About Adult Still's Disease
Adult Still's disease, also known as adult-onset Still’s disease or Wissler-Fanconi syndrome, is a rare form of arthritis which is characterized by high fevers, inflammation of the joints, and a salmon-coloured rash on the skin1. Adult Still's disease shares similar traits and symptoms to systemic juvenile idiopathic arthritis2.
Who can develop adult Still's disease?
A fairly rare form of arthritis, adult Still's disease affects less than 1 in 100,000 adults. Adult Still's disease affects men and women in approximately equal numbers. Though it can occur in adults of all ages, it tends to strike two age groups most commonly: those from age 15 - 25, and those from age 36 - 463.
The cause of adult Still's disease syndrome remains unknown, although scientists think that it may be the result of an environmental trigger such as a prior viral or bacterial infection, ultraviolet light exposure, toxins, stress, among others. Researchers are doubtful that there is a genetic connection as the disease rarely affects members of the same family.
Main symptoms of adult Still's disease
Adult Still's disease has several warning signs, which may be present at the onset of disease. These include4:
Getting a diagnosis of adult Still's disease
The first step in the process of diagnosing adult Still's disease is eliminating the possibility of other diseases that have similar symptoms. If a doctor believes that a person may have adult Still's disease, they will usually be referred to a rheumatologist – a specialist in the treatment of arthritis. Rheumatologists have many years of extra training on top of their regular medical schooling and are experts at diagnosing and treating all forms of arthritis, including adult Still's disease.
To get a firm diagnosis of adult Still's disease, doctors may run a number of tests. These may include4:
Once your rheumatologist has diagnosed adult Still's disease, there are effective treatments available to help you manage the symptoms. While there is no known cure for adult Still's disease, treatments are available, and your rheumatologist is the best person to discuss these with, and together with you, can create a tailored treatment plan to address all aspects of your lifestyle. It is important to discuss traditional healing practices and alternative medications you may want to incorporate in your treatment plan with your rheumatologist to ensure that it is aligned with your needs, preferences, and values.
Medications5
There are several groups of medications which are used to treat adult Still's disease. The type of medication you take will depend on your symptoms, the severity of your disease, and any side effects you may experience. These groups of medications include5:
Exercise6
Keeping active is important for your joints. Exercise is also good for improving mood and reducing fatigue. Though you may not feel like you have the energy to exercise, doing a small amount can make a big difference. Start with a daily walk around the block or 10 squats during commercial break and gradually increase the amount you do. Remember to choose an activity that you enjoy doing. If motivation is low, join a local fitness class and call up a friend. Always plan for a variety of back ups in case life gets in the way.
Joint protection and pacing6
Fatigue is a common symptom of adult Still's disease. Learning to protect joints and pace activities can be helpful. When possible, try not to over work the joints, take breaks often and avoid movements that cause discomfort. Changing position and stretching regularly may also be helpful. However, these strategies do require some intentional planning. An occupational therapist or physical therapist may also be able to provide tailored guidance and support.
Key take-aways
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Who can develop adult Still's disease?
A fairly rare form of arthritis, adult Still's disease affects less than 1 in 100,000 adults. Adult Still's disease affects men and women in approximately equal numbers. Though it can occur in adults of all ages, it tends to strike two age groups most commonly: those from age 15 - 25, and those from age 36 - 463.
The cause of adult Still's disease syndrome remains unknown, although scientists think that it may be the result of an environmental trigger such as a prior viral or bacterial infection, ultraviolet light exposure, toxins, stress, among others. Researchers are doubtful that there is a genetic connection as the disease rarely affects members of the same family.
Main symptoms of adult Still's disease
Adult Still's disease has several warning signs, which may be present at the onset of disease. These include4:
- High (40 degrees Celsius or higher) spiking fever, often occurring in the late afternoon or early evening; rarely, fevers can occur twice a day
- Joint pain and inflammation
- Faint, salmon-coloured rash, which may be bumpy or flat and will often occur with fever
- Muscle pain, which often comes and goes with fever and may be severe
- Sore throat
- Swollen lymph nodes in the neck
- Enlarged liver and/or spleen
- Poor appetite, nausea, and weight loss
Getting a diagnosis of adult Still's disease
The first step in the process of diagnosing adult Still's disease is eliminating the possibility of other diseases that have similar symptoms. If a doctor believes that a person may have adult Still's disease, they will usually be referred to a rheumatologist – a specialist in the treatment of arthritis. Rheumatologists have many years of extra training on top of their regular medical schooling and are experts at diagnosing and treating all forms of arthritis, including adult Still's disease.
To get a firm diagnosis of adult Still's disease, doctors may run a number of tests. These may include4:
- CT scan or sonogram, to check for inflammation of liver and/or spleen
- X-ray imaging, to check for changes in the wrists, spine, feet, or finger joints
- Echocardiogram, to look for inflammation of the lining of the heart or lungs
- Blood tests, including those to examine white and red blood cell counts, and liver function
Once your rheumatologist has diagnosed adult Still's disease, there are effective treatments available to help you manage the symptoms. While there is no known cure for adult Still's disease, treatments are available, and your rheumatologist is the best person to discuss these with, and together with you, can create a tailored treatment plan to address all aspects of your lifestyle. It is important to discuss traditional healing practices and alternative medications you may want to incorporate in your treatment plan with your rheumatologist to ensure that it is aligned with your needs, preferences, and values.
Medications5
There are several groups of medications which are used to treat adult Still's disease. The type of medication you take will depend on your symptoms, the severity of your disease, and any side effects you may experience. These groups of medications include5:
- Non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (for example Advil® or Motrin IB®), naproxen (or Naprosyn®), diclofenac (or Voltaren® and Arthrotec®). These drugs are often used to control mild symptoms.
- Corticosteroids, such as prednisone may be used to control high fever spikes, severe joint swelling and pain, and complications with internal organs.
- Methotrexate, a disease-modifying anti-rheumatic drug (DMARD), is often used in the treatment of adult Still's disease.
- Biologic response modifiers, including anakinra (Kineret®), infliximab (Remicade®, Avsola®, Inflectra®, Renflexis®, Remsima®) and etanercept (Enbrel®, Brenzys®, Erelzi®), have been used with success in a number of patients.
Exercise6
Keeping active is important for your joints. Exercise is also good for improving mood and reducing fatigue. Though you may not feel like you have the energy to exercise, doing a small amount can make a big difference. Start with a daily walk around the block or 10 squats during commercial break and gradually increase the amount you do. Remember to choose an activity that you enjoy doing. If motivation is low, join a local fitness class and call up a friend. Always plan for a variety of back ups in case life gets in the way.
Joint protection and pacing6
Fatigue is a common symptom of adult Still's disease. Learning to protect joints and pace activities can be helpful. When possible, try not to over work the joints, take breaks often and avoid movements that cause discomfort. Changing position and stretching regularly may also be helpful. However, these strategies do require some intentional planning. An occupational therapist or physical therapist may also be able to provide tailored guidance and support.
Key take-aways
- Be aware of the warning signs of Adult Still's Disease. Speak to your doctor if you experience high fevers, inflammation of the joints, and a salmon-coloured rash on the skin.
- The type of medication you take will depend on your symptoms, the severity of your disease, and any side effects you may experience.
- Minimizing inflammation is key to preventing joint destruction.
- Learning to protect joints and pace activities can be helpful in managing adult Still’s disease.
| 1 | Clinical manifestations and diagnosis of adult-onset Still’s disease https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-adult-onset-stills-disease |
| 2 | Nigrovic et al. (2018) Genetics and the classification of arthritis in adults and children. 2018;70(1):7. doi:10.1002/ART.40350 https://pubmed.ncbi.nlm.nih.gov/29024575/ |
| 3 | Magadur- et al (1995). Epidemiology of adult Still’s disease: Estimate of the incidence by a retrospective study in West France. doi:10.1136/ard.54.7.587 https://ard.bmj.com/content/54/7/587 |
| 4 | Adult-Onset Still’s Disease - NORD (National Organization for Rare Disorders). https://rarediseases.org/rare-diseases/adult-onset-stills-disease/ |
| 5 | Jamilloux et al. (2014). Treatment of adult-onset still’s disease: A review. doi:10.2147/TCRM.S64951 https://pubmed.ncbi.nlm.nih.gov/25653531/ |
| 6 | Versus Arthritis |
Arthritis Consumer Experts
ACE thanks Arthritis Research Canada (ARC) for its scientific review of ACE and JointHealth™ information and education programs.