Site Map

Adult Still disease

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).

I Would Like to Learn About:

Causes

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.

Symptoms

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.

Exams and Tests

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:

Treatment

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:

Outlook (Prognosis)

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.

Possible Complications

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:

When to Contact a Medical Professional

Call your provider if you have symptoms of adult Still disease.

If you have already been diagnosed with the condition, you should call your provider if you have a cough or difficulty breathing.

Prevention

There is no known prevention.

Related Information

Pericarditis
Pleural effusion

References

Alonso ER, Marques AO. Adult-onset still disease. In: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. 7th ed. Philadelphia, PA: Elsevier; 2019:chap 173.

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.

ADAM Quality Logo

A.D.A.M., Inc. is accredited by URAC, for Health Content Provider (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics. This site complies with the HONcode standard for trustworthy health information: verify here.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- 2021 A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.