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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).

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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.

• Joint pain, warmth, and swelling are common. Most often, several joints are involved at the same time. Often, people with the condition have morning stiffness of joints that lasts for at least one hour.
• The fever comes on quickly once per day, most commonly in the afternoon or evening.
• The skin rash is often salmon-pink colored and comes and goes with the fever.

Additional symptoms include:

• Abdominal pain and swelling
• Pain when taking a deep breath (pleurisy)
• Sore throat
• Swollen lymph nodes (glands)
• Weight loss

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:

• Complete blood count (CBC), may show a high number of white blood cells (granulocytes) and reduced number of red blood cells.
• C-reactive protein (CRP), a measure of inflammation, will be higher than normal.
• ESR (sedimentation rate), a measure of inflammation, will be higher than normal.
• Ferritin level will be very high.
• Fibrinogen level will be high.
• Liver function tests will show high levels of AST and ALT.
• Rheumatoid factor and ANA test will be negative.
• Blood cultures and viral studies will be negative.

Other tests may be needed to check for inflammation of the joints, chest, liver, and spleen:

• Abdominal ultrasound
• CT scan of the abdomen
• X-rays of the joints, chest, or stomach area (abdomen)

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:

• Methotrexate
• Anakinra (interleukin-1 receptor agonist)
• Tocilizumab (interleukin 6 inhibitor)
• Tumor necrosis factor (TNF) antagonists such as etanercept (Enbrel)

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 potentially life threatening complication 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 bone marrow biopsy is needed to make the diagnosis.

Other complications may include:

• Arthritis in several joints
• Liver disease
• Pericarditis
• Pleural effusion
• Spleen enlargement

When to Contact a Medical Professional

Contact 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, Smolen JS, van der Heijde D, Weinblatt ME, Weisman MH, eds. Rheumatology. 8th ed. Philadelphia, PA: Elsevier; 2023: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 pubmed.ncbi.nlm.nih.gov/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 pubmed.ncbi.nlm.nih.gov/30279267/.

National Organization for Rare Disorders website. Rare diseases.org. Adult onset Still's disease. rarediseases.org/rare-diseases/adult-onset-stills-disease/. Updated 2021. Accessed March 3, 2023.

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 pubmed.ncbi.nlm.nih.gov/26426623/.

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Review Date: 1/25/2023  

Reviewed By: Neil J. Gonter, MD, Assistant Professor of Medicine, Columbia University, NY and private practice specializing in Rheumatology at Rheumatology Associates of North Jersey, Teaneck, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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