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Criteria for Diagnosis of Still’s Disease

Yamaguchi criteria for classification of adult Still’s disease

Presence of 5 or more criteria, of which at least 2 are Major (96% sensitivity; 92% specificity)

Yamaguchi criteria for classification of adult Still’s disease
Presence of 5 or more criteria, of which at least 2 are Major (96% sensitivity; 92% specificity)

Major Criteria

  • Temperature of >39°C for >1 wk
  • Leukocytosis >10,000/mm3 with >80% PMNs
  • Typical rash
  • Arthralgias >2 wk

Minor Criteria

  • Sore throat
  • Lymph node enlargement
  • Splenomegaly
  • Liver dysfunction (high AST/ALT)
  • Negative ANA, RF

Cush criteria for classification of adult Still’s disease

Requires all of the following:

  • Fever > 39 degrees
  • Arthralgia or arthritis
  • Rheumatoid factor < 1:80
  • ANA < 1:100

In addition to any two of the following:

  • WBC count > 15,000
  • Stills rash
  • Pleuritis or Pericarditis
  • Hepatomegaly, Splenomegaly, or Lymphadenopathy

Abbreviations: ALT, alanine transaminase; ANA, antinuclear antibody; AST, aspartate transminase; PMN, polymorphonuclear leukocyte; RF, rheumatoid factor; WBC, white blood cells.

Fautrel classification criteria for adult-onset Still’s disease

Four or more major criteria are required, or 3 major and 2 minor criteria

Major criteria

  • Spiking fever ≥39 °C
  • Arthralgia
  • Transient erythema
  • Pharyngitis
  • Polymorphonuclear cells ≥80%
  • Glycosylated ferritin ≤20%

Minor criteria

  • Maculopapular rash
  • Leukocytosis ≥10,000/mm3

 

References:

  1. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992 Mar;19(3):424-30. [Medline]
  2. Cush JJ, Medsger TA Jr, Christy WC, Herbert DC, Cooperstein LA. Adult-onset Still’s disease. Clinical course and outcome. Arthritis Rheum. 1987 Feb;30(2):186-94. [Medline]
  3. Lebrun D, Mestrallet S, Dehoux M, Golmard JL, Granger B, Georgin-Lavialle S, Arnaud L, Grateau G, Pouchot J, Fautrel B. Validation of the Fautrel classification criteria for adult-onset Still’s disease. Semin Arthritis Rheum. 2018 Feb;47(4):578-585. [Medline]

 

Created: Nov 23, 2007
Update: Sep 17, 2021.

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