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What to know about adult-onset Still' s diseaseMedically reviewed by Nancy Carteron, M.D., FACR — By Rachel Ann Tee-Melegrito on September 22, 2022Adult-onset Still’s disease (AOSD) refers to a rare inflammatory condition that affects multiple organs and usually presents in young adults. The symptoms typically include fever, rash, and joint pain.
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Lucas Martinez 3 minutes ago
AOSD describes a rare systemic inflammatory condition that affects adults. It is similar to systemic...
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Elijah Patel Member
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AOSD describes a rare systemic inflammatory condition that affects adults. It is similar to systemic juvenile idiopathic arthritis (SJIA), which is a serious type of juvenile idiopathic arthritis (JIA).
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Aria Nguyen Member
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However, the distinguishing difference, as the names suggest, is that AOSD occurs in adults, whereas SJIA develops before the age of 16 years. The name derives from Sir George Frederic Still, who discovered the condition.
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Hannah Kim 2 minutes ago
In this article, we discuss adult-onset Still’s disease in more detail, including its potential ca...
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Ella Rodriguez 11 minutes ago
The cause of the condition is unknown, but it results in characteristic symptoms that include fever,...
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James Smith Moderator
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In this article, we discuss adult-onset Still’s disease in more detail, including its potential causes, symptoms, and treatments. Definition
Share on PinterestLeventKonuk/Getty ImagesAOSD is a rare auto-inflammatory disease that affects the entire body.
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Charlotte Lee 10 minutes ago
The cause of the condition is unknown, but it results in characteristic symptoms that include fever,...
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Ella Rodriguez Member
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The cause of the condition is unknown, but it results in characteristic symptoms that include fever, a pink-colored rash, and joint pain. AOSD presents in adulthood, with most cases beginning between 16 and 35 years of age.
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Harper Kim 16 minutes ago
There are two peaks in the age of onset: 15–25 years and 36–46 years. Evidence suggests that AOS...
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Sophia Chen 12 minutes ago
However, some health experts also use this term to describe individuals with SJIA who have reached a...
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Noah Davis Member
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There are two peaks in the age of onset: 15–25 years and 36–46 years. Evidence suggests that AOSD has an estimated incidence of 0.14–0.40 cases per 100,000 people and a prevalence of 1–34 cases per 1 million people. Doctors typically diagnose AOSD when certain symptoms develop in adulthood.
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Lily Watson 6 minutes ago
However, some health experts also use this term to describe individuals with SJIA who have reached a...
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However, some health experts also use this term to describe individuals with SJIA who have reached adulthood. Symptoms
The symptoms, course, and severity of AOSD vary from person to person.
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Noah Davis 11 minutes ago
The condition may appear suddenly and disappear. A person may develop and experience a single episod...
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In other people, the condition persists. The three hallmark symptoms present in 75–95% of those af...
The condition may appear suddenly and disappear. A person may develop and experience a single episode and then have no symptoms for several years.
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Chloe Santos 10 minutes ago
In other people, the condition persists. The three hallmark symptoms present in 75–95% of those af...
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Harper Kim Member
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In other people, the condition persists. The three hallmark symptoms present in 75–95% of those affected are:Fever: A person may experience a fever higher than 39°C that recurs daily and returns to normal between spikes. It often occurs later in the day and precedes other symptoms.Rashes: A visible symptom of AOSD is a pink or salmon-colored maculopapular rash that is not itchy.
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Ava White Moderator
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The rash predominantly appears on the trunk and parts of the limbs near the trunk. Heat or rubbing the skin can induce the rash.Joint pain: Initially, joint pain may be mild and transient, affecting fewer than five joints. Commonly involved joints include the knees, wrists, and ankles.
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However, it may progress and become severe and destructive, affecting multiple joints, including sma...
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Christopher Lee Member
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However, it may progress and become severe and destructive, affecting multiple joints, including small ones. Other common symptoms include:muscle pain that gets worse with fever spikespharyngitisa sore throat that recurs during disease flaresswollen lymph nodesan enlarged spleen
Meanwhile, less common symptoms may include:an enlarged liverinflammation of the outer lining of the lungsinflammation of heart tissueabdominal pain
The course of AOSD occurs in three main patterns:Monophasic: This refers to a single episode of symptoms that may last from weeks to months but persists for less than a year.Polyphasic, or intermittent: This describes when a person develops more than one episode of symptoms. The individual may be symptom-free for weeks to years between episodes, and subsequent episodes tend to be shorter and less severe.Chronic: In the chronic form, the symptoms persist over time.
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Ethan Thomas Member
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Causes and risk factors
At present, researchers are unsure of the cause of AOSD and refer to the condition as idiopathic. However, they believe that a combination of genetic factors and an infection that acts as a trigger may play a role. Some evidence suggests that genes that produce proteins to help mediate inflammation likely play a role in the development of the condition.
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Christopher Lee Member
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For example, genes such as IL1A and TNFRSF1A provide instructions for making proteins called interleukin-1 alpha and tumor necrosis factor receptor 1, respectively. High activity of these proteins is common in those with AOSD. Similarly, certain infectious agents may trigger or exacerbate symptoms of AOSD.
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Sophia Chen 50 minutes ago
These may include:Yersinia enterocoliticaMycoplasma pneumoniaecytomegalovirus (CMV)
As such, AOSD ma...
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Grace Liu 28 minutes ago
The symptoms typically present before the age of 40 years, and the conditions may be slightly more p...
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Ethan Thomas Member
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These may include:Yersinia enterocoliticaMycoplasma pneumoniaecytomegalovirus (CMV)
As such, AOSD may be a reactive condition in which certain infections act as triggers in a genetically predisposed individual. Currently, research suggests there are no known risk factors, and there is usually no family history.
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The symptoms typically present before the age of 40 years, and the conditions may be slightly more p...
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A person will receive a diagnosis if they meet five or more criteria, including at least two of the ...
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The symptoms typically present before the age of 40 years, and the conditions may be slightly more prevalent in females. Diagnosis
There is no single test to distinguish AOSD from other conditions. A doctor will typically diagnose AOSD through:a comprehensive clinical evaluation and history takingidentification of characteristic findingsruling out other possible conditionsblood tests and imaging tests
Doctors may use various imaging tests, such as:an X-ray or MRI scan to see organ, bone, and joint damage, including narrowing of the wrist joint spacean ultrasound to check for liver or spleen enlargementan echocardiogram to detect inflammation of the pericardium or myocardium of the heart
They may also carry out several blood tests, including:blood tests for inflammatory markers, such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)complete blood count (CBC)serum ferritin levels, which are often more than five times higher than the upper limit of normal in people with AOSDliver enzyme levelsrheumatoid factor (RF) and antinuclear antibody (ANA) panel to rule out other rheumatic conditions such as rheumatoid arthritis
There are different classification criteria for AOSD, but the Yamaguchi criteria is the most commonly used and has the highest sensitivity.
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A person will receive a diagnosis if they meet five or more criteria, including at least two of the ...
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A person will receive a diagnosis if they meet five or more criteria, including at least two of the major criteria. The major criteria are:having a high fever of at least 39°C for at least 1 weekjoint pain or arthritis for at least 2 weeksa non-itchy salmon-colored rash on the trunk or limbsan elevated white blood cell count of at least 10,000 cells per microliter
The minor criteria are:a sore throatswollen lymph nodesa swollen liver or spleenunusual liver function test resultsnegative test results for RF and ANA
Treatment
The aims of treatment are:reducing inflammationcontrolling symptomspreventing end organ damage
Doctors may prescribe or recommend various medications to treat AOSD.
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These may include: over-the-counter or prescription nonsteroidal anti-inflammatory drugs (NSAIDs)cor...
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These steps include:Engaging in regular physical activity: Exercising and staying active can help ma...
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These may include: over-the-counter or prescription nonsteroidal anti-inflammatory drugs (NSAIDs)corticosteroids, such as prednisonedisease-modifying drugs (DMARDs) such as methotrexate, often in combination with corticosteroids to lower the associated side effectsbiologics, including:interleukin-1 (IL-1) inhibitors, such as canakinumab (Ilaris)IL-6 inhibitors, such as tocilizumab (Actemra)tumor necrosis factor-alpha inhibitors, such as infliximab (Remicade)
A person may still need to take these medications even after the symptoms go away. This maintenance therapy can keep inflammation under control and prevent further bone, cartilage, and organ damage. Management
People can take steps to help manage the symptoms of AOSD.
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These steps include:Engaging in regular physical activity: Exercising and staying active can help ma...
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Summary
Adult-onset Still’s disease is a rare autoimmune inflammatory condition characterized by s...
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Dylan Patel Member
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These steps include:Engaging in regular physical activity: Exercising and staying active can help maintain the range of motion of the joints and keep the muscles strong. It can also help a person maintain a moderate weight, reducing strain on the joints.Adhering to a healthy eating pattern: Alongside exercise, a nutritious, balanced diet can help control a person’s weight, improve energy levels, and reduce fatigue.Reducing strain: Using joint protection techniques, pacing activities carefully, and including sufficient breaks in the day can help reduce the strain on joints.Improving mental well-being: Stress can worsen the symptoms of AOSD. Good sleep hygiene, relaxation techniques, talking therapies, and counseling can all help people manage their mental health.
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Summary
Adult-onset Still’s disease is a rare autoimmune inflammatory condition characterized by s...
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Chloe Santos 81 minutes ago
AOSD can have different patterns, meaning that some people only experience a single episode, whereas...
Summary
Adult-onset Still’s disease is a rare autoimmune inflammatory condition characterized by symptoms of fever, joint pain, and salmon-pink rashes. The condition develops in adulthood and is similar to systemic juvenile idiopathic arthritis, which occurs in childhood.
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Ava White 87 minutes ago
AOSD can have different patterns, meaning that some people only experience a single episode, whereas...
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However, medications, self-care, and lifestyle behaviors can help people manage the condition. A per...
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Joseph Kim Member
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AOSD can have different patterns, meaning that some people only experience a single episode, whereas others have persistent symptoms. Currently, there is no known cause and no set treatment protocol.
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However, medications, self-care, and lifestyle behaviors can help people manage the condition. A per...
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Last medically reviewed on September 22, 2022Immune System / VaccinesMedically reviewed by Nancy Car...
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However, medications, self-care, and lifestyle behaviors can help people manage the condition. A person should talk with a doctor to identify the best treatment and management plan for their symptoms.
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Last medically reviewed on September 22, 2022Immune System / VaccinesMedically reviewed by Nancy Car...
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Last medically reviewed on September 22, 2022Immune System / VaccinesMedically reviewed by Nancy Carteron, M.D., FACR — By Rachel Ann Tee-Melegrito on September 22, 2022
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