Adult Still’s disease

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:

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.

Johns Hopkins patient information

Last revised: December 4, 2012
by Amalia K. Gagarina, M.S., R.D.

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