Adult Still disease
Still's disease - adult; Adult-onset Still's disease; AOSD; Wissler-Fanconi syndromeAdult 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).
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.
Complete blood count
A complete blood count (CBC) test measures the following:The number of white blood cells (WBC count)The number of red blood cells (RBC count)The numb...
Read Article Now Book Mark Article - C-reactive protein (CRP), a measure of inflammation, will be higher than normal.
C-reactive protein
C-reactive protein (CRP) is produced by the liver. The level of CRP rises when there is inflammation in the body. It is one of a group of proteins,...
Read Article Now Book Mark Article - ESR (sedimentation rate), a measure of inflammation, will be higher than normal.
ESR
ESR stands for erythrocyte sedimentation rate. It is commonly called a "sed rate. "It is a test that indirectly measures the level of certain protei...
Read Article Now Book Mark Article - Ferritin level will be very high.
Ferritin
The ferritin blood test measures the level of ferritin in the blood. Ferritin is a protein inside your cells that stores iron. It allows your body ...
Read Article Now Book Mark Article - Fibrinogen level will be high.
- Liver function tests will show high levels of AST and ALT.
Liver function tests
Liver function tests are common tests that are used to see how well the liver is working. Tests include:AlbuminAlpha-1 antitrypsinAlkaline phosphata...
Read Article Now Book Mark ArticleAST
The aspartate aminotransferase (AST) blood test measures the level of the enzyme AST in the blood.
Read Article Now Book Mark ArticleALT
The alanine transaminase (ALT) blood test measures the level of the enzyme ALT in the blood.
Read Article Now Book Mark Article - Rheumatoid factor and ANA test will be negative.
Rheumatoid factor
Rheumatoid factor (RF) is a blood test that measures the amount of the RF antibody in the blood.
Read Article Now Book Mark ArticleANA
The antinuclear antibody panel is a blood test that looks at antinuclear antibodies (ANA). ANA are antibodies produced by the immune system that bind...
Read Article Now Book Mark Article - 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
Abdominal ultrasound
Abdominal ultrasound is a type of imaging test. It is used to look at organs in the abdomen, including the liver, gallbladder, spleen, pancreas, and...
Read Article Now Book Mark Article - CT scan of the abdomen
CT scan of the abdomen
An abdominal CT scan is an imaging method. This test uses x-rays to create cross-sectional pictures of the belly area. CT stands for computed tomog...
Read Article Now Book Mark Article - 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.
Arthritis
Arthritis is inflammation or degeneration of one or more joints. A joint is the area where 2 bones meet. There are more than 100 different types of...
Read Article Now Book Mark ArticlePrednisone 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
Pericarditis
Pericarditis is a condition in which the sac-like covering around the heart (pericardium) becomes inflamed.
Read Article Now Book Mark Article - Pleural effusion
Pleural effusion
A pleural effusion is a buildup of fluid between the layers of tissue that line the lungs and chest cavity.
Read Article Now Book Mark Article - 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.
Cough
Coughing is an important way to keep your throat and airways clear. But too much coughing may mean you have a disease or disorder. Some coughs are d...
Read Article Now Book Mark ArticleDifficulty breathing
Breathing difficulty may involve:Difficult breathing Uncomfortable breathingFeeling like you are not getting enough air
Read Article Now Book Mark ArticlePrevention
There is no known prevention.
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/.
- Lyme disease and related tick-borne infections - InDepth(In-Depth)
- Sickle cell disease - InDepth(In-Depth)
- Atherosclerosis(Alt. Medicine)
- Coronary artery disease - InDepth(In-Depth)
- Parkinson disease - InDepth(In-Depth)
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.