Spotlight on adult Still's disease

We are always happy to present information requested by our readers, and are excited to be receiving requests for spotlight features on different types of arthritis. Please contact us at if you would like us to profile a specific type of arthritis.

Adult Still's disease is a rare form of arthritis which is characterized by high fevers, inflammation of the joints, and a salmon-coloured rash on the skin. In children, this disease is known as systemic onset juvenile rheumatoid arthritis; when it occurs in people over age 15, it is known as adult Still's disease.

Adult Still's disease affects men and women in approximately equal numbers. Though it can occur in adults of all ages, it tends to strike two age groups most commonly: those from age 15 - 25, and those from age 36 - 46. A fairly rare form of arthritis, adult Still's disease affects approximately 1 in 100,000 adults.

The cause of adult Still's disease syndrome remains unknown, although scientists think that it may be the result of an environmental trigger such as a prior viral or bacterial infection, ultraviolet light exposure, toxins, stress, among others. Because the disease only very rarely affects members of the same family, researchers are doubtful that there is a genetic connection.

Diagnosis of adult Still's disease

Adult Still's disease has several warning signs, which may be present at the onset of disease. These include:
  • High (40 degrees or higher) spiking fever, often occurring in the late afternoon or early evening; rarely, fevers can occur twice a day
  • Joint pain and inflammation
  • Faint, salmon-coloured rash, which may be bumpy or flat and will often occur with fever
  • Muscle pain, which often comes and goes with fever and may be severe
  • Sore throat
  • Swollen lymph nodes in the neck
  • Enlarged liver and/or spleen
  • Poor appetite, nausea, and weight loss

The early symptoms of adult Still's disease are very similar to those of many other diseases, including rheumatic fever (due to the streptococcus bacteria, infectious mononucleosis, lymphoma, and other forms of arthritis like lupus. For this reason, it is often a very challenging disease to diagnose, and one, which can easily be misdiagnosed. If you suspect that you may have adult Still's disease, speak with your doctor.

The first step in the process of diagnosing adult Still's disease is often eliminating other diseases that have similar symptoms to those of adult Still's disease. Once other conditions are proven not to be present, doctors may begin to investigate the possibility of adult Still's disease.

If your doctor believes you may have adult Still's disease, you will usually be referred to a rheumatologist-a specialist in the treatment of arthritis. Rheumatologists have many years of extra training on top of their regular medical schooling, and are experts at diagnosing and treating all forms of arthritis, including adult Still's disease.

To get a firm diagnosis of adult Still's disease, doctors may run a number of tests. These may include:
  • CT scan or sonogram, to check for inflammation of liver and/or spleen
  • X-ray imaging, to check for changes in the wrists, spine, feet, or finger joints
  • Echocardiogram, to look for inflammation of the lining of the heart or lungs
  • Blood tests, including those to examine white and red blood cell counts, and liver function

Treatment of adult Still's disease

Once your rheumatologist has diagnosed adult Still's disease, there are effective treatments available to help you manage the symptoms. While there is no known cure for adult Still's disease, treatments are available, and your rheumatologist is the best person to discuss these with and formulate a treatment plan to address all aspects of adult Still's disease.

There are several groups of medications which are used to treat adult Still's disease. The type of medication you take will depend on your symptoms, the severity of your disease, and any side effects you experience. These groups of medications include:
  • Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (for example Advil® or Motrin IB®), naproxen (or Naprosyn®), diclofenac (or Voltaren® and Arthrotec®). These drugs are often used to control mild symptoms.
  • Corticosteroids, such as prednisone may be used to control high fever spikes, severe joint swelling and pain, and complications with internal organs.
  • Methotrexate, a disease-modifying anti-rheumatic drug (DMARD), is often used in the treatment of adult Still's disease.
  • Biologic response modifiers, including anakinra (Kineret ), infliximab (Remicade ), and etanercept (Enbrel ), have been used with success in a number of patients; however, because the number of people with adult Still's disease is so small, large-scale tests have not yet been done to establish how well these drugs work in patients with this disease.

People with adult Still's disease are at high risk for joint destruction, caused by chronic inflammation. In severe cases, joint replacement surgery may be required. It is very important for people living with adult Still's disease to keep to their medication regime, as minimizing inflammation is key to preventing joint destruction.

In some people, adult Stills disease clears up on its own within one year, or after only a single episode; for others, it can be a chronic, on-going condition for years, which can cause debilitating arthritic symptoms, and even death. Others will experience periodic flares of disease activity, with periods of remission in between.

As with any form of arthritis, maintaining a healthy lifestyle is a critical part of any treatment plan for adult Still's disease. Poor diet, lack of exercise, and high levels of stress may make disease activity worse, so healthy eating, appropriate levels of aerobic and strengthening exercise, and relaxation are highly recommended.