Adult-Onset Still’s Disease (AOSD)

Medicine 3

Head of Department:
Prof. Dr. med. univ. Georg Schett

Understanding causes

Still's disease belongs to the systemic auto-inflammatory diseases, which usually manifest themselves in early childhood (as systemic juvenile idiopathic arthritis (SJIA)) or in adulthood (Still's syndrome). During the course of the disease, the innate immune system is activated. To date, little is known about which trigger(s) in AOSD leads to this activation.


The onset of the disease is often between the ages of 15 and 40. However, two age peaks between 15 and 25 years and between 36 and 46 years are also observed for the onset of the disease. Less than 10% of patients are older than 50 years at the beginning of the disease.

Both, Still's disease and SJIA, are associated with a high activity of interleukin-1 (inflammatory messenger).

Figures around the disease

Still's disease in adults occurs worldwide. It is estimated that less than 1 person per 100,000 inhabitants suffers from AOSD. Women and men are equally affected, but women seem to develop joint problems more often than men.

Typical symptoms

Clinical:

  • Intermittent fever with accompanying fever peaks (often occurring in the afternoon or evening)
  • Arthritides (joint pain)
  • Salmon coloured exanthema
  • Possible swelling of the lymph nodes
  • sore throat
  • enlargement of the liver and spleen (hepatomegaly and/or splenomegaly)

Lab:

  • Increase of the C-reactive protein (CRP)
  • Increase of the blood sedimentation rate (BKS, ESR)
  • Increase of further so-called "acute phase proteins" (e.g. ferritin massively increased, S100 proteins increased).

How does the physician make the diagnosis?

Still's disease is always diagnosed in the context of an exclusion diagnosis. Infections, malignancies and other rheumatological diseases (mainly systolic vasculitis) must first be excluded.

The standard Yamaguchi Classification criteria of 1992 (Yamaguchi M et al., J Rheumatol 1992; 19:424-430) are used: Five or more criteria are required, whereby at least two main criteria must be fulfilled.

Main criteria:

  •     Fever > 39°C, ≥ 1 week
  •     Arthralgia or arthritis, ≥ 2 weeks
  •     Typical skin rash
  •     Leukocytosis > 10,000/mm3 with > 80% polymorphonuclear cells

Additional criteria

  •     Sore throat
  •     Development of a significant lymphadenopathy
  •     Hepatomegaly or splenomegaly
  •     Abnormal liver function test
  •     Negative for antinuclear antibodies (IF), rheumatoid factors (IgM)

How is Adult-Onset Still's Disease (AOSD) treated?

In principle, the choice of therapy is based on a consensus between the rheumatologist and the patient. A German guideline recommendation does not currently exist for Still's disease.

  • Initial treatment is usually with glucocorticoids
  • In individual cases use of csDMARD (conventional disease-modifying drugs, such as cyclosporin A, cyclophosphamide, leflunomide or methotrexate)
  • Approved biologicals: Anakinra (Kineret) or Canakinumab Ilaris (both inhibit the messenger IL-1)
  • symptomatic therapy

Practical tips for everyday life

With fever > 39°C, ≥ 1 week and exclusion of malignancies and infections, autoinflammatory diseases should also be considered and a rheumatologist should be consulted. Especially, if ferritin is also massively increased (> 3-5 times the normal value).

 
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