Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Pb 4950 Nydalen, 0424, Oslo, Norway.
Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
Respir Res. 2020 Jul 23;21(1):197. doi: 10.1186/s12931-020-01459-0.
Systemic sclerosis (SSc) is a multi-organ autoimmune disease with complex interactions between immune-mediated inflammatory processes and vascular pathology leading to small vessel obliteration, promoting uncontrolled fibrosis of skin and internal organs. Interstitial lung disease (ILD) is a common but highly variable manifestation of SSc and is associated with high morbidity and mortality. Treatment approaches have focused on immunosuppressive therapies, which have shown some efficacy on lung function. Recently, a large phase 3 trial showed that treatment with nintedanib was associated with a reduction in lung function decline. None of the conducted randomized clinical trials have so far shown convincing efficacy on other outcome measures including quality of life determined by patient reported outcomes. Little evidence is available for non-pharmacological treatment and supportive care specifically for SSc-ILD patients, including pulmonary rehabilitation, supplemental oxygen, symptom relief and adequate information. Improved management of SSc-ILD patients based on a holistic approach is necessary to support patients in maintaining as much quality of life as possible throughout the disease course and to improve long-term outcomes.
系统性硬化症（SSc）是一种多器官自身免疫性疾病，免疫介导的炎症过程和血管病理学之间存在复杂的相互作用，导致小血管闭塞，促进皮肤和内脏器官的不受控制的纤维化。间质性肺疾病（ILD）是 SSc 的一种常见但高度可变的表现，与高发病率和死亡率相关。治疗方法侧重于免疫抑制疗法，这些疗法在肺功能方面显示出了一定的疗效。最近，一项大型 3 期试验表明，尼达尼布治疗与肺功能下降减少相关。迄今为止，进行的随机临床试验都没有在其他预后指标（包括由患者报告的结局确定的生活质量）上显示出令人信服的疗效。对于 SSc-ILD 患者的非药物治疗和支持性护理，包括肺康复、补充氧气、缓解症状和提供充足信息，几乎没有证据。需要基于整体方法来改善 SSc-ILD 患者的管理，以支持患者在整个疾病过程中尽可能保持生活质量，并改善长期预后。
