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Adult onset Still's Disease

Definition


Definition of Adult onset Still's Disease
Adult-onset Still's disease is a rare form of inflammatory arthritis that was characterized by EG Bywaters in 1971. The underlying cause is unknown. It usually presents with high spiking fevers, joint and muscle pains, a salmon colored rash and other symptoms of systemic inflammation.

Symptoms


Symptoms of Adult onset Still's Disease
The disease typically affects 16–35 year olds and presents with arthralgia, elevated serum ferritin, a 'salmon-pink' rash, pyrexia and lymphadenopathy. Rheumatoid factor (RF) and anti-nuclear antibody (ANA) are classically negative. Patients experiencing a flare-up from Adult-onset Still's disease usually report extreme fatigue, swelling of the lymph glands, and less commonly fluid accumulation in the lungs and heart.

Causes


Causes of Adult onset Still's Disease
Although it's not certain what causes adult Still's disease, the condition may be triggered by a viral or bacterial infection.

Diagnosis


Diagnosis of Adult onset Still's Disease
The diagnosis is clinical, not based upon serology. At least seven sets of diagnostic criteria have been devised, however the Yamaguchi criteria have the highest sensitivity. Diagnosis requires at least five features, with at least two of these being major diagnostic criteria.

Treatment


Treatment of Adult onset Still's Disease
The goal of treatment for adult Still's disease is to control the symptoms of arthritis. Aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, are usually the first treatment used.

Prednisone may be used for more severe cases.

If the disease persists for a long time (becomes chronic), medicines that suppress the immune system might be needed. Such medicines include:

  1. Anakinra (interleukin-1 receptor agonist)
  2. Methotrexate
  3. Tumor necrosis factor (TNF) antagonists such as Enbrel (etanercept)

Prognosis


Prognosis of Adult onset Still's Disease
Studies show that in about 20% of patients, all symptoms go away in a year and never come back. In about 30% of patients, all symptoms go away but then come back several times over the next few years.

Prevention


Prevention of Adult onset Still's Disease
Adult Still's disease can't be prevented. However, sticking to your treatment regimen and calling your doctor if you develop problems may prevent complications.


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