Sabtu, 22 Desember 2012

Adult Still's disease

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Adult Still's disease

Definition :
Adult Still's disease
 Adult Still's disease is a rare inflammatory condition that may lead to chronic arthritis and other complications. A separate condition, formerly known as Still's disease, is now commonly referred to as systemic onset juvenile rheumatoid arthritis (JRA) or juvenile idiopathic arthritis (JIA).
There's no cure for adult Still's disease, but treatment may offer symptom relief for adult Still's disease and help prevent complications.

Symptoms:

Most people with adult Still's disease experience a combination of the following signs and symptoms:
  • Fever. You may experience a daily fever of at least 102 F (38.9 C) for a week or longer. The fever usually peaks in the late afternoon or early evening. Sometimes, you may experience two fever spikes daily. Between episodes, your temperature will likely return to normal.
  • Rash. A salmon-pink bumpy or flat rash may come and go with the fever. The rash usually appears on your trunk, arms or legs. Physical contact such as rubbing your skin may provoke the rash to appear.
  • Achy and swollen joints. You may find that your joints — especially your knees, wrists, ankles, elbows, hands and shoulders — are stiff, painful and inflamed. Usually, the joint discomfort lasts at least two weeks.
  • Muscle pain. Muscular pain associated with adult Still's disease usually ebbs and flows with the fever, but the pain may be severe enough to disrupt your daily activities.
Other signs and symptoms may include:
  • Sore throat
  • Swollen lymph nodes in your neck
  • Enlarged liver or spleen
  • Inflammation of the lining of the heart or lungs
Having any of these signs or symptoms doesn't necessarily mean that you have adult Still's disease. The signs and symptoms of this disorder may mimic those of other conditions, including infectious mononucleosis, a type of cancer called lymphoma or other rheumatic diseases, including rheumatoid arthritis.

When to see a doctor
If you have a high fever, rash and achy joints, see your doctor to determine what may be the cause. Also, if you have adult Still's disease and develop a cough, difficulty breathing, chest pain or any other unusual symptoms, call your doctor.

Causes:

 Although it's not certain what causes adult Still's disease, the condition may be triggered by a viral or bacterial infection.

Complications:

Most complications from adult Still's disease arise from chronic inflammation of your body organs and joints.
  • Joint destruction. Chronic inflammation can damage your joints. The most commonly involved joints are your knees and wrists. Your neck, foot, finger and hip joints also may be affected, but much less frequently. In severe cases, joint replacement surgery may be necessary for your hip or knee joints.
  • Inflammation of your heart. Adult Still's disease can lead to an inflammation of the sac-like covering of your heart (pericarditis) or of the muscular portion of your heart (myocarditis).
  • Excess fluid around your lungs. Inflammation may cause an excess of fluid to build up in the fluid-filled space (pleural space) that surrounds your lungs.
Treatments and drugs:

Doctors use a variety of drugs to treat adult Still's disease. The type of drug you'll take depends on the severity of your symptoms and whether you experience side effects.
  • Nonsteroidal anti-inflammatory drugs (NSAIDs). These drugs, such as aspirin, ibuprofen (Advil, Motrin, others) or naproxen (Aleve, Anaprox, others), help reduce inflammation. They are often useful for controlling mild symptoms. If you take NSAIDs, you'll need regular blood tests to monitor your liver function, because high doses of NSAIDs can damage your liver.
  • Glucocorticoids. People with high-fever spikes, severe joint symptoms or complications with their internal organs might require glucocorticoids, such as prednisone. Glucocorticoids are corticosteroid medications that help subdue your body's immune response. While glucocorticoids are often a successful treatment for adult Still's disease, these drugs may lower your body's resistance to infections and will increase your risk of developing osteoporosis.
  • Methotrexate. The medication methotrexate (Rheumatrex, Trexall) has been used successfully in a small series of people to treat adult Still's disease. It may also be used as a "steroid-sparing agent," meaning that if you take methotrexate, smaller doses of corticosteroids may be able to control your disease.
Biologic response modifiers
Several drugs that are not standard therapy for adult Still's disease have been reported to help some people with this condition. One group inhibits tumor necrosis factor-alpha (TNF-alpha), an immune system cell involved in inflammation. TNF blockers include infliximab (Remicade), adalimumab (Humira) and etanercept (Enbrel). Although small studies have shown some promise, it's unknown whether TNF blockers provide long-term benefit in controlling the disease.
Other medications, including rituximab (Rituxan), cyclosporine (Sandimmune, Gengraf, Neoral) and anakinra (Kineret), also have been used successfully in the treatment of adult Still's disease in small groups of people.

Outlook
Even with treatment, it's difficult to predict the course of adult Still's disease. Some people might only experience a single episode, while for others adult Still's disease may develop into a chronic condition. About one-third of people with the disorder fall into each of the following groups:
  • Recovery. In some cases, the signs and symptoms disappear within one year and do not recur.
  • Occasional flare-ups. People with this form of the condition experience unpredictable eruptions of their signs and symptoms but feel normal between episodes.
  • Chronic arthritis. The chronic form of adult Still's disease may last for years and cause debilitating arthritis. Fortunately, most people with chronic adult Still's disease retain good function.

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