Scholarlink

<p>Systemic sclerosis (scleroderma, SSc) is a&#xa0;rare autoimmune disease characterized by vasculopathy and fibrosis of internal organs. Interstitial lung disease associated with SSc (SSc-ILD) occurs in up to 30% of all patients and is a&#xa0;key determinant of mortality. Treatment options include both immunosuppressive and antifibrotic medications. The updated guidelines from the European Alliance of Associations for Rheumatology (EULAR) provide, among others, specific recommendations for the management of SSc-ILD. The medications with the strongest evidence are mycophenolate mofetil, cyclophosphamide and rituximab but also to a&#xa0;lesser extent, tocilizumab. All these therapies are used off-label for SSc-ILD. In Germany, nintedanib is the only approved antifibrotic drug. A&#xa0;large German registry study has shown that the use of proton pump inhibitors provides a&#xa0;clear survival benefit in SSc-ILD. New therapies are currently under clinical investigation ranging from monoclonal antibodies to more advanced approaches, such as CAR T‑cell therapy, in refractory or rapidly progressive cases.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Sklerodermie-assoziierte interstitielle Lungenerkrankung

  • 期刊论文

  • 发表日期：

    2025年6月12日

查看全文
  • Oliver Wiemann,
  • Peter Korsten

摘要

Systemic sclerosis (scleroderma, SSc) is a rare autoimmune disease characterized by vasculopathy and fibrosis of internal organs. Interstitial lung disease associated with SSc (SSc-ILD) occurs in up to 30% of all patients and is a key determinant of mortality. Treatment options include both immunosuppressive and antifibrotic medications. The updated guidelines from the European Alliance of Associations for Rheumatology (EULAR) provide, among others, specific recommendations for the management of SSc-ILD. The medications with the strongest evidence are mycophenolate mofetil, cyclophosphamide and rituximab but also to a lesser extent, tocilizumab. All these therapies are used off-label for SSc-ILD. In Germany, nintedanib is the only approved antifibrotic drug. A large German registry study has shown that the use of proton pump inhibitors provides a clear survival benefit in SSc-ILD. New therapies are currently under clinical investigation ranging from monoclonal antibodies to more advanced approaches, such as CAR T‑cell therapy, in refractory or rapidly progressive cases.