Scholarlink

<p>Still’s disease is a&#xa0;rare but potentially life-threatening autoinflammatory systemic disease primarily characterized by pronounced systemic inflammation with intermittent high fever, a&#xa0;characteristic rash and multiorgan involvement. Manifest arthritis is not mandatory at disease onset and can develop later or be absent. Historically, age-based classifications separated systemic juvenile idiopathic arthritis (sJIA) from adult-onset Still’s disease (AOSD), despite largely overlapping clinical, immunological and therapeutic features. Current EULAR/PReS recommendations summarize both entities under the term Still’s disease and propose a&#xa0;clinically oriented case definition. The pathophysiology is dominated by dysregulated activation of the innate immune system as well as the interleukin (IL)-1, IL‑6 and IL-18 pathways. Biomarkers such as IL-18 and S100 proteins support the diagnostics, risk stratification and disease monitoring. An early implementation of a&#xa0;structured treat-to-target strategy with temporally defined interim goals and prompt cytokine blockade improves the prognosis and can prevent chronic disease progression.</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

Still-Syndrom

  • 期刊论文

  • 发表日期：

    2026年4月17日

查看全文
  • Claas H. Hinze,
  • Christoph Kessel,
  • Dirk Föll

摘要

Still’s disease is a rare but potentially life-threatening autoinflammatory systemic disease primarily characterized by pronounced systemic inflammation with intermittent high fever, a characteristic rash and multiorgan involvement. Manifest arthritis is not mandatory at disease onset and can develop later or be absent. Historically, age-based classifications separated systemic juvenile idiopathic arthritis (sJIA) from adult-onset Still’s disease (AOSD), despite largely overlapping clinical, immunological and therapeutic features. Current EULAR/PReS recommendations summarize both entities under the term Still’s disease and propose a clinically oriented case definition. The pathophysiology is dominated by dysregulated activation of the innate immune system as well as the interleukin (IL)-1, IL‑6 and IL-18 pathways. Biomarkers such as IL-18 and S100 proteins support the diagnostics, risk stratification and disease monitoring. An early implementation of a structured treat-to-target strategy with temporally defined interim goals and prompt cytokine blockade improves the prognosis and can prevent chronic disease progression.