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Adult-onset Still's disease

Dr. Nadheer K M (AIIMS)MBBS

December 07, 2020

December 07, 2020

Adult-onset Still's disease
Adult-onset Still's disease

Adult-onset Still's disease (also adult Still's disease) is a disorder characterized by sudden spikes of high fever, rashes and pain in the joints. It is named after Sir George F. Still, an English physician.

Even though the condition was first described in children, it occurs in adults and is called AOSD or adult-onset Still’s disease.

It is characterised by joint inflammation that can particularly impact the wrist joint. (Read more: Joint pain)

Read on to know about the symptoms, causes, diagnosis and treatment of adult-onset Still's disease.

Adult-onset Still's disease symptoms

Still's disease typically involves symptoms that are similar to other conditions such as lupus and lymphoma, a form of cancer.

The signs and symptoms differ from person to person, depending on how far the disease has progressed. The condition could be chronic or episodic. Some of the common symptoms are as follows:

  • Sore throat: One of the initial symptoms of adult Still’s disease is swollen lymph nodes which become tender, leading to a sore throat.
  • Fever: A body temperature of at least 102 degrees Fahrenheit (38.88 degrees Celsius). The fever lasts for a week or more and generally peaks late in the afternoon or during early evenings.
  • Rash: A salmon-pink rash may appear on one’s trunk, arms or legs—the rash might come and go with the fever.
  • Muscle pain: Similar to a rash, muscle pain may flow with the fever as and when it comes and goes, but in terms of magnitude, it may have the ability to disrupt one’s daily activities.
  • Achy and swollen joints: The joint discomfort and pain, usually lasting for two weeks or so, arises out of joints being inflamed, stiff, and painful, leading to pain in hands, shoulders, ankles, and elbows.

Adult-onset Still's disease causes

The cause of Still’s disease isn’t known. Some experts suggest that a viral infection or bacterial infection may cause the condition. Others are of the opinion that it is either an autoinflammatory condition or can be caused by exposure to toxic substances.

Auto-inflammatory syndromes are the result of a problem with the innate immune system (the immune system we are born with)—they are different from autoimmune disorders. People with antiinflammatory conditions may experience repeat episodes of inflammation for no apparent reason.

Adult-onset Still's disease risk factors

Age is the primary risk factor for Still's disease in adults, with an occurrence that peaks twice: once between 15 and 25 years and again between 36 and 46 years.

Gender doesn't seem to be a factor, with males and females equally at risk for adult-onset Still's disease.

Adult-onset Still's disease diagnosis

It is not possible to diagnose Still’s disease in one go. If the doctor suspect adult Still's disease upon clinical examination:

  • He/she would check if the patient fits the at-risk description
  • He/she may recommend an imaging test to see the damage that has been caused by the disease.
  • Following this, a blood test may also be conducted in order to rule out other conditions with similar symptoms.

Adult-onset Still's disease treatment

The objective of the treatment is to reduce inflammation and symptoms like pain and fever. For this, the doctor may prescribe:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen or naproxen sodium for pain relief.
  • Corticosteroids like prednisone to reduce inflammation. These drugs may be given along with methotrexate—this also helps to reduce the dosage of steroids.
  • Biologics: Medicines like infliximab, adalimumab and etanercept (TNF-alpha antagonists) are being used in different types of arthritis to reduce inflammation.
  • Experimental medicines like anakinra affect the activity of interleukins, or cytokines/proteins that mediate inflammation in the body.
  • Ilaris (canakinumab) received US Food and Drug Administration (FDA) approval for the treatment of patients with active-onset Still’s disease in June 2020, based on its effectiveness in treating systemic idiopathic juvenile arthritis and a randomised double-blind trial with 36 patients with adult Still's disease.
  • Actemra or tocilizumab is used in the treatment of systemic-onset juvenile idiopathic arthritis (soJIA), which is considered to be very similar to adult-onset Still's disease with one exception: the age of the patients.

Adult-onset Still's disease lifestyle changes

Adult onset Still's disease can be chronic or episodic. For the chronic condition, it is important to understand which medicines you've been prescribed and the correct dosage.

Follow your doctor's advice on diet. For example, if you are taking prednisone to reduce inflammation, your doctor will tell you to take more calcium-rich foods and vitamin D supplements and vitamin-D foods to prevent osteoporosis.

Exercises for arthritis are a good way to maintain joint mobility, strengthen supporting muscles and reduce swelling and pain. Try to build these into your daily routine.

The pain of arthritis—especially chronic pain—can take a toll on your overall well-being and mental health as well. Talk to friends and family whenever you feel overwhelmed. Do not hesitate to reach out to a psychologist for help.

Adult-onset Still's disease complication

The condition often leads to complications owing to chronic joint and organ inflammation. Some of the main complications of adult-onset Still's disease are:

  • Heart inflammation: The disorder may lead to pericarditis (inflammation in the sac-like portion of one’s heart) or myocarditis (inflammation in the muscular part of the heart).
  • Joint damage: The disorder can also damage one’s joints, usually one’s knees and wrists. Rarely, it may affect one’s neck, finger, hip, and foot joints.
  • Excess fluid in one’s lungs: Fluid build-up around one’s lungs may be caused by the inflammation, leading to difficulty in breathing.
  • Macrophage activation syndrome: This is one of the rare complications that may arise from Still’s disease. It involves low blood cell count, high triglyceride levels and abnormal liver function. This condition can be fatal.


References

  1. Bruno Fautrel, Adult-onset Still disease Best Practice & Research Clinical Rheumatology, Volume 22, Issue 5, 2008, Pages 773-792
  2. Gerfaud-Valentin, M., Maucort-Boulch, D., Hot, A., Iwaz, J., Ninet, J., Durieu, I., Broussolle, C., & Sève, P. (2014). Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Medicine, 93(2), 91–99
  3. Hong, D., Yang, Z., Han, S., Liang, X., Ma, K., & Zhang, X. (2014). Interleukin 1 inhibition with anakinra in adult-onset Still disease: a meta-analysis of its efficacy and safety. Drug design, development and therapy, 8, 2345–2357
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