硬皮病肺病患者的两年经验:病例系列

Two-year experience with mycophenolate mofetil in patients with scleroderma lung disease: a case series.

作者信息

Yilmaz Neslihan, Can Meryem, Kocakaya Derya, Karakurt Sait, Yavuz Sule

机构信息

Rheumatology Department, Faculty of Medicine, Marmara University, Istanbul, Turkey; Rheumatology Department, Faculty of Medicine, Istanbul Science University, Istanbul, Turkey.

出版信息

Int J Rheum Dis. 2014 Nov;17(8):923-8. doi: 10.1111/1756-185X.12399. Epub 2014 May 26.

DOI:10.1111/1756-185X.12399
PMID:24864029
Abstract

To assess the effect of mycophenolate mofetil (MMF) on pulmonary functions in patients with systemic sclerosis-associated lung disease (SSc-ILD) who experienced an inadequate response to first line cyclophosphamide (CYC) therapy. Twelve consecutive SSc-ILD patients who received MMF due to inadequate response to CYC as a first line agent, were retrospectively reviewed. Over the course of 2 years, pulmonary function tests (PFT) and high-resolution computed tomography (HRCT) scans were performed. Following initial baseline tests, PFTs were continued at a frequency of every 6 months and HRCT scans were performed every 12 months. After MMF treatment, values of forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) improved in three (25%) and two (16.6%) patients, respectively. It is also noted that the evaluation of serial HCRT scans showed no change in 54.5% of patients. Our case series suggested that PFT and imaging scores seemed to be stabilized by MMF in SSc-ILD patients who were inadequate responders to CYC.

摘要

评估霉酚酸酯(MMF)对一线环磷酰胺(CYC)治疗反应不佳的系统性硬化症相关肺病(SSc-ILD)患者肺功能的影响。回顾性分析了12例因对CYC作为一线药物反应不佳而接受MMF治疗的连续性SSc-ILD患者。在2年的时间里,进行了肺功能测试(PFT)和高分辨率计算机断层扫描(HRCT)。在初始基线测试后,PFT每6个月进行一次,HRCT扫描每12个月进行一次。MMF治疗后,分别有3例(25%)和2例(16.6%)患者的用力肺活量(FVC)和一氧化碳弥散量(DLCO)值有所改善。还注意到,连续HCRT扫描评估显示54.5%的患者没有变化。我们的病例系列表明,MMF似乎能使对CYC反应不佳的SSc-ILD患者的PFT和影像学评分稳定。

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