Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany

Table 4 Consensus approach to the categorization of definitive or probable SJIA

	Definitive SJIA	Probable SJIA
Inclusion criteria
Typical fever pattern^a	+++	+++
Arthritis in at least one joint	+++	–
Typical (evanescent erythematous) rash	+	+
Generalized lymphadenopathy	+	+
Hepatomegaly or splenomegaly	+	+
Serositis	+	+
Marked systemic inflammation^b	–	+++
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

ISSN: 1546-0096