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Multisystem inflammatory syndrome in children and adults

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Disease definition

A rare systemic disease characterized by a life-threatening hyperinflammatory state several weeks after infection with SARS-CoV-2, predominantly occurring in children. The primary infection is typically mild or asymptomatic, and patients are generally previously healthy individuals. Typical presenting signs and symptoms are persistent fever, gastrointestinal symptoms, mucocutaneous inflammation, lymphopenia, and high levels of circulating inflammatory markers. Some patients develop severe disease with cardiac involvement, hypotension, and shock. Presentation is similar in adults, although the severity of cardiac dysfunction, incidence of thrombosis, and mortality may be higher.

ORPHA:598363

Classification level: Disorder

Synonym(s):
  • MIS-C/A

Source: PubMed ID 33838966 32838310 33640145

Prevalence: Unknown

Inheritance: -

Age of onset: Childhood

ICD-10: U10.9

ICD-11: RA03

UMLS: C5680268

A summary on this disease is available in Français, Español, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2021.pdf) - Orphanet Urgences
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