Still's disease - adult; Adult-onset Still's disease; AOSD; Wissler-Fanconi syndrome
Adult Still disease (ASD) is a rare illness that causes high fevers, rash, and joint pain. It may lead to long-term (chronic) arthritis.
Adult Still disease is a severe version of juvenile idiopathic arthritis (JIA), which occurs in children. Adults can have the same condition, although it is much less common. It is also called adult-onset Still disease (AOSD).
Fewer than 1 out of 100,000 people develop ASD each year. It affects women more often than men.
The cause of adult Still disease is unknown. No risk factors for the disease have been identified.
Almost all people with the disease will have fever, joint pain, sore throat, and a rash.
Additional symptoms include:
The spleen or liver may become swollen. Lung and heart inflammation may also occur.
AOSD can only be diagnosed after many other diseases (such as infections and cancer) are ruled out. You may need many medical tests before a final diagnosis is made.
A physical exam may show a fever, rash, and arthritis. The health care provider will use a stethoscope to listen for changes in the sound of your heart or lungs.
The following blood tests can be helpful in diagnosing adult Still disease:
Other tests may be needed to check for inflammation of the joints, chest, liver, and spleen:
The goal of treatment for adult Still disease is to control the symptoms of arthritis. Aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, are most often used first.
Prednisone may be used for more severe cases.
If the disease is severe or persists for a long time (becomes chronic), medicines that suppress the immune system might be needed. Such medicines include:
In many people, symptoms may come back several times over the next few years.
Symptoms continue for a long time (chronic) in about one third of people with adult Still disease.
A rare form of the disease, called macrophage activation syndrome, can be very severe with high fevers, severe illness and low blood cell counts. The bone marrow is involved and biopsy is needed to make the diagnosis.
Other complications may include:
Call your provider if you have symptoms of adult Still disease.
There is no known prevention.
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Gerfaud-Valentin M, Maucort-Boulch D, Hot A, et al. Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Medicine (Baltimore). 2014;93(2):91-99. PMID: 24646465 www.ncbi.nlm.nih.gov/pubmed/24646465.
Kaneko Y, Kameda H, Ikeda K, et al. Tocilizumab in patients with adult-onset still's disease refractory to glucocorticoid treatment: a randomised, double-blind, placebo-controlled phase III trial. Ann Rheum Dis. 2018;77(12):1720-1729. PMID: 30279267 www.ncbi.nlm.nih.gov/pubmed/30279267.
National Organization for Rare Disorders website. Rare diseases.org. Adult onset Still's disease. rarediseases.org/rare-diseases/adult-onset-stills-disease/. Accessed March 30, 2019.
Ortiz-Sanjuán F, Blanco R, Riancho-Zarrabeitia L, et al. Efficacy of anakinra in refractory adult-onset Still's disease: multicenter study of 41 patients and literature review. Medicine (Baltimore). 2015;94(39):e1554. PMID: 26426623 www.ncbi.nlm.nih.gov/pubmed/26426623.BACK TO TOP
Review Date: 1/10/2019
Reviewed By: Gordon A. Starkebaum, MD, MACR, ABIM Board Certified in Rheumatology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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