An estimated 2·5 million people worldwide have systemic sclerosis, and lung involvement is the leading cause of death in these patients. Approximately one quarter of patients develop pulmonary problems within the first 3 years of diagnosis and the survival of patients with systemic sclerosis-associated interstitial lung disease ( ) is reported to be between 29 and 69% at 10 years. The optimal treatment for SSc-ILD is not known but current management includes the use of immunosuppressants, most commonly mycophenolate and cyclophosphamide, alongside regular pulmonary function tests. The recommended the use of mycophenolate over cyclophosphamide because it was better tolerated and associated with less toxicity despite both drugs having similar efficacy. Dr Donald Tashkin from the David Geffen School of Medicine at University of California Los Angeles (CA, USA) told The Lancet Respiratory Medicine, “Mycophenolate administered over 24 months to patients with SSc-ILD has been shown to result in an absolute, albeit relatively modest, improvement in forced vital capacity (FVC), in addition to significantly improving some health-related quality of life measures, such as dyspnoea (Transition Dyspnoea Index) and cough.”

中文翻译：

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Nintedanib用于系统性硬化相关的间质性肺疾病。

全世界估计有2·500万人患有系统性硬化症，肺部受累是这些患者死亡的主要原因。大约四分之一的患者在诊断的头三年内出现肺部问题，据报道，系统性硬化症相关性间质性肺疾病（）的患者10年生存率在29％至69％之间。目前尚不清楚SSc-ILD的最佳治疗方法，但目前的治疗方法包括使用免疫抑制剂，最常见的是霉酚酸酯和环磷酰胺，以及常规的肺功能检查。推荐使用霉酚酸酯而不是环磷酰胺，因为尽管两种药物具有相似的功效，但其耐受性更好且毒性较低。《柳叶刀呼吸医学》（“ Lancet Respiratory Medicine”）显示，在24个月内向SSc-ILD患者服用麦考酚酸酯，尽管可以显着改善某些与健康相关的质量，但仍可绝对改善强迫肺活量（FVC），尽管相对适度。生活指标，例如呼吸困难（过渡呼吸困难指数）和咳嗽。”