Definitive SJIA | Probable SJIA | |
---|---|---|
Inclusion criteria | ||
Typical fever patterna | +++ | +++ |
Arthritis in at least one joint | +++ | – |
Typical (evanescent erythematous) rash | + | + |
Generalized lymphadenopathy | + | + |
Hepatomegaly or splenomegaly | + | + |
Serositis | + | + |
Marked systemic inflammationb | – | +++ |
Extremely elevated S100 proteins (calgranulins) | – | + |
Exclusion criteria | ||
Infection | X | X |
Malignancy | X | X |
Hereditary autoinflammatory syndrome | X | X |
Requirements | All obligatory criteria are fulfilled and at least one minor criterion. | All obligatory and at least two minor criteria are fulfilled. |