Table of ContentsView AllTable of ContentsWhat It IsSymptomsCausesWhen to See a DoctorTreatmentComplicationsPrognosis
Table of ContentsView All
View All
Table of Contents
What It Is
Symptoms
Causes
When to See a Doctor
Treatment
Complications
Prognosis
Still’s disease is a type of inflammatory arthritis that causes fevers, a salmon-pink rash, and inflammation of joints and sometimes internal organs. While there is no cure for this condition, medication and a healthy lifestyle can help reduce inflammation and manage symptoms.
Still’s disease is also called systemic-onset juvenile idiopathic arthritis (SJIA), as it was first diagnosed in children. SJIA is a type of juvenile idiopathic arthritis (JIA), a group of arthritis conditions affecting children. Still’s disease can also occur in adults. When it does, it is called adult-onset Still’s disease (AOSD).
This article discusses Still’s disease and its symptoms. It also covers treatment and prognosis.
What Is Still’s Disease?
Still’s disease is a rare type of inflammatory arthritis that can affect the whole body. It can cause joint pain along with whole-body symptoms like fever. There is an adult-onset form of the disease and a childhood form. The primary difference between the two conditions is the age when symptoms begin.
Prevalence
JIA affects one in 1,000 children.In total, the condition is thought to affect around 300,000 children in the United States.SJIA is believed to account for between 10% and 20% of children with JIA.
Adult-onset Still’s disease is rare. It is believed to affect fewer than one in every 100,000 people.It affects men and women equally, and most diagnoses are in young adults ages 16 to 35.The true number of people living with AOSD is unknown because the condition is easily mistaken for other types of inflammatory arthritis due to its rarity and variable symptomology.
What Does Still’s Disease Feel Like?
Still’s disease causes systemic (whole-body) symptoms in both adults and children. The most common symptoms of this condition include:
Still’s Disease in Children
Children may also develop arthritis in the spine, jaws, and hip joints. These are areas where SJIA can cause the most destruction and require joint replacement surgeries. The more joints that are affected, the more severe SJIA will be. About a quarter of the children with SJIAwill develop chronic polyarthritis(arthritis affecting five or more joints) continuing into adulthood.
Growth Issues in Children
SJIA can cause growth issues in children. When joints are severely affected, growth may be slowed, especially in the arms and legs. Sometimes, this is the disease itself and, other times, this is related to medications for treating the condition.
Rarer SymptomsAdditional, but rarer, symptoms of Still’s in children and adults include:Enlarged liver or spleenInflammation in the heart lining, a condition called pericarditisInflammation of the lungs, called pleuritisAbdominal pain and swellingPain with breathingWeight loss without any effort
Rarer Symptoms
Additional, but rarer, symptoms of Still’s in children and adults include:Enlarged liver or spleenInflammation in the heart lining, a condition called pericarditisInflammation of the lungs, called pleuritisAbdominal pain and swellingPain with breathingWeight loss without any effort
Additional, but rarer, symptoms of Still’s in children and adults include:
What Triggers Still’s Disease?
Other research suggests Still’s disease is an autoimmune disease.Autoimmune diseasesare conditions where the immune system attacks its own healthy tissues.
Neither theory about what may cause Still’s has been confirmed and the precise cause is still unknown.
Potential Causes of Autoimmune Diseases
It can be difficult to diagnose Still’s disease because it shares certain characteristics with other conditions, includinglupusandLyme disease.
It is important for people who have symptoms of Still’s to contact their doctors. If there are breathing troubles, seeking immediate medical attention is a priority.
Before making a diagnosis, other medical problems will need to be ruled out. A medical history and physical exam are usually the first steps. Blood work can check inflammation levels, blood cell counts, iron levels, and elevated liver enzymes. Imaging can look for joint damage, inflammation, enlargement of the liver or spleen, and fluid buildup in the lungs and chest cavity.
What Is the Best Treatment for Still’s Disease?
There is no cure for Still’s disease and there is no way to prevent it.Once a diagnosis has been made, the goal is to control symptoms and slow down the disease in order to prevent or lessen complications.
Early treatment usually involves prevention of inflammation using nonsteroidal anti-inflammatory drugs (NSAIDs), including ibuprofen and naproxen.High-dose aspirin is sometimes used for adult-onset Still’s disease but is not recommended in children with SJIA because of the high risk of toxicity, compared to other NSAIDs.
Corticosteroids, such as prednisone, can help when symptoms don’t respond to NSAIDs, but these medications cannot be used for long periods, due to side effects.
For people whose symptoms persist, immunosuppressant drugs are a second-line therapy. These medications affect the inflammatory response of the immune system and include medications such as methotrexate and hydroxychloroquine.
More recently, Still’s disease has been found to involve substances that cause inflammation, including a cytokine protein, interleukin.This knowledge has allowed doctors to start treating Still’s with biologic medications, including biologic injections such as infliximab (Remicade), adalimumab (Humira), etanercept (Enbrel), and Tocilizumab (Actemra), an intravenous treatment (through a vein using an intravenous drip).
Potential complications of Still’s disease include:
Still’s Disease Prognosis
While there is no cure for Still’s disease, it is treatable and manageable. Treatment can reduce inflammation and other symptoms. Some people may develop chronic arthritis, but medications can help. It is also important to practice a healthy life, which includes a balanced diet, exercise, not smoking, stress management, and getting enough rest.
What is the survival rate of Still’s disease?Most people with Still’s disease have a normal life expectancy. However, certain Still’s disease complications like macrophage activation syndrome can be life-threatening.
What is the survival rate of Still’s disease?
Most people with Still’s disease have a normal life expectancy. However, certain Still’s disease complications like macrophage activation syndrome can be life-threatening.
Summary
Still’s disease is a form of inflammatory arthritis that can cause joint pain and systemic symptoms like fever, rash, and muscle pain. The condition is most common in children, but there is also an adult-onset version.
There is no cure for Still’s disease, but it can be treated with pain medication, corticosteroids, and other medications. Most people with Still’s disease have a normal lifespan, but some of its complications can be life-threatening.
10 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Ramanan AV, Dick AD, Guly C, et al.Tocilizumab in patients with anti-TNF refractory juvenile idiopathic arthritis-associated uveitis (APTITUDE): a multicentre, single-arm, phase 2 trial.Lancet Rheumatol. 2020;2(3):e135-e141. doi:10.1016/S2665-9913(20)30008-4Pooni R, Sandborg C, Lee T.Building a viable telemedicine presence in pediatric rheumatology.Pediatr Clin North Am. 2020;67(4):641-645. doi:10.1016/j.pcl.2020.04.006Maller J, Fox E, Park KT, et al.Inflammatory bowel disease in children with systemic juvenile idiopathic arthritis.J Rheumatol. 2021;48(4):567-574. doi:10.3899/jrheum.200230Giacomelli R, Ruscitti P, Shoenfeld Y.A comprehensive review on adult onset Still’s disease.J Autoimmun. 2018;93:24-36. doi:10.1016/j.jaut.2018.07.018Mzabi A, Fakhfakh R, Thabet M, et al.Elderly-onset adult Still’s disease.Reumatologia. 2021;59(3):188-196. doi:10.5114/reum.2021.107323Arthritis Foundation.Juvenile idiopathic arthritis (JIA).Macovei LA, Burlui A, Bratoiu I, et al.Adult-onset Still’s disease-a complex disease, a challenging treatment.Int J Mol Sci. 2022;23(21). doi:10.3390/ijms232112810Anderson CW, Shah PA, Roberts JR.Adult-onset Still’s disease: Is this truly a diagnosis of exclusion?.Hawaii J Med Public Health. 2017;76(11 Suppl 2):3–6.Genetic and Rare Diseases Information Center (GARD).Adult-onset Still’s Disease.Mitrovic S, Fautrel B.Complications of adult-onset Still’s disease and their management.Expert Rev Clin Immunol. 2018;14(5):351-65. doi:10.1080/1744666X.2018.1465821Additional ReadingAutoinflammatory Alliance.Systemic juvenile idiopathic arthritis (SJIA).Beukelman T.Treatment advances in systemic juvenile idiopathic arthritis.F1000Prime Rep. 2014;6:21. doi:10.12703/P6-21Medline Plus.Adult Still disease.Mellins ED, Macubas CM, Grom AA.Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions.Nat Rev Rheumatol. 2011;7(7):416–426. doi:10.1038/nrrheum.2011.68
10 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Ramanan AV, Dick AD, Guly C, et al.Tocilizumab in patients with anti-TNF refractory juvenile idiopathic arthritis-associated uveitis (APTITUDE): a multicentre, single-arm, phase 2 trial.Lancet Rheumatol. 2020;2(3):e135-e141. doi:10.1016/S2665-9913(20)30008-4Pooni R, Sandborg C, Lee T.Building a viable telemedicine presence in pediatric rheumatology.Pediatr Clin North Am. 2020;67(4):641-645. doi:10.1016/j.pcl.2020.04.006Maller J, Fox E, Park KT, et al.Inflammatory bowel disease in children with systemic juvenile idiopathic arthritis.J Rheumatol. 2021;48(4):567-574. doi:10.3899/jrheum.200230Giacomelli R, Ruscitti P, Shoenfeld Y.A comprehensive review on adult onset Still’s disease.J Autoimmun. 2018;93:24-36. doi:10.1016/j.jaut.2018.07.018Mzabi A, Fakhfakh R, Thabet M, et al.Elderly-onset adult Still’s disease.Reumatologia. 2021;59(3):188-196. doi:10.5114/reum.2021.107323Arthritis Foundation.Juvenile idiopathic arthritis (JIA).Macovei LA, Burlui A, Bratoiu I, et al.Adult-onset Still’s disease-a complex disease, a challenging treatment.Int J Mol Sci. 2022;23(21). doi:10.3390/ijms232112810Anderson CW, Shah PA, Roberts JR.Adult-onset Still’s disease: Is this truly a diagnosis of exclusion?.Hawaii J Med Public Health. 2017;76(11 Suppl 2):3–6.Genetic and Rare Diseases Information Center (GARD).Adult-onset Still’s Disease.Mitrovic S, Fautrel B.Complications of adult-onset Still’s disease and their management.Expert Rev Clin Immunol. 2018;14(5):351-65. doi:10.1080/1744666X.2018.1465821Additional ReadingAutoinflammatory Alliance.Systemic juvenile idiopathic arthritis (SJIA).Beukelman T.Treatment advances in systemic juvenile idiopathic arthritis.F1000Prime Rep. 2014;6:21. doi:10.12703/P6-21Medline Plus.Adult Still disease.Mellins ED, Macubas CM, Grom AA.Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions.Nat Rev Rheumatol. 2011;7(7):416–426. doi:10.1038/nrrheum.2011.68
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Ramanan AV, Dick AD, Guly C, et al.Tocilizumab in patients with anti-TNF refractory juvenile idiopathic arthritis-associated uveitis (APTITUDE): a multicentre, single-arm, phase 2 trial.Lancet Rheumatol. 2020;2(3):e135-e141. doi:10.1016/S2665-9913(20)30008-4Pooni R, Sandborg C, Lee T.Building a viable telemedicine presence in pediatric rheumatology.Pediatr Clin North Am. 2020;67(4):641-645. doi:10.1016/j.pcl.2020.04.006Maller J, Fox E, Park KT, et al.Inflammatory bowel disease in children with systemic juvenile idiopathic arthritis.J Rheumatol. 2021;48(4):567-574. doi:10.3899/jrheum.200230Giacomelli R, Ruscitti P, Shoenfeld Y.A comprehensive review on adult onset Still’s disease.J Autoimmun. 2018;93:24-36. doi:10.1016/j.jaut.2018.07.018Mzabi A, Fakhfakh R, Thabet M, et al.Elderly-onset adult Still’s disease.Reumatologia. 2021;59(3):188-196. doi:10.5114/reum.2021.107323Arthritis Foundation.Juvenile idiopathic arthritis (JIA).Macovei LA, Burlui A, Bratoiu I, et al.Adult-onset Still’s disease-a complex disease, a challenging treatment.Int J Mol Sci. 2022;23(21). doi:10.3390/ijms232112810Anderson CW, Shah PA, Roberts JR.Adult-onset Still’s disease: Is this truly a diagnosis of exclusion?.Hawaii J Med Public Health. 2017;76(11 Suppl 2):3–6.Genetic and Rare Diseases Information Center (GARD).Adult-onset Still’s Disease.Mitrovic S, Fautrel B.Complications of adult-onset Still’s disease and their management.Expert Rev Clin Immunol. 2018;14(5):351-65. doi:10.1080/1744666X.2018.1465821
Ramanan AV, Dick AD, Guly C, et al.Tocilizumab in patients with anti-TNF refractory juvenile idiopathic arthritis-associated uveitis (APTITUDE): a multicentre, single-arm, phase 2 trial.Lancet Rheumatol. 2020;2(3):e135-e141. doi:10.1016/S2665-9913(20)30008-4
Pooni R, Sandborg C, Lee T.Building a viable telemedicine presence in pediatric rheumatology.Pediatr Clin North Am. 2020;67(4):641-645. doi:10.1016/j.pcl.2020.04.006
Maller J, Fox E, Park KT, et al.Inflammatory bowel disease in children with systemic juvenile idiopathic arthritis.J Rheumatol. 2021;48(4):567-574. doi:10.3899/jrheum.200230
Giacomelli R, Ruscitti P, Shoenfeld Y.A comprehensive review on adult onset Still’s disease.J Autoimmun. 2018;93:24-36. doi:10.1016/j.jaut.2018.07.018
Mzabi A, Fakhfakh R, Thabet M, et al.Elderly-onset adult Still’s disease.Reumatologia. 2021;59(3):188-196. doi:10.5114/reum.2021.107323
Arthritis Foundation.Juvenile idiopathic arthritis (JIA).
Macovei LA, Burlui A, Bratoiu I, et al.Adult-onset Still’s disease-a complex disease, a challenging treatment.Int J Mol Sci. 2022;23(21). doi:10.3390/ijms232112810
Anderson CW, Shah PA, Roberts JR.Adult-onset Still’s disease: Is this truly a diagnosis of exclusion?.Hawaii J Med Public Health. 2017;76(11 Suppl 2):3–6.
Genetic and Rare Diseases Information Center (GARD).Adult-onset Still’s Disease.
Mitrovic S, Fautrel B.Complications of adult-onset Still’s disease and their management.Expert Rev Clin Immunol. 2018;14(5):351-65. doi:10.1080/1744666X.2018.1465821
Autoinflammatory Alliance.Systemic juvenile idiopathic arthritis (SJIA).Beukelman T.Treatment advances in systemic juvenile idiopathic arthritis.F1000Prime Rep. 2014;6:21. doi:10.12703/P6-21Medline Plus.Adult Still disease.Mellins ED, Macubas CM, Grom AA.Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions.Nat Rev Rheumatol. 2011;7(7):416–426. doi:10.1038/nrrheum.2011.68
Autoinflammatory Alliance.Systemic juvenile idiopathic arthritis (SJIA).
Beukelman T.Treatment advances in systemic juvenile idiopathic arthritis.F1000Prime Rep. 2014;6:21. doi:10.12703/P6-21
Medline Plus.Adult Still disease.
Mellins ED, Macubas CM, Grom AA.Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions.Nat Rev Rheumatol. 2011;7(7):416–426. doi:10.1038/nrrheum.2011.68
Meet Our Medical Expert Board
Share Feedback
Was this page helpful?Thanks for your feedback!What is your feedback?OtherHelpfulReport an ErrorSubmit
Was this page helpful?
Thanks for your feedback!
What is your feedback?OtherHelpfulReport an ErrorSubmit
What is your feedback?
