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-coloured 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.

Diffuse pericapitate joint space narrowing with radiocarpal involvement is commonly seen 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 (paediatric 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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