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<p>Kawasaki disease is a&#xa0;vasculitis of the medium-sized vessels. Initially, it presents with a&#xa0;strong systemic inflammatory response, which subsides within a&#xa0;few weeks with or without treatment. The differential diagnosis, particularly distinguishing it from other infectious and autoinflammatory diseases, can be challenging even for experienced clinicians. In most children the disease has no long-term consequences; however, up to 25% develop coronary artery aneurysms (CAA). Adequate and early treatment can reduce the risk of CAA to ca. 5%. Infants, especially those under 6&#xa0;months of age, have a&#xa0;significantly higher risk, with CAA rates up to 60%.</p><p>Increased incidences of CAA are also observed in patients with an initial nonresponse to intravenous immunoglobulins (IVIG) and early detection of CAA. Other risk factors are still being debated.</p><p>These patients should initially receive more intensive treatment besides standard IVIG therapy. The choice of additional medications is also a&#xa0;subject of debate.</p><p>Larger CAA increase the risk of coronary thrombosis, stenosis and subsequent myocardial infarction, leading to significantly increased long-term morbidity and mortality. Particularly in cases of giant CAA (z-score &gt; 10), coronary artery occlusion can occur even in the acute phase. In contrast, patients without CAA have a&#xa0;life expectancy comparable to the general population.</p><p>The identification of specific biomarkers for the early diagnosis of Kawasaki disease, the detection of high-risk patients and monitoring disease progression and treatment success remains a&#xa0;central clinical and scientific research goal. There is also a&#xa0;significant need for clearer evidence to guide the selection of optimal patient-specific treatment.</p>

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Das Kawasaki-Syndrom

  • 期刊论文

  • 发表日期：

    2026年5月7日

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  • Toni Hospach,
  • André Jakob

摘要

Kawasaki disease is a vasculitis of the medium-sized vessels. Initially, it presents with a strong systemic inflammatory response, which subsides within a few weeks with or without treatment. The differential diagnosis, particularly distinguishing it from other infectious and autoinflammatory diseases, can be challenging even for experienced clinicians. In most children the disease has no long-term consequences; however, up to 25% develop coronary artery aneurysms (CAA). Adequate and early treatment can reduce the risk of CAA to ca. 5%. Infants, especially those under 6 months of age, have a significantly higher risk, with CAA rates up to 60%.

Increased incidences of CAA are also observed in patients with an initial nonresponse to intravenous immunoglobulins (IVIG) and early detection of CAA. Other risk factors are still being debated.

These patients should initially receive more intensive treatment besides standard IVIG therapy. The choice of additional medications is also a subject of debate.

Larger CAA increase the risk of coronary thrombosis, stenosis and subsequent myocardial infarction, leading to significantly increased long-term morbidity and mortality. Particularly in cases of giant CAA (z-score > 10), coronary artery occlusion can occur even in the acute phase. In contrast, patients without CAA have a life expectancy comparable to the general population.

The identification of specific biomarkers for the early diagnosis of Kawasaki disease, the detection of high-risk patients and monitoring disease progression and treatment success remains a central clinical and scientific research goal. There is also a significant need for clearer evidence to guide the selection of optimal patient-specific treatment.