Table 4.
Consensus approach to the categorization of definitive or probable SJIA
| 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. |
+++: obligatory criterion; +: minor criterion; X: exclusion criterion
a fever of at least two weeks’ duration that is documented to be daily (“quotidian”) for at least three days
b marked elevation of C-reactive protein, erythrocyte sedimentation rate, leukocytes/granulocytes and/or ferritin
- not specifically addressed in the definition
SJIA Systemic juvenile idiopathic arthritis