Adult Onset Still Disease

Understanding adult onset still disease

Also known as simply adult Still's disease, Adult-onset Still’s disease (AOSD) is a rare condition that affects about 1 in every 100,000 adults. This illness causes rashes, high fever, and joint pain and may lead to chronic arthritis if left untreated. AOSD is a severe version of the juvenile idiopathic arthritis (JIA), which mostly affects children, though adults can get it too.

What are the causes of Still's disease?

There are various speculations surrounding the cause of this condition. Some people say it's caused by an infection caused by a microbe, while others claim it occurs due to an autoimmune issue. Definitely, the exact cause is still unknown.

Some research states that cytokines may have a role in the development of this condition. Research is still ongoing to help shed more light on this rare disease, thus improving care for patients.

Signs & symptoms

The symptoms of Still's disease usually begins with a high fever that occurs daily for a week or longer. Patients can expect to have a body temperature of about 102 F (38.9 C). The fever tends to intensify in the late afternoon or early evening. Patients experience two spikes of fever every day with the temperature reverting to normal in between.

A rash also appears on the arms, legs or trunk, and tends to come and go with the fever. It's usually salmon-pink. A sore throat is one of the initial symptoms, and you can have swollen and tender lymph nodes. The knee and wrist joints might become stiff, inflamed and swollen. You might also experience aching in your shoulders, hands, elbows and ankles.

Another common symptom is muscle pain, which comes and goes with the fever and can be severe enough to ruin your normal routine. These symptoms are likely to resemble those of other conditions, such as lymphoma and lupus. So, you'll need a medical diagnosis to confirm the condition.

Medical diagnosis

With Adult-onset Still’s disease, there is no specific test for diagnosing it. Doctors usually strive to rule out related conditions during a medical visit. You'll need to undergo several tests before a conclusion can be made.

Certain conditions, such as Lyme disease, mononucleosis, and some cancers, share the initial symptoms of Still's disease. You will also take a blood test to determine the level of ferritin, which usually increases in AOSD. The three primary symptoms the doctor will examine are fever, rash, and joint pain.

Other tests that might be helpful during a diagnosis include complete blood count, c-reactive protein, liver function tests, rheumatoid factor and ANA test, and sedimentation rate. You may also have imaging tests, such as a CT, ultrasound, and X-rays, to check for inflamed heart lining, an enlarged liver or spleen, fluid build up in the chest area, and joint inflammation.

Treatments for adult-onset Still’s disease

Still's disease has no cure and there's still no possible way to prevent it. Therefore, the purpose of treatment is usually to control the symptoms and the course of the condition.

Arthritis usually occurs with AOSD, so treatment usually focuses on addressing arthritis. Prednisone is the commonly prescribed medication to lower inflammation but may reduce your body's immune response. In the early stage of the condition, patients are usually given nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen and naproxen, to relieve inflammation and pain.

Immunosuppressive drugs, such as biologic response modifiers and methotrexate, may be needed if your arthritis becomes chronic. You can also get medications for boosting your immune response such as Actemra, Kineret, Rheumatrex, Enbrel, and Rumicade.

It's important to be consistent in the use of the prescribed medications. Doctors also recommend exercises to help in maintaining joint and muscle strength. You may also be directed to use vitamin supplements, such as vitamin D and calcium, to prevent osteoporosis, especially when taking prednisone.

Adult-onset Still’s disease has no cure, but proper treatment and care make it manageable. In some cases, symptoms tend to recur, and you can use medication to get relief. Those who are at high risk of AOSD are people between the ages of 15 and 46. This condition affects both men and women. If left untreated, AOSD can lead to complications such as liver disease, spleen enlargement, pericarditis, and joint arthritis. Visit the doctor once you notice unusual symptoms and always follow the given instructions when using medication.

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Last Reviewed:
June 14, 2017
Last Updated:
October 25, 2017
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