MedlinePlus Medical Encyclopedia: Adult Still's disease

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Adult Still's disease

Contents of this page:
Definition Causes, incidence, and risk factors Symptoms Signs and tests	Treatment Expectations (prognosis) Complications Calling your health care provider

Definition

Adult Still's disease is an illness with fever, rash, and joint pain. It may lead to chronic arthritis.

Causes, incidence, and risk factors

The cause of Adult Still's disease is unknown. The condition rarely occurs in adults. It is more common in children, where it is called Systemic Juvenile Rheumatoid Arthritis. No risk factors for the disease have been identified.

Symptoms

Almost all patients will have fever, joint pain, sore throat, and a rash. The fever usually comes on quickly once per day, most commonly in the afternoon or evening. The rash is typically salmon pink colored and comes and goes with the fever.

Another common symptom is joint pain and inflammation (warmth and swelling of the joint). Usually, several joints are involved at the same time.

Additional symptoms include swollen lymph nodes (glands), pain with a deep breath (pleurisy), abdominal pain and swelling, and weight loss.

Signs and tests

The physical exam may show the fever, rash, and arthritis. Other signs include enlargement of the lymph nodes, liver, or spleen. Also, the presence of changes in the sound of the heart or lungs may indicate pericarditis or pleurisy.

Blood tests that can be helpful in diagnosing Adult Still's Disease include:

Elevation in the ESR (sedimentation rate)
Elevation in the White Blood Cell count
Elevation in liver function tests
Decrease in the Red Blood Cell count
Very high elevation in the Ferritin level
Negative rheumatoid factor and ANA test

Other tests may include:

joint X-rays
chest X-ray that may show pericarditis or pleural effusion
abdominal X-ray, CT scan or ultrasound for liver and spleen enlargement

Adult Still's Disease can only be diagnosed after other diseases are excluded. It may require many medical tests before a final diagnosis is made.

Treatment

The symptoms of arthritis are generally controlled with adequate doses of salicylates (aspirin) or nonsteroidal anti-inflammatory medications (NSAIDs) such as ibuprofen. Prednisone may be used for more severe cases. In the disease becomes chronic immunosuppressive medications might be needed. These may include methotrexate or new biologic therapies.

Expectations (prognosis)

Studies show that about 20% of patients have all of the symptoms go away in a year and never come back. About 30% of patients have all of the symptoms go away, but they come back several times over the next years. The rest of the patients (about 50%) will develop a chronic arthritis.

Complications

arthritis
liver disease
spleen enlargement
pericarditis
pleural effusion

Calling your health care provider

Call for an appointment with your health care provider if symptoms are present that are suggestive of Adult Still's disease.

Call your health care provider if cough, difficulty breathing, or other symptoms develop in a person with Adult Still's.

Update Date: 9/11/2003

Updated by: Megan E. B. Clowse, M.D., M.P.H., Division of Rheumatology, Johns Hopkins Hospital, Baltimore, MD. Review provided by VeriMed Healthcare Network.

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