Learn About Adult Still's Disease

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).

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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 of 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 of 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.

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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)
Who are the top Adult Still's Disease Local Doctors?
Elite
Highly rated in
18
conditions

Division Of Immunology

Rheumatology, Allergy And Rare Diseases, Irccs San Raffaele Hospital And Vita Salute San Raffaele University 
Milan, IT 

Giulio Cavalli is in Milan, Italy. Cavalli is rated as an Elite expert by MediFind in the treatment of Adult Still's Disease. He is also highly rated in 18 other conditions, according to our data. His top areas of expertise are Erdheim-Chester Disease, Non-Langerhans-Cell Histiocytosis, Histiocytosis, and Reticulohistiocytoma.

Elite
Highly rated in
40
conditions

Division Of Immunology

Rheumatology, Allergy And Rare Diseases 
Milan, IT 20132

Lorenzo Dagna is in Milan, Italy. Dagna is rated as an Elite expert by MediFind in the treatment of Adult Still's Disease. He is also highly rated in 40 other conditions, according to our data. His top areas of expertise are Erdheim-Chester Disease, Non-Langerhans-Cell Histiocytosis, Histiocytosis, and Reticulohistiocytoma.

 
 
 
 
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Elite
Highly rated in
25
conditions

Division Of Immunology

Rheumatology, Allergy And Rare Diseases, Irccs Ospedale San Raffaele & Vita Salute San Raffaele University 
Milan, IT 

Corrado Campochiaro is in Milan, Italy. Campochiaro is rated as an Elite expert by MediFind in the treatment of Adult Still's Disease. He is also highly rated in 25 other conditions, according to our data. His top areas of expertise are Adult Still's Disease, Scleroderma, Vasculitis, and Takayasu Arteritis.

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 of 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.

What are the latest Adult Still's Disease Clinical Trials?
Multi-center Registration Study of Adult Onset Still's Disease in Chinese Population
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Glycosylated Ferritin in Differential Diagnosis of Still's Disease, Sepsis and Other Macrophagic Activation Syndromes.
What are the Latest Advances for Adult Still's Disease?
Good result for twelve years after bilateral hip and knee arthroplasties for ankylosis with juvenile idiopathic arthritis: a case report.
The use of cyclophosphamide in the treatment of Still's disease - a case report.
Tired of the same old research?
Check Latest Advances
Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report.
Who are the sources who wrote this article ?

Published Date : January 31, 2021
Published By : Diane M. Horowitz, MD, Rheumatology and Internal Medicine, Northwell Health, Great Neck, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

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 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 May 10, 2021.

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/.