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Adult-Onset Still Disease

Rare systemic inflammatory disease with high fever, transient rash, and arthritis.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Adult-Onset Still Disease?

Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology. First described by Sir George Frederic Still in children in 1896, an analogous adult entity was later recognized. It belongs to the autoinflammatory spectrum.

The classic triad consists of daily short-duration high fever spikes (39-40°C), salmon-colored macular rash, and arthralgia/arthritis. Fever spikes typically peak once in the evening and return to normal by morning. The rash appears with the fever and then disappears.

High ferritin levels (>1000 ng/ml) and especially low glycosylated ferritin ratio (<20%) are characteristic laboratory findings. Yamaguchi and Fautrel criteria assist diagnosis. The disease may follow monophasic, intermittent, or chronic course.

Symptoms

Daily high fever spikes (above 39°C, usually evenings)
Salmon-colored transient macular rash (synchronous with fever)
Arthralgia and polyarticular arthritis
Sore throat (often the first symptom)
Lymphadenopathy and hepatosplenomegaly
Serositis (pericarditis, pleuritis)
Severe fatigue and weight loss
Myalgia and muscle weakness

Risk Factors

Young adult age group (16-35)
Female sex slightly more common
Recent viral infection (trigger)
Genetic predisposition (HLA-DR4, DR7)
History of macrophage activation syndrome
Environmental infectious triggers
Family history of autoinflammatory syndrome
Late-onset cases may be more resistant

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Unexplained daily high fever spikes (over 2 weeks)
  • Rashes appearing and disappearing with fever
  • Sore throat with joint pain
  • Very high ferritin (>1000)
  • Lymph node enlargement and hepatosplenomegaly
  • Unexplained systemic inflammation

Treatment Methods

01
High-dose NSAIDs initially (indomethacin)
02
Systemic corticosteroid (oral prednisolone or pulse methylprednisolone)
03
Methotrexate as steroid-sparing agent
04
IL-1 inhibitors (anakinra, canakinumab) — selected therapy
05
IL-6 inhibitor tocilizumab (in resistant cases)
06
Cyclosporine A and azathioprine alternatives
07
Intensive care monitoring in macrophage activation syndrome
08
Regular ferritin and inflammation marker monitoring

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.