Janardana Ramya, Irodi Aparna, Chebbi Pramod P, Goel Ruchika, Vimala Leena R, Padiyar Shivraj, Peediyakal Anoof, Mathew John, Nair Aswin, Christopher Devasahayam J, Danda Debashish
Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, India.
Clinical Immunology & Rheumatology, St. John's Medical College, Bengaluru, India.
J Scleroderma Relat Disord. 2021 Oct;6(3):271-276. doi: 10.1177/23971983211024410. Epub 2021 Jun 30.
There is a paucity of real-world data on mycophenolate mofetil/mycophenolate sodium in systemic sclerosis-related interstitial lung disease.
To study the efficacy of mycophenolate mofetil/ mycophenolate sodium in systemic sclerosis-related interstitial lung disease.
In this single-centre study, clinical, laboratory and imaging details of consecutive patients with systemic sclerosis-related interstitial lung disease receiving mycophenolate mofetil/mycophenolate sodium from rheumatology and pulmonology clinics between January 2008 and March 2017 were retrospectively retrieved. The change in percentage of predicted normal forced vital capacity at last follow-up visit as compared with baseline was studied. In addition, high-resolution computed tomography scans at baseline and 2-year follow-up visit were scored as either stable/improved or worsened by experienced thoracic radiologists blinded to the clinical details of patients.
Altogether, 88 patients (85.2% females) with mean age (SD) of 33.8 years (± 11.3) and median (interquartile range) duration of disease since non-Raynaud's symptoms of 36 months (13.5-60) were studied. Diffuse systemic sclerosis comprised 85.2% of them. The mean baseline forced vital capacity was 61.2 ± 17.9% and median scores for ground glass opacities and fibrosis in high-resolution computed tomography were 0.5 (0-1.3) and 1 (0-1.3), respectively. At a median follow-up duration of 30 months (interquartile range = 16.5-49), the percentage of forced vital capacity improved by 1.8% (-3.82 to 9.07) as compared with baseline visit ( = 0.02). In the 2-year follow-up, the ground glass opacity and fibrosis scores in high-resolution computed tomography improved in 17.3% and 7.7% of patients and stabilized in 63.5% and 78.8% patients, respectively.
Mycophenolate mofetil/mycophenolate sodium was efficacious in improving /stabilizing forced vital capacity irrespective of the baseline high-resolution computed tomography lung scores in our patients with systemic sclerosis-related interstitial lung disease during the ⩾ 2-year follow-up period.
关于霉酚酸酯/麦考酚钠在系统性硬化症相关间质性肺病中的真实世界数据较少。
研究霉酚酸酯/麦考酚钠在系统性硬化症相关间质性肺病中的疗效。
在这项单中心研究中，回顾性检索了2008年1月至2017年3月期间从风湿病科和肺病科诊所接受霉酚酸酯/麦考酚钠治疗的系统性硬化症相关间质性肺病连续患者的临床、实验室和影像学详细信息。研究了最后一次随访时预测正常用力肺活量百分比与基线相比的变化。此外，由对患者临床细节不知情的经验丰富的胸放射科医生对基线和2年随访时的高分辨率计算机断层扫描进行评分，分为稳定/改善或恶化。
共研究了88例患者（85 .2%为女性），平均年龄（标准差）为33.8岁（±11.3），自非雷诺症状出现以来的疾病持续时间中位数（四分位间距）为36个月（13.5 - 60）。其中弥漫性系统性硬化症占85.2%。平均基线用力肺活量为61.2±17.9%，高分辨率计算机断层扫描中磨玻璃影和纤维化的中位数评分分别为0.5（0 - 1.3）和1（0 - 1.3）。在中位随访期30个月（四分位间距=16.5 - 49）时，用力肺活量百分比与基线访视相比提高了1.8%（-3.82至9.07）（P = 0.02）。在2年随访中，高分辨率计算机断层扫描中的磨玻璃影和纤维化评分分别在17.3%和7.7%的患者中得到改善，在63.5%和78.8%的患者中保持稳定。
在我们患有系统性硬化症相关间质性肺病的患者中，在至少2年的随访期内，霉酚酸酯/麦考酚钠无论基线高分辨率计算机断层扫描肺部评分如何，在改善/稳定用力肺活量方面均有效。
