Adult Still Disease
Adult Still disease is a rare type of inflammatory arthritis. Common symptoms are fevers, rash and joint pain. The condition can occur in some people as a single episode that goes away. In other people, the condition doesn’t go away, or it goes away but comes back.
Adult Still disease can damage joints, particularly the wrists. Treatment involves medicine to reduce pain and help control the disease. Prednisone is often used if pain relievers such as ibuprofen (Advil, Motrin IB, others) are not enough.
Most people with adult Still disease have a combination of the following symptoms:
- Fever. Fever may rise to at least 102 degrees Fahrenheit (38.9 degrees Celsius). The fever may spike once or twice a day for a week or longer.
- Rash. A rash might come and go with the fever. The rash usually appears on the trunk, arms or legs.
- Sore throat. This is one of the first symptoms of adult Still disease. The lymph nodes in the neck might be swollen and tender.
- Achy and swollen joints. Joints — especially in the knees and wrists— might be stiff, painful and inflamed. Ankles, elbows, hands and shoulders also might ache. The joint discomfort usually lasts at least two weeks.
- Muscle pain. Muscular pain usually comes and goes with the fever. The pain can be severe enough to disrupt daily activities.
Symptoms of this disorder can differ from person to person. They can mimic those of other conditions, including lupus and a type of cancer called lymphoma.
When to see a doctor
If you have a high fever, rash and achy joints, see your health care provider. Also, if you have adult Still disease and develop a cough, difficulty breathing, chest pain or any other symptoms that are not usual, call your health care provider.
The cause of adult Still disease is not known. Some researchers suspect it might be triggered by a viral or bacterial infection.
Age is the main risk factor for adult Still disease. It is most likely to occur in adults between the ages of 15 and 25 and between the ages of 36 and 46. Males and females are equally at risk.
Adult Still disease inflames the organs and joints. Most complications from the disease result from this inflammation.
- Joint destruction. The chronic swelling and irritation that occurs with adult Still disease can damage the joints. The most commonly involved joints are the knees and wrists. Sometimes other joints, including the neck, foot, finger and hip joints, also are affected.
- Inflammation of the heart. Adult Still disease can inflame the saclike covering of the heart, called the pericardium. This results in inflammation of the pericardium, called pericarditis. The disease can also inflame the muscular part of the heart, called the myocardium. This results in inflammation of the myocardium, called myocarditis.
- Excess fluid around the lungs. Inflammation may cause fluid to build up around the lungs. When this happens, it can be hard to take deep breaths.
- Macrophage activation syndrome. This is a rare but serious complication of adult Still disease. It happens when the immune system goes into overdrive and potentially harms organs such as the heart, liver, spleen and kidneys.
No single test identifies adult Still disease. Imaging tests can reveal damage caused by the disease. Blood tests can help rule out other conditions that have similar symptoms.
A variety of medicines are used to treat adult Still disease. The type of medicine depends on how bad the symptoms are and possible side effects.
- Nonsteroidal anti-inflammatory drugs (NSAIDs). Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin IB, others) or naproxen sodium (Aleve), may help with mild joint pain and inflammation. Stronger NSAIDs are available by prescription. Since NSAIDs can damage the liver, regular blood tests may be needed to check liver function.
- Steroids. Many people who have adult Still disease require treatment with steroids, such as prednisone. These powerful drugs reduce inflammation. They may lower the body’s resistance to infections and increase the risk of developing osteoporosis and diabetes.
- Methotrexate. The medicine methotrexate (Trexall) is often used in combination with prednisone. The prednisone dose is reduced when combined with methotrexate.
- Biologic response modifiers. If other medicines haven’t worked, your health care provider may recommend a biologic response modifier. Biologic response modifiers are medicines that block proteins causing inflammation. These medicines are often referred to as biologics. Anakinra (Kineret), canakinumab (Ilaris) and tocilizumab (Actemra) are some biologics that are used to treat adult Still disease. Other biologics that may be helpful for treating adult Still disease include etanercept (Enbrel), infliximab (Remicade), adalimumab (Humira) and rituximab (Rituxan).
References
- Adult Still’s disease. Arthritis Foundation. http://www.arthritis.org/about-arthritis/types/adult-onset-stills-disease/. Accessed Feb. 10, 2023.
- Mandl LA, et al. Clinical manifestations and diagnosis of adult Still’s disease. https://www.uptodate.com/contents/search. Accessed Feb. 10, 2023.
- Mandl LA, et al. Treatment of adult Still’s disease. https://www.uptodate.com/contents/search. Accessed Feb. 10, 2023.
- Adult-onset Still’s disease. National Center for Advancing Translational Sciences. https://rarediseases.info.nih.gov/diseases/436/adult-onset-stills-disease. Accessed Feb. 10, 2023.
- Exercise and arthritis. American College of Rheumatology. http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Living-Well-with-Rheumatic-Disease/Exercise-and-Arthritis. Accessed Feb. 10, 2023.
- Macovei LA, et al. Adult-onset Still’s Disease: A complex disease, a challenging treatment. International Journal of Molecular Science. 2022; doi:10.3390/ijms232112810.
- Glucocorticoid-induced osteoporosis. Endocrine Society. https://www.endocrine.org/patient-engagement/endocrine-library/glucocorticoid-induced-osteoporosis. Accessed Feb. 10, 2023.
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