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. 2021 Jan 18;14(4):484–494. doi: 10.1016/j.jiph.2021.01.008

Table A1.

RCPCH, U.S. CDC, and WHO case definitions of PIMS-TS and MIS-C.

RCPCH case definitiona U.S. CDC case definitionb WHO case definitionc
  • 1

    A child presenting with persistent fever (>38.5 °C), inflammation (neutrophilia, elevated CRP and lymphopenia) and evidence of single or multi-organ dysfunction (shock, cardiac, respiratory, renal, gastrointestinal or neurological disorder) with additional features (see below). This may include children fulfilling full or partial criteria for Kawasaki disease.

  • 1

    Age <21 years

  • 1

    Age 0-19 years

  • 2

    Exclusion of any other microbial cause, including bacterial sepsis, staphylococcal or streptococcal shock syndromes, infections associated with myocarditis such as enterovirus (waiting for results of these investigations should not delay seeking expert advice).

  • 2

    Clinical presentation including the following:

  • 2

    Fever for ≥3 days

  • 3

    SARS-CoV-2 PCR testing may be positive or negative

  • Fever >38.0 °C for ≥24 hours, or report

    • a
      At least 2 of the following clinical features:
Clinical
  • of subjective fever lasting ≥24

      • i
        Rash or bilateral non-purulent conjunctivitis or mucocutaneous inflammation signs
  • All: persistent fever

  • Laboratory evidence of inflammation including, but not limited to, one or more of the following:

      • i
        Hypotension or shock
  • Most: oxygen requirement, hypotension

      • Elevated CRP
        • 1
          Features of myocardial dysfunction, pericarditis, valvulitis, or coronary abnormalities
  • Some: Abdominal pain, confusion, conjunctivitis, cough, diarrhea, headache, lymphadenopathy, mucus membrane changes, neck swelling, rash, respiratory symptoms, sore throat, swollen hands and feet, syncope, vomiting

      • Elevated ESR
        • 1
          Evidence of coagulopathy
Laboratory
      • Elevated fibrinogen
        • 1
          Acute gastrointestinal problems
  • All: abnormal fibrinogen, absence of potential causative organisms (other than SARS-CoV-2), high CRP, high D-dimers, high ferritin, hypoalbuminemia, lymphopenia, neutrophilia in most

      • Elevated procalcitonin
      • i
        Elevated markers of inflammation such as ESR, CRP, or procalcitonin
  • Some: acute kidney injury, anemia, coagulopathy, high IL-10 if available, high IL-6 if available, neutrophilia, proteinuria, raised CK, raised LDH, raised triglycerides, raised troponin, thrombocytopenia, transaminitis

      • Elevated D-dimer
      • i
        No other obvious microbial cause of inflammation, including bacterial sepsis, staphylococcal or streptococcal shock syndromes
Imaging and ECG
      • Elevated ferritin
      • i
        Evidence of COVID-19 (RT-PCR, antigen test or serology positive), or likely contact with patients with COVID-19
  • Echo and ECG: myocarditis, valvulitis, pericardial effusion, coronary artery dilation

      • Elevated LDH
  • CXR: patchy symmetrical infiltrates, pleural effusion;

      • Elevated IL-6
  • Abdominal ultrasound: colitis, ileitis, lymphadenopathy, ascites, hepatosplenomegaly

      • Neutrophilia
  • Chest CT: may demonstrate coronary artery abnormalities

      • Lymphocytopenia
      • Hypoalbuminemia
  • Evidence of clinically severe illness requiring hospitalization, with multisystem (≥2) organ involvement (cardiac, renal respiratory, hematologic, gastrointestinal, dermatologic, or neurological)

  • 3

    No alternative plausible diagnoses

  • 4

    Positive for current or recent SARS-CoV-2 infection by RT-PCR, serology, or antigen test; or COVID-19 exposure within the 4 weeks prior to the onset of symptoms

RCPCH, Royal College of Paediatrics and Child Health; CDC, Centers for Disease Control and Prevention; WHO, World Health Organization; CRP, C-reactive protein; CK, creatinine kinase; ESR, erythrocyte sedimentation rate; LDH, lactate dehydrogenase; IL, interleukin; SARS-CoV-2, severe acute respiratory syndrome coronavirus 2; RT-PCR, reverse transcription polymerase chain reaction; COVID-19, Coronavirus disease 2019; ECG, electrocardiogram; CXR, chest x-ray; CT, computerized tomography.