What is Still's disease?

Still's disease, a disorder featuring inflammation, is characterized by high spiking fevers, evanescent (transient) salmon-colored rash, and/or arthritis. Still's disease was first described in children, but it is now known to occur, much less commonly, in adults (in whom it is referred to as adult-onset Still's disease).

What causes Still's disease?

There have been a number of schools of thought regarding the cause(s) 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 a hypersensitive or autoimmune disorder. In fact, the cause of Still's disease is not yet known.

How does Still's disease fit in with juvenile rheumatoid arthritis?

Still's disease is one type of juvenile rheumatoid arthritis (JRA) and is also known as systemic-onset JRA. 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. Still's disease is named after the English physician Sir George F. Still (1861-1941).

What are symptoms and signs of Still's disease?

Still's disease usually begins with systemic (body-wide) symptoms. Extreme fatigue can accompany waves of high fevers that rise daily to 102 degrees F (39 degrees C) or even higher and rapidly return to normal levels or below. Fever spikes often occur at nearly 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 swelling. This usually involves many joints (polyarticular arthritis). Any joint can be affected, although there are preferential patterns of joint involvement in Still's disease.

How is Still's disease diagnosed?

Still's disease is diagnosed purely on the basis of the typical clinical features of the illness. These features include high intermittent fever, joint inflammation and pain, muscle pain, faint salmon-colored skin rash, swelling of the lymph glands or enlargement of the spleen and liver, and inflammation around the heart (pericarditis) or lungs (pleuritis). Persistent arthritis (arthritis lasting at least six weeks) is required to make a firm diagnosis of Still's disease. Other diseases (especially infections, cancers, and other types of arthritis) are excluded.

Many patients with Still's disease develop markedly elevated white blood cell counts, as if they have a serious infection, but no infection is found. Low red blood counts (anemia) and elevated blood tests for inflammation (such as sedimentation rate and C-reactive protein) are common. However, the classic blood tests for rheumatoid arthritis (rheumatoid factor) and systemic lupus erythematosus (antinuclear antibodies) are usually negative.

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

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

The fever and other systemic features tend to run their course within several months. The arthritis can be a long-term problem. It usually stays on after the systemic features have gone. The arthritis can then become chronic and persist into adulthood. Persisting arthritis of the wrists, with eventual fusion, is common.

How is Still's disease treated?

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 nonsteroid drugs (NSAIDs). 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 gold, hydroxychloroquine (Plaquenil), penicillamine (Cuprimine, Depen), azathioprine (Imuran), methotrexate (Rheumatrex, Trexall), and cyclophosphamide (Cytoxan).

What research is being done on Still's disease?

A wide variety of research is ongoing related to Still's disease. At one of the recent meetings of the American College of Rheumatology, for example, a paper was presented which demonstrated the effectiveness of intravenous immunoglobulin therapy in adult-onset Still's disease. More studies are needed to confirm these results.

Also, TNF-inhibitors (etanercept [Enbrel] and infliximab [Remicade]) have been used successfully in people who have failed other treatments (much as they are now used in rheumatoid arthritis). The intravenous infusions of infliximab helped decrease the Still's disease signs and symptoms, including fever, arthritis, muscle pains, enlarged spleen, and rash, in virtually all patients in some studies.

Recent research suggests that anakinra (Kineret) may be a beneficial treatment for Still's disease.

For more information about Still's disease, please visit the following sites:

• Pediatric Rheumatology home page (http://www.goldscout.com)



• Arthritis Foundation (http://www.arthritis.org)

Still's Disease At A Glance

• Still's disease presents with a high spiking fever.
• Still's disease is commonly associated with a rash without itching.
• Still's disease always causes joint inflammation (arthritis).
• The cause of Still's disease is not known.
• Still's disease can be associated with inflammation of internal organs.
• Still's disease is diagnosed by the characteristic clinical features. These features include high intermittent fever; joint inflammation and pain, muscle pain, faint salmon-colored skin rash, swelling of the lymph glands or enlargement of the spleen and liver, and inflammation of the lungs (pleuritis) or around the heart (pericarditis).
• Classic blood tests for rheumatic diseases are negative.
• Treatment is directed toward the individual areas of inflammation.

References:

Koopman, William, et al., eds. Clinical Primer of Rheumatology. Philadelphia: Lippincott Williams & Wilkins, 2003.

Kelley's Textbook of Rheumatology, W B Saunders Co, edited by Shaun Ruddy, et al., 2000.

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