Adult-onset Still’s disease
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Age of Onset
Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease
Autosomal recessive ?Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype
X-linked dominant ?X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder
Mitochondrial or multigenic ?Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor ?Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Other Names (AKA)
Adult Still's disease; Still's disease adult onset
Kidney and Urinary Diseases; Musculoskeletal Diseases
Adult-onset Still's disease (AOSD) is an inflammatory condition that affects multiple
Still's disease which occurs in children (under the age of 16) is known as systemic onset juvenile rheumatoid arthritis (JRA).
- High fever
- Joint pain
- Skin rash
- Enlarged spleen
- Enlarged lymph nodes
- High levels of ferritin in the blood
The first symptoms of AOSD usually occur in early adulthood and include high fevers, arthritis, and a skin rash. Symptoms may occur continually or over short periods of time. Arthritis can lead to permanent joint damage over time. Some people with AOSD have serious complications that include severe allergic reactions, abnormal blood clotting (disseminated intravascular coagulation), and
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
|Medical Terms||Other Names||
|80%-99% of people have these symptoms|
[ more ]
Elevated white blood count
High white blood count
Increased blood leukocyte number
[ more ]
Increased blood neutrophil counts
[ more ]
|Restrictive ventilatory defect||
Stiff lung or chest wall causing decreased lung volume
Increased spleen size
|30%-79% of people have these symptoms|
Pain in stomach
[ more ]
Generalized swelling of lymph nodes
Swollen lymph nodes affecting all regions of the body
[ more ]
[ more ]
Swelling or irritation of membrane around heart
Inflammation of tissues lining lungs and chest
|5%-29% of people have these symptoms|
|Abnormal circulating lipid concentration||0003119|
|Bone marrow hypocellularity||
Bone marrow failure
|Elevated hepatic transaminase||
High liver enzymes
Inflammation of heart muscle
Recurrent sore throat
Specialists that may be involved in the care of someone with adult-onset Still's disease include:
Orthopedist Rheumatologist Dermatologist
The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.
- Canakinumab(Brand name: Ilaris) Manufactured by Novartis
FDA-approved indication: ILARIS is indicated for the treatment of active Still’s disease, including Adult-Onset Still’s Disease (AOSD) and Systemic Juvenile Idiopathic
Arthritis(SJIA) in patients aged 2 years and older.
National Library of Medicine Drug Information Portal
Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.
Conditions with similar signs and symptoms from Orphanet
Many other inflammatory, neoplastic, and infectious conditions with a similar presentation must be ruled out in order to diagnose AOSD. Differential diagnoses include infections (endocarditis, occult infections, secondary syphilis, viral rash), malignancies (lymphoma) or autoimmune diseases (such as polyarteritis nodosa, vasculitis, or polymyositis). Genetic counseling (if relevant) AOSD is a sporadic non-inheritable disease and genetic counseling is therefore not needed.
Visit the Orphanet disease page for more information.
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
- MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
- The Arthritis Foundation has an information page on adult onset stills disease. Click on the link above to view the information page.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- Giacomelli R, Ruscitti P, Shoenfeld Y. A comprehensive review on adult onset Still's disease. J Autoimmun. 2018; 93:24-36. https://pubmed.ncbi.nlm.nih.gov/30077425.
- Ruscitti P, Giacomelli R. Pathogenesis of adult onset still's disease: current understanding and new insights. Expert Rev Clin Immunol. 2018; 14(11):965-976. https://pubmed.ncbi.nlm.nih.gov/30295095.
- Wang MY, Jia JC, Yang CD, Hu QY. Pathogenesis, disease course, and prognosis of adult-onset Still's disease: an update and review. Chin Med J (Engl). 2019; 132(23):2856-2864. https://pubmed.ncbi.nlm.nih.gov/31856058.
- Mimura T, Kondo Y, Ohta A, et al. Evidence-based clinical practice guideline for adult Still's disease. Mod Rheumatol. 2018; 28(5):736-757. https://pubmed.ncbi.nlm.nih.gov/2965190.
- Yoo DH. Biologics for the treatment of adult-onset still's disease. Expert Opin Biol Ther. 2019; 19(11):1173-1190. https://pubmed.ncbi.nlm.nih.gov/31379214.
- Park EH, Lee EY, Shin K, Kim HA. Tocilizumab-induced anaphylaxis in patients with adult-onset Still's disease and systemic juvenile idiopathic arthritis: a case-based review. Rheumatol Int. 2020; 40(5):791-798. https://pubmed.ncbi.nlm.nih.gov/31598752.