Suliman Sally, Al Harash Abdalhamid, Roberts William Neil, Perez Rafael L, Roman Jesse
Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Louisville Health Sciences Center, Louisville, KY, USA.
Division of Rheumatology, University of Louisville Health Sciences Center, Louisville, KY, USA.
Respir Med Case Rep. 2017 Jul 15;22:109-112. doi: 10.1016/j.rmcr.2017.07.007. eCollection 2017.
Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. Although certain immunosuppressive therapies may slow disease progression, current treatment strategies are not curative; consequently, SSc-ILD continues to be a major cause of morbidity and mortality. We present four cases of SSc-ILD that emphasize the importance of early screening and detection, close follow-up, and aggressive management. We also highlight the need for well-conducted clinical trials designed to identify new and effective treatments.
硬皮病相关间质性肺病（SSc-ILD）是一种以系统性炎症和肺部进行性瘢痕形成为特征的肺纤维化疾病，可导致呼吸衰竭。尽管某些免疫抑制疗法可能会减缓疾病进展，但目前的治疗策略无法治愈该疾病；因此，SSc-ILD仍然是发病和死亡的主要原因。我们介绍了4例SSc-ILD病例，强调了早期筛查与检测、密切随访以及积极管理的重要性。我们还强调了开展精心设计的临床试验以确定新的有效治疗方法的必要性。
