Adult-onset Still's disease diagnostic study of choice

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Adult-onset Still's disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

The diagnosis of adult-onset Still's disease (AOSD) is made clinically along with supporting laboratory abnormalities and exclusion of other rheumatologic disorders, malignancy and infections. Three diagnostic criteria have been established in order to aid in the diagnosis of AOSD. Yamaguchi criteria is widely adopted.

Diagnostic study of choice/ Diagnostic criteria

The diagnosis of adult-onset Still's disease is made clinically along with supporting laboratory abnormalities and exclusion of other rheumatologic disorders, malignancy and infections. Three diagnostic criteria have been established in order to aid in the diagnosis of AOSD. Yamaguchi criteria is widely adopted. The following table outlines the major criteria system for the diagnosis of AOSD:[1][2][3]

Criteria Yamaguchi criteria Cush criteria Fautrel criteria
Major criteria Arthralgia >2 weeks Daily fever >39° Spiking fever ⩾39°
Fever >39°, intermittent, high spiking, ⩾1 week Still's (maculopapular) rash Arthralgia
Maculopapular rash on trunk and/or extremities WBCs >12000/ml +ESR >40 mm/1st hour Transient erythema
Leukocytosis- WBCs >10000 (>80% neutrophils) Νegative RF and ANA Pharyngitis
- Carpal ankylosis Polymorphonuclear cells ⩾80%
- Glycosylated ferritin ⩽20%
Minor criteria Sore throat Onset age <35 years Maculopapular rash
Lymphadenopathy Arthritis Leukocytes ⩾10×109/l
Abnormal liver function tests (LDH, ALT, AST) Prodromal sore throat -
Negative ANA and RF Reticuloendothelial involvement or abnormal LFTs
Hepatosplenomegaly Serositis

References

  1. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, Tanimoto K, Matsumoto Y, Ota T (March 1992). "Preliminary criteria for classification of adult Still's disease". J. Rheumatol. 19 (3): 424–30. PMID 1578458.
  2. Cush JJ, Medsger TA, Christy WC, Herbert DC, Cooperstein LA (February 1987). "Adult-onset Still's disease. Clinical course and outcome". Arthritis Rheum. 30 (2): 186–94. PMID 3827959.
  3. Fautrel B, Zing E, Golmard JL, Le Moel G, Bissery A, Rioux C, Rozenberg S, Piette JC, Bourgeois P (May 2002). "Proposal for a new set of classification criteria for adult-onset still disease". Medicine (Baltimore). 81 (3): 194–200. PMID 11997716.

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