What is the definition of Adult Still's Disease?

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).

What are the alternative names for Adult Still's Disease?

Still's disease - adult; Adult-onset Still's disease; AOSD; Wissler-Fanconi syndrome

What are the causes for Adult Still's Disease?

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.

What are the symptoms for Adult Still's Disease?

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 several hours.
• 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.

What are the current treatments for Adult Still's Disease?

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)

What is the outlook (prognosis) for Adult Still's Disease?

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.

What are the possible complications for Adult Still's 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:

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

When should I contact a medical professional for Adult Still's Disease?

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.

How do I prevent Adult Still's Disease?

There is no known prevention.

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.