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.

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What causes Still's disease?

There have been a number of schools of thought regarding the cause (or causes) of Still's disease. One concept is that Still's disease is due to infection with a microbe. Another idea is that Still's disease is an autoimmune disorder. In fact, the precise cause of Still's disease is not yet known.

What are risk factors for Still's disease?

There are no specific known risk factors for the development of Still's disease.

How does Still's disease relate to juvenile idiopathic arthritis?

Still's disease is one type of juvenile idiopathic arthritis (JIA) and is also known as systemic-onset JIA. It is referred to as "juvenile" because it typically affects children under 16 years of age. By systemic, it is meant that along with joint inflammation it typically begins with symptoms and signs of systemic (body-wide) illness, such as high fevers, gland swelling, and internal organ involvement. By idiopathic, it is meant that the disease has no known cause. Systemic-onset JIA was formerly known as systemic-onset juvenile rheumatoid arthritis (JRA) and is the same disease. Several years ago, the naming system for all types of JRA changed, and JRA is now called juvenile idiopathic arthritis or JIA.

What are Still's disease symptoms and signs?

Still's disease usually begins with systemic (body-wide) symptoms. Extreme fatigue can accompany waves of high fevers that rise daily to 102 F (39 C) or even higher and rapidly return to normal levels or below. Fever spikes often occur at approximately the same time every day. A faint salmon-colored skin rash characteristically comes and goes and does not itch.

Poor appetite, nausea, and weight loss are common. There is also commonly swelling of the lymph glands, enlargement of the spleen and liver, and sore throat. Some patients develop inflammation around the heart (pericarditis) and lungs (pleuritis), with occasional fluid accumulation around heart (pericardial effusion) and lungs (pleural effusion). Arthritis, with joint swelling, often occurs after rash and fevers have been present for some time. Although the arthritis may initially be overlooked because of the impressive nature of the systemic symptoms, everyone with Still's disease eventually develops joint pain and swollen joints. This usually involves many joints (polyarticular arthritis). Any joint can be affected, although there are preferential patterns of joint involvement in Still's disease.

Medically Reviewed by a Doctor on 12/8/2015

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