Still's Disease

What Is Still’s Disease?

Still’s disease, also known as systemic juvenile idiopathic arthritis (SJIA), is a form of arthritis that affects your whole body. It causes:

  • Fever
  • Joint pain
  • Rash

It’s a rare subtype of juvenile idiopathic arthritis (JIA). That's the most common form of arthritis in kids and teens. Only about 10%-20% of kids with JIA have Still’s disease.

Even though it’s a subtype of JIA, the causes, symptoms, and treatment are different. Still’s disease is more severe than other types of JIA. You can get it at any point in childhood, but most people start to feel symptoms around age 2.

Causes

Doctors don’t know exactly what causes Still’s disease. Experts think it may be a combination of certain triggers from your genes and your environment.

What they know more about is how it’s different from JIA.

The immune system you have when you’re born is your innate immune system. As you age, you form an adaptive immune system to deal with the viruses and bacteria that get past your innate immune system.

JIA is an autoimmune disease. That means when you have it, your adaptive immune system attacks healthy cells and tissues as if they're harmful to your body. This causes the symptoms of JIA.

Kids with Still’s disease don’t have the things that usually show up with an overactive adaptive immune system. Instead, they have high levels of some inflammatory proteins. These proteins are also high in other diseases known as autoinflammatory diseases.

Experts believe that this is what causes the inflammation that's common with Still’s disease.

Symptoms

Along with your joints, Still's disease can affect your liver, heart, and lungs. It can make you have:

  • A flat, pink rash
  • Fever around the same time every day
  • Warm, swollen, painful joints
  • Swollen lymph nodes
  • Inflammation in internal organs, such as your spleen, liver, or heart
  • High blood pressure in the arteries that lead to your lungs
  • Shortness of breath from scarred lung tissue

Over time, if Still’s disease is untreated, inflammation can damage your joints and lead to:

  • Stunted growth
  • Limited range of motion in your joints
  • Joint replacement
  • Hair loss
  • Anorexia (a lack or loss of appetite)
  • Anemia (low iron)
  • An overall feeling of being unwell

Symptoms can come and go in flares that can last days or months.

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Getting a Diagnosis

There aren’t specific tests that let your doctor know if you have Still’s disease. So, they'll base their diagnosis on:

  • Your symptoms
  • A physical exam
  • Blood tests to look for signs of inflammation and check for infection
  • Images like an X-ray, CT scan of your chest, or a look at how your heart is working called an echocardiogram

Rarely, if your doctor isn’t clear about their diagnosis, they may take a biopsy of the skin where your rash is to get a closer look at it.

Questions for Your Doctor

When you visit your child's doctor, it's a good idea to have a list of questions on hand so you can get all the information you need.

You can ask:

  • What's causing my symptoms?
  • What are my treatment options?
  • Do these treatments have side effects?
  • What is the goal of my treatment?
  • Are there symptoms I should watch out for?
  • What changes can I make at home that could help with my symptoms?

 

Treatment

Still’s disease doesn’t have a cure, but it’s possible to ease symptoms enough to go into remission. Treating the disease early is key.

Your doctor may recommend:

Nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen and naproxen to help ease pain and inflammation

Corticosteroids to take by mouth or IV to help bring down fever, pain, and swelling in joints

Biologic medications to take as a shot or IV infusion that target inflammatory proteins. These medicines can include:

  • Adalimumab (Humira)
  • Anakinra (Kineret)
  • Canakinumab (Ilaris)
  • Etanercept (Enbrel)
  • Infliximab (Remicade)
  • Tocilizumab (Actemra)

Nonbiologic disease-modifying antirheumatic drugs (DMARDs) you take with biologics to treat your arthritis

Taking Care of Yourself

Along with treatment, make sure your child gets regular exercise when possible. Physical activity helps build muscle, boost energy, and ease pain.

You can also use cold or heat to help loosen stiff joints.

A good diet, especially one high in calcium, is important for kids with Still’s disease. This condition raises their chances of osteoporosis. The use of corticosteroids brings them up, as well.

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What to Expect

Still’s disease is a lifelong condition for most people. Children may continue to have symptoms into adulthood.

The goal of treatment used to be to manage pain and get symptoms under control. But treatment advances make doctors hopeful that more people with Still’s disease can get rid of their symptoms completely.

Getting Support

Your child may have many different emotions about having Still’s disease. You can help them through these feelings and support your child as they deal with symptoms and treatment.

You should:

  • Allow them to express how they feel with no judgment.
  • Remind them they didn’t do anything to cause it.
  • Find ways for them to exercise and move their body.
  • Inform their teachers at school so they have support in other places.

 

WebMD Medical Reference Reviewed by David Zelman, MD on September 29, 2020

Sources

SOURCES:

Arthritis Foundation: “Systemic Juvenile Idiopathic Arthritis.”

Hospital for Special Surgery: “Systemic Juvenile Idiopathic Arthritis (SJIA).”

Mayo Clinic: “Juvenile idiopathic arthritis.”

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