Table 1. Selected... | Rockefeller University Press

Table 1.

Selected diagnostic/classification criteria for diagnosis of AOSD

Yamaguchi (12) (classification—1992)	Fautrel (13) (classification—2002)	Cush (2) (diagnostic—1987)	EULAR/PReS (4) (expert consensus diagnostic recommendations)
≥5 criteria (≥2 major)	≥4 major or 3 major + 2 minor	AOSD diagnosis by Yamaguchi or Fautrel plus ≥1 ICU-level organ failure:	Operational definitions to facilitate early diagnosis (no formal scoring system)
Major criteria Fever >39°C for ≥1 wk Arthralgia/arthritis for ≥2 wk Typical rash Leukocytosis >10 × 10⁹/liter with >80% PMN cells Minor criteria Sore throat Lymphadenopathy Hepatosplenomegaly Abnormal LFTs Negative ANA and RF	Major criteria Fever ≥39°C Arthralgia Transient erythema Pharyngitis PMN cells ≥80% Glycosylated ferritin ≤20% Minor criteria Maculopapular rash Leukocytosis ≥10 × 10⁹/liter	Acute respiratory failure Lung infiltrate Pleural effusion Cardiovascular failure (shock, myocarditis, and tamponade) Hematologic disorder (DIC, hemophagocytosis, and thrombotic microangiopathy)	Spiking fever ≥39°C for ≥7 days Evanescent, salmon-pink, truncal rash associated with fever spikes; atypical rashes possible Arthralgia/myalgia; arthritis may develop later (supportive but not required) Neutrophilic leukocytosis Elevated CRP and ferritin Elevated IL-18 and/or S100 proteins strongly supportive when available
Exclusion of infection, malignancy, and other rheumatic disease	Exclude infection and malignancy	Exclusion of alternative causes (infection, drug reaction, or malignancy)	Exclusion of infection, malignancy, other immune-mediated inflammatory diseases, and monogenic autoinflammatory disorders

Yamaguchi (12) (classification—1992)	Fautrel (13) (classification—2002)	Cush (2) (diagnostic—1987)	EULAR/PReS (4) (expert consensus diagnostic recommendations)
≥5 criteria (≥2 major)	≥4 major or 3 major + 2 minor	AOSD diagnosis by Yamaguchi or Fautrel plus ≥1 ICU-level organ failure:	Operational definitions to facilitate early diagnosis (no formal scoring system)
Major criteriaFever >39°C for ≥1 wkArthralgia/arthritis for ≥2 wkTypical rashLeukocytosis >10 × 10⁹/liter with >80% PMN cellsMinor criteriaSore throatLymphadenopathyHepatosplenomegalyAbnormal LFTsNegative ANA and RF	Major criteriaFever ≥39°CArthralgiaTransient erythemaPharyngitisPMN cells ≥80%Glycosylated ferritin ≤20%Minor criteriaMaculopapular rashLeukocytosis ≥10 × 10⁹/liter	Acute respiratory failureLung infiltratePleural effusionCardiovascular failure (shock, myocarditis, and tamponade)Hematologic disorder (DIC, hemophagocytosis, and thrombotic microangiopathy)	Spiking fever ≥39°C for ≥7 daysEvanescent, salmon-pink, truncal rash associated with fever spikes; atypical rashes possibleArthralgia/myalgia; arthritis may develop later (supportive but not required)Neutrophilic leukocytosisElevated CRP and ferritinElevated IL-18 and/or S100 proteins strongly supportive when available
Exclusion of infection, malignancy, and other rheumatic disease	Exclude infection and malignancy	Exclusion of alternative causes (infection, drug reaction, or malignancy)	Exclusion of infection, malignancy, other immune-mediated inflammatory diseases, and monogenic autoinflammatory disorders

ANA, antinuclear antibody; RF, rheumatoid factor; PMN, polymorphonuclear; ICU, intensive care unit; DIC, disseminated intravascular coagulation; LFT, liver function tests.