Adult-Onset Still's Disease

What Is Adult-Onset Still’s Disease?

Adult-onset Still’s disease is a rare type of arthritis that starts in adulthood. It can cause:

  • Fever
  • Rash
  • Joint pain

Adult-onset Still’s disease is the adult form of a subtype of arthritis in kids called systemic juvenile idiopathic arthritis (SJIA).

Adult-onset Still’s disease typically affects adults under age 35. Fewer than 1 in 100,000 people get it each year. There is no cure, but you can control your symptoms with treatment.

Causes

Doctors don’t know what’s behind adult-onset Still’s disease. Research suggests that infection or toxic substances in your body can trigger it. Your immune system overreacts to these triggers and sets off the disease.

Experts consider adult-onset Still’s disease an auto-inflammatory condition because the immune system plays a role in its symptoms. At birth, you have an innate immune system. As you age, you form an adaptive immune system. That deals with the bacteria and viruses that get by your innate one.

When you have an autoimmune disease, your symptoms happen because your adaptive immune system overreacts and attacks your healthy cells and tissues. In auto-inflammatory diseases like adult-onset Still's disease, the innate immune system overreacts and causes inflammation.

Research also shows that people with adult-onset Still’s disease have higher levels of proteins called cytokines than people who don't have the disease.

Your immune system sends out cytokines to fight inflammation in the body.

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Symptoms

In adult-onset Still’s disease, symptoms typically come and go in episodes. For some, these go on for about a year, and then the disease goes away. For others, the episodes come on at random, going away for weeks or months and then coming back. You may also have chronic adult-onset Still’s disease, where your symptoms are more constant.

Usually, the first things you'll feel are:

  • A high fever that spikes once or twice a day
  • A warm, pink rash on your trunk and arms that appears with the fever

Your joints start to ache a few weeks after your fever and rash show up. This joint pain and stiffness can last for 2 weeks. You’ll usually feel it in your wrists and knees.

It could also show up in your:

  • Ankles
  • Shoulders
  • Elbows
  • Fingers

You may also have:

  • Pain and swelling in your abdomen
  • Sore throat
  • Swollen lymph nodes
  • Weight loss
  • Pain when you take a deep breath

Your spleen and liver can also swell, and you may have inflammation in your heart or lungs.

Getting a Diagnosis

There is no single test your doctor can do to confirm that you have Still’s disease. Instead, they'll gather information using:

  • Your symptoms
  • Physical exams
  • Blood tests to see if you have other issues like infection or cancer
  • Ultrasound of your abdomen
  • CT scan
  • X-ray

Imaging tests like ultrasounds, X-rays, and CT scans can help your doctor see if the disease damaged any of your organs.

Questions for Your Doctor

To be sure you get all the information you need at your doctor visits, write down your questions beforehand and take them with you. These may include:

  • What’s causing my symptoms?
  • What other conditions could be causing these symptoms?
  • Which tests do I need?
  • Will this be temporary, or will I have it for a long time?
  • What's the best treatment for me?
  • Will my treatment have side effects?
  • Are there things I can do at home to help with my symptoms?
  • How should I manage other health issues I have?
  • Are there restrictions I need to follow?
  • Should I see a specialist?

 

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Treatment

There are several medications that can help with your symptoms. Your doctor will choose the best options for you depending on how severe your disease is and the side effects that you feel.

These medicines include:

Nonsteroidal anti-inflammatory drugs (NSAIDs). You can try over-the-counter options such as ibuprofen or naproxen to help with joint pain and inflammation. Your doctor can prescribe stronger NSAIDs if the over-the-counter options don't work.

NSAIDs can damage your liver. So if you take these, your doctor may ask you for regular blood tests to see how your liver is working.

Steroids. Ones such as prednisone (Deltasone) are a common treatment for most people with adult-onset Still’s disease. They work well to ease inflammation. But they can also make infection and osteoporosis more likely.

Methotrexate (Trexall). Your doctor may prescribe this in combination with prednisone to treat your arthritis and help your joints. If you take both, you'll take a lower dose of prednisone.

Biologic response modifiers. Some drugs power down your immune system to help ease inflammation. These include:

  • Adalimumab (Humira)
  • Etanercept (Enbrel)
  • Infliximab (Remicade)

Doctors are still looking into how they work long term. If other medications haven't worked, your doctor may suggest other immunosuppressive drugs such as:

  • Anakinra (Kineret)
  • Tocilizumab (Actemra)

 

Taking Care of Yourself

As you go about your daily life, there are things you can do to help manage your disease. These include:

Follow your treatment plan. Keep taking your medications, even if you aren’t having symptoms. You’ll help lower your chances of inflammation and prevent further problems.

Ask about supplements. On top of your healthy, well-balanced diet, you may need a vitamin D and calcium boost. If prednisone is part of your treatment plan, these supplements can help ward off osteoporosis.

Get regular exercise. Aching joints can make exercise seem tough. But doctors recommend moving your body when you can. You’ll help your range of motion and can even ease some of your pain and stiffness.

What to Expect

You can’t cure adult-onset Still’s disease, but staying on top of your treatment can help keep your symptoms under control and prevent complications. For about one-third of people with the disease, symptoms continue for a long time and become chronic arthritis.

Early diagnosis and treatment can help make the future brighter. 

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Getting Support

Because adult-onset Still’s disease is rare, it's smart to connect with national organizations that can put you in touch with specialists and other people who can help. Ask your doctor for recommendations, or visit the websites of the:

  • Arthritis Foundation
  • National Organization for Rare Disorders
  • Genetic and Rare Diseases (GARD) Information Center
  • National Institute of Arthritis and Musculoskeletal and Skin Diseases

 

WebMD Medical Reference Reviewed by Brunilda Nazario, MD on September 30, 2020

Sources

SOURCES:

Arthritis Foundation: “Adult Still’s Disease.”

Hospital for Special Surgery: “Adult Onset Still's Disease.”

Mount Sinai: “Adult Still's disease.”

National Organization for Rare Disorders: “Adult Onset Still’s Disease.”

Mayo Clinic: “Adult Still’s disease.”

BMC Medicine: “Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers.”

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