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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.

    The disease is much less common and now called adult-onset Still's disease (AOSD).

    Causes

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

    Still's disease that occurs in children is called systemic juvenile idiopathic arthritis.

    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, patients 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 with 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 Still's disease can only be diagnosed after other diseases 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 usually 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:

    • Anakinra (interleukin-1 receptor agonist)
    • Methotrexate
    • 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

    • 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 Still's disease.

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

    References

    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: Saunders Elsevier;2012:chap 43.

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

    Pay S, Turkcapar N, Kalyoncu M, et al. A multicenter study of patients with adult-onset Still's disease compared with systemic juvenile idiopathic arthritis. Clin Rheumatol. 2006;25:639-644.

    Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still's disease. Ann Rheum Dis. 2006;65(5):564-572.

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              Review Date: 3/22/2013

              Reviewed By: Ariel D. Teitel, MD, MBA, Clinical Associate Professor of Medicine, NYU Langone Medical Center. Review provided by VeriMed Healthcare Network. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang

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