Still's Disease (Systemic-Onset Juvenile Idiopathic Arthritis)

  • Medical Editor: William C. Shiel Jr., MD, FACP, FACR
    William C. Shiel Jr., MD, FACP, FACR

    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

  • Medical Editor: Catherine Burt Driver, MD
    Catherine Burt Driver, MD

    Catherine Burt Driver, MD

    Catherine Burt Driver, MD, is board certified in internal medicine and rheumatology by the American Board of Internal Medicine. Dr. Driver is a member of the American College of Rheumatology. She currently is in active practice in the field of rheumatology in Mission Viejo, Calif., where she is a partner in Mission Internal Medical Group.

Quick GuideRheumatoid Arthritis Symptoms and Treatment

Rheumatoid Arthritis Symptoms and Treatment

What is the frequency of Still's disease and its features?

Still's disease accounts for 10%-20% of all cases of JIA. It affects about 25,000-50,000 children in the United States. It is rare in adults, a majority of whom are 20-35 years of age at the onset of symptoms.

Of all patients with Still's disease, 100% have high intermittent fever, and 100% have joint inflammation and pain, muscle pain with fevers, and develop persistent chronic arthritis. Approximately 95% of Still's disease patients have the faint salmon-colored skin rash, 85% have swelling of the lymph glands or enlargement of the spleen and liver, 85% have a marked increase in the white blood cell count, 60% have inflammation of the lungs (pleuritis) or around the heart (pericarditis), 40% have severe anemia, and 20% have abdominal pain.

What is the treatment for Still's disease?

Still's disease can cause serious damage to the joints, particularly the wrists. It can also impair the function of the heart and lungs. Treatment of Still's disease is directed toward the individual areas of inflammation. Many symptoms are often controlled with anti-inflammatory drugs such as aspirin or other nonsteroidal drugs (NSAIDs). It has been reported that some patients with Still's disease can frequently have elevations of liver function blood tests as a side effect of aspirin and sometimes other anti-inflammatory medications. Cortisone medications (steroids) such as prednisone are used to treat more severe features of illness.

For patients with persistent illness, medications that affect the inflammatory aspects of the immune system are used. Medications now being used are analogous to the classic "second-line" therapies used for patients with rheumatoid arthritis. These include hydroxychloroquine (Plaquenil), penicillamine (Cuprimine, Depen), azathioprine (Imuran), methotrexate (Rheumatrex, Trexall), and cyclophosphamide (Cytoxan).

Recently, because Still's disease has been found to involve a specific chemical messenger of inflammation known as interleukin 1 (IL-1), the injectable biologic medication anakinra (Kineret), which blocks IL-1, has been found to be a very effective treatment for Still's disease. Still's disease also involves interleukin 6 (IL-6). Tocilizumab (Actemra), an intravenous treatment which blocks IL-6, is approved to treat systemic JIA in children.

Medically Reviewed by a Doctor on 12/8/2015

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