Table 2 Currently available diagnostic/classification criteria for SD

Major criteria

• Arthritis in ≥ 1 joint with or preceded by fever ≥ 2 weeks (documented to be daily for ≥ 3 days), with ≥ 1 day of:

 ◦ Evanescent (non-fixed) erythematous rash

 ◦ General lymph node enlargement

 ◦ Hepatomegaly and/or splenomegaly

 ◦ Serositis

• Onset before age 18 years

• Fever for ≥ 3 consecutive days, reoccurring over 2 weeks

• Evanescent (non-fixed) erythematous rash

• Arthritis

• Fever ≥ 39 °C lasting ≥ 1 week

• Arthralgia or arthritis lasting ≥ 2 weeks

• Typical rash

• Leukocytosis ≥ 10,000/µL with ≥ 80% neutrophils

• Spiking fever ≥ 39 °C

• Arthralgia

• Transient erythema

• ≥ 80% granulocytes

• Pharyngitis

• Glycosylated ferritin < 20%

Minor criteria

–

• Generalized lymph node enlargement and/or hepatomegaly and/or splenomegaly

• Serositis

• Arthralgia lasting ≥ 2 weeks (in the absence of arthritis

• Leukocytosis (≥ 15,000/mm³) with neutrophilia

• Sore throat

• Lymphadenopathy

• Hepatomegaly or splenomegaly

• Abnormal liver function tests

• Negative RF and ANA

• Maculopapular rash

• Leucocytes ≥ 10,000/µL

Exclusion criteria

• All other forms of JIA must be excluded

• Other known conditions

• Infections, malignancies, and all other conditions that may mimic AOSD

–

Additional criteria

–

• Fever (as described) accompanied by either the 2 other major criteria, or 1 of the major criterion and 2 minor criteria

• The presence of 5 criteria listed above, including ≥ 2 major criteria

• The presence of 4 major criteria listed above, or 3 major and 2 minor criteria