Adult-onset Still disease

Adult-onset Still disease is a rare multisystem inflammatory disorder.

Still disease in adults is rare affecting around 1.5 per 100,000 people, it occurs in a bimodal distribution with one peak around the age of 15-25 years old and another around the age of 35-45 years old 1. It affects females more than males 2.

Adult-onset Still disease classically is described as a triad of:

  • high fevers
  • arthralgia
  • salmon-colored rash

Less common are:

Elevated inflammatory markers (e.g. CRP), leukocytosis, thrombocytosis and elevated ferritin are common 3.

The cause of adult-onset Still disease is unknown but genetic, environmental and infectious factors may play a role 3,4. Elevated levels of various cytokines are seen in adult-onset Still disease. Interleukin-18-induced activation of macrophages and neutrophils appears to be important in Still disease. Interleukin-1-β also seems to be important in Still disease causing neutrophil production and diapedesis 5

The Yamaguchi criteria is probably the most commonly used and sensitive scoring system for diagnosing adult-onset Still disease 6.

Adult-onset Still disease is a seronegative disease meaning a negative rheumatoid factor and anti-nuclear antibodies are commonly found.

Pericapitate and trapezoid-metacarpal joint space narrowing without radiocarpal involvement is characteristic 7. This pattern is rarer in rheumatoid arthritis.

Steroids are commonly used to achieve remission of acute disease. Disease modifying anti-rheumatic drugs may be used to maintain remission 3.

Three main patterns of disease have been described:

  1. single acute episode before a period of lifelong remission
  2. acute symptomatic episodes separated by months-years of no symptoms
  3. chronic articular disease causing joint destruction 3

Adult-onset Still disease was described first in 1971, by the renowned British rheumatologist Eric G L Bywaters (1910-2003) 8, who thought the disease resembled systemic juvenile idiopathic arthritis (pediatric Still disease) 1.

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Article information

rID: 59949
Synonyms or Alternate Spellings:
  • Adult Still's disease
  • Adult-onset Still disease (AOSD)
  • Adult-onset Still's disease (AOSD)

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