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Adult Still's disease

Still's disease - adult; AOSD

 

Adult Still's disease is a rare illness that causes high fevers, rash, and joint pain. It may lead to long-term (chronic) arthritis.

Still's disease is a severe version of juvenile idiopathic arthritis (JIA), which occurs in children. Adults can have the same condition although it is much less common. It is also called adult-onset Still's disease (AOSD).

Causes

 

Fewer than 1 out of 100,000 people develop adult-onset Still's disease each year. It affects women more often than men.

The cause of adult Still's disease is unknown. No risk factors for the disease have been identified.

 

Symptoms

 

Almost all people with the disease will have fever, joint pain, sore throat, and a rash.

  • Joint pain, warmth, and swelling are common. Most often, several joints are involved at the same time. Often, people with the condition have morning stiffness of joints that lasts for several hours.
  • The fever comes on quickly once per day, most commonly in the afternoon or evening.
  • The skin rash is often salmon-pink colored and comes and goes with the fever.

Additional symptoms include:

  • Abdominal pain and swelling
  • Pain when taking a deep breath (pleurisy)
  • Sore throat
  • Swollen lymph nodes (glands)
  • Weight loss

The spleen or liver may become swollen. Lung and heart inflammation may also occur.

 

Exams and Tests

 

Adult-onset Still's disease can only be diagnosed after many other diseases (such as infections and cancer) are ruled out. You may need many medical tests before a final diagnosis is made.

A physical exam may show a fever, rash, and arthritis. The health care provider will use a stethoscope to listen for changes in the sound of your heart or lungs.

The following blood tests can be helpful in diagnosing adult Still's disease:

  • Complete blood count (CBC), may show a high number of white blood cells and reduced number of red blood cells.
  • C-reactive protein (CRP), a measure of inflammation, will be higher than normal.
  • ESR (sedimentation rate), a measure of inflammation, will be higher than normal.
  • Ferritin level will be very high.
  • Fibrinogen level will be high.
  • Liver function tests will show high levels of AST and ALT .
  • Rheumatoid factor and ANA test will be negative.

Other tests may be needed to check for inflammation of the joints, chest, liver, and spleen:

  • Abdominal ultrasound
  • CT scan of the abdomen
  • X-rays of the joints, chest, or stomach area (abdomen)

 

Treatment

 

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 most often used first.

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:

  • Methotrexate
  • Anakinra (interleukin-1 receptor agonist)
  • Tocilizumab (interleukin 6 inhibitor)
  • Tumor necrosis factor (TNF) antagonists such as Enbrel (etanercept)

 

Outlook (Prognosis)

 

Studies show that in about 1 in 5 people all symptoms go away in a year and never come back. In many people, symptoms go away but then come back several times over the next few years.

Symptoms continue for a long time (chronic) in about half of patients with adult Still's disease.

 

Possible Complications

 

Complications may include:

  • Arthritis in several joints
  • Liver disease
  • Pericarditis
  • Pleural effusion
  • Spleen enlargement

 

When to Contact a Medical Professional

 

Call your health care provider if you have symptoms of adult-onset Still's disease.

If you have already been diagnosed with the condition, you should call your provider if you have a cough or difficulty breathing .

 

 

Open References

References

Clarke JT. Other Rheumatologic Diseases. In: Bolognia JL, Jorizzo JL, Schaffer JV, et al, eds. Dermatology . 3rd ed. Philadelphia, PA: Elsevier Mosby; 2012:chap 45.

Gerfaud-Valentin M, Maucort-Boulch D, Hot A, et al. Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Medicine (Baltimore). 2014;93:91. PMID: 24646465 www.ncbi.nlm.nih.gov/pubmed/24646465 .

Lee LA, Werth VP. The Skin and Rheumatic Diseases. In: Firestein GS, Budd RC, Gabriel SE, et al, eds. Kelley's Textbook of Rheumatology . 9th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 43.

 

        A Closer Look

         

        Talking to your MD

         

          Self Care

           

            Tests for Adult Still's disease

             
               

              Review Date: 1/20/2015

              Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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