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Still's Disease

Statistics

  • Onset rarely occurs in people over the age of 40.

  • Still's disease accounts for 10-20% of all cases of JRA.

What is Still's Disease?

First described by Dr. George Still in 1897, Still's disease is a form of juvenile rheumatoid arthritis that has systemic features as the major symptoms. Although more common in children it can present in adults, known as Adult-Onset Still's Disease.

What causes it?

The cause is unknown, but like many other types of arthritis, abnormal immune response, genetic predisposition and environmental triggers, and infectious agents are all being considered.What are the symptoms?High fevers are a common symptom, rising as high 106 degrees and falling quickly, only to rise again. Salmon colored skin rash that does not itch is also common. Other physical symptoms include joint pain, fatigue, and sore throat. The heart, lungs, spleen and lymph glands may become inflamed.

How is it diagnosed?Diagnosis is difficult because the symptoms so often resemble those of other types of arthritis. Diagnosis is made after careful physical exam, medical history, and by the use of blood tests and x-rays. The rheumatoid factor and ANA which may be positive in other types of arthritis, are usually negative with Still's disease.How is it treated?The treatment of JA focuses on relieving pain, reducing inflammation, slowing or stopping joint damage, and improving the individual's sense of well-being and ability to function.

Medications:
NSAIDS (Nonsteroidal anti-inflammatory drugs) are commonly the first type of drug used to treat the joint pain associated with Still's. Available over the counter or by prescription, they fight inflammation or swelling and relieve pain. Aspirin is avoided if possible due to the risk of Reye's syndrome in children.

DMARDS (disease modifying anti-rheumatic drugs) are also known as SAARDS (slow acting anti rheumatic drugs. As their name might suggest these medications take some time to be effective, up to several months. Most of them were originally invented to treat other diseases but were noted to have a positive effect on rheumatic diseases. Most of them do not have any common properties other than their ability to slow down the progression of the disease. They are usually given in combination with a NSAID.

Stronger medicines may be needed to stop serious symptoms such as inflammation of the sac around the heart (pericarditis). Corticosteroids like prednisone may be added to the treatment plan to control severe symptoms. The risk of serious side effects in children is fairly high. Once the disease is brought under control the corticosteroid is likely to be reduced or discontinued. Reduction of corticosteroids should always be done under the supervision of a doctor. Never stop a steroid suddenly without a doctor's approval.

Physical Therapy & Exercise:
Physical therapy is an important part of the treatment plan. Exercise can help to maintain muscle tone and preserve and recover the range of motion of the joints. A physical therapist can design an appropriate exercise program.

Joint protection:
Splints, braces and supports can protect the joints and help prevent further damage. A doctor or a physical or occupational therapist can help get a splint and ensure that it fits properly. Other ways to reduce stress on joints include self-help devices, devices to help with getting on and off chairs, toilet seats, and beds; and changes in the ways that a child carries out daily activities.

Surgery:
Some cases of Still's are very aggressive, causing severe joint destruction. Several types of surgery are available to people with severe joint damage. These procedures can help reduce pain, improve the affected joint's function and appearance, and improve the patient's ability to perform daily activities. Surgical procedures include joint replacement, tendon reconstruction, and synovectomy.

What research is being done?Many types of research are ongoing. At one of the latest meetings of the American College of Rheumatology a paper was presented which demonstrated the effectiveness of intravenous immunoglobulin therapy in adult-onset Still disease. This was a pilot study. More studies are needed to confirm these results.For more information:International Still's Disease Foundation 
MedicineNet - Still's Disease 
Still's Disease Homepage 
University of Washington - Arthritis in Children
References:
Koopman: Arthritis and Allied Conditions, 13th ed., Copyright © 1997 Williams & Wilkins
Medicinenet
International Still's Disease Foundation