Adult Still’s disease

Adult Still’s disease is a rare type of arthritis that features a sore throat, a salmon-colored rash and a high fever that spikes once or twice a day. Joint pain tends to develop a few weeks after these initial signs and symptoms.

The cause of adult Still’s disease is unknown, but researchers are investigating the possibility that it might be triggered by some type of infection. Some people experience just one episode of adult Still’s disease. In other people, the condition persists or recurs.

Adult Still’s disease is an inflammatory type of arthritis, similar to rheumatoid arthritis. This inflammation can destroy affected joints, particularly the wrists. Treatment involves medications, such as prednisone, that help control inflammation.

Most people with adult Still’s disease experience a combination of the following signs and symptoms:

• Sore throat. One of the very first symptoms of adult Still’s disease is a sore throat. The lymph nodes in your neck also might be swollen and tender.
• Fever. You may experience a daily fever of at least 102 F (38.9 C) for a week or longer. The fever usually peaks in the late afternoon or early evening. Sometimes, you may experience two fever spikes daily. Between episodes, your temperature will likely return to normal.
• Rash. A salmon-pink rash may come and go with the fever. The rash usually appears on your trunk, arms or legs. Physical contact, such as rubbing your skin, may provoke the rash to appear.
• Achy and swollen joints. You may find that your joints — especially your knees, wrists, ankles, elbows, hands and shoulders — are stiff, painful and inflamed. Usually, the joint discomfort lasts at least two weeks.
• Muscle pain. Muscular pain associated with adult Still’s disease usually ebbs and flows with the fever, but the pain may be severe enough to disrupt your daily activities.

Having any of these signs or symptoms doesn’t necessarily mean that you have adult Still’s disease. The signs and symptoms of this disorder may mimic those of other conditions, including mononucleosis and a type of cancer called lymphoma.

When to see a doctor

If you have a high fever, rash and achy joints, see your doctor to determine what may be the cause. Also, if you have adult Still’s disease and develop a cough, difficulty breathing, chest pain or any other unusual symptoms, call your doctor.

Although it’s not certain what causes adult Still’s disease, the condition may be triggered by a viral or bacterial infection.

Age is the main risk factor for adult Still’s disease, with incidence in adults peaking twice: once from 15 to 25 years and again from 36 to 46 years. Males and females are equally at risk of acquiring the disorder. Multiple cases of adult Still’s disease in families are uncommon, so it’s unlikely that this disorder is inherited.

Most complications from adult Still’s disease arise from chronic inflammation of your body organs and joints.

• Joint destruction. Chronic inflammation can damage your joints. The most commonly involved joints are your knees and wrists. Your neck, foot, finger and hip joints also may be affected, but much less frequently.
• Inflammation of your heart. Adult Still’s disease can lead to an inflammation of the sac-like covering of your heart (pericarditis) or of the muscular portion of your heart (myocarditis).
• Excess fluid around your lungs. Inflammation may cause fluid to build up around your lungs, which can make it hard to breathe deeply.

You’re likely to first seek advice from your family doctor, but he or she might refer you to a specialist in joint diseases (rheumatologist).

Because appointments can be brief and there’s often a lot of ground to cover, it’s a good idea to be well prepared for your appointment. Here’s some information to help you get ready for your appointment, and what to expect from your doctor.

What you can do

• Write down your symptoms, including when they first started and how often they flare up.
• Write down your key medical information, including any other health conditions with which you’ve been diagnosed.
• List all the medications you’re currently taking, including prescription and over-the-counter drugs, vitamins, or supplements.
• Take a family member or friend along, if possible. Someone who accompanies you can help remember information that you missed or forgot.
• Write down questions to ask your doctor. Creating your list of questions in advance can help you make the most of your time with your doctor.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

• What are your symptoms, and when did they first develop?
• Do your symptoms come and go, or are they continuous?
• When are your symptoms most likely to flare up?
• What treatments or self-care measures have you tried so far?
• Have any treatments or self-care measures helped?
• Have you been diagnosed with any other medical conditions?

There’s no single test used to diagnose adult Still’s disease. Imaging tests can reveal the damage that has been caused by adult Still’s disease, while blood tests can help rule out other conditions that have similar symptoms.

Doctors use a variety of drugs to treat adult Still’s disease. The type of drug you’ll take depends on the severity of your symptoms and whether you experience side effects.

• Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter NSAIDs, such as ibuprofen (Advil, Motrin IB, others) or naproxen (Aleve), may help with mild joint pain and inflammation. Stronger NSAIDs are available by prescription. NSAIDs can damage the liver, so you may need regular blood tests to check liver function.
• Steroids. Most people who have adult Still’s disease require treatment with steroids, such as prednisone. These powerful drugs reduce inflammation, but may lower your body’s resistance to infections and increase your risk of developing osteoporosis.
• Methotrexate. The medication methotrexate (Rheumatrex, Trexall) is often used in combination with prednisone, which allows the prednisone dose to be reduced.
• Biologic response modifiers. Drugs such as infliximab (Remicade), adalimumab (Humira) and etanercept (Enbrel) have shown some promise, but their long-term benefit is still unknown. If other medications haven’t worked, your doctor may suggest trying anakinra (Kineret) or rituximab (Rituxan).

Here are a few ways to help you make the most of your health if you have adult Still’s disease:

• Understand your medications. Even if you’re symptom-free some days, it’s important to take your medications as your doctor recommends. Controlling inflammation helps reduce the risk of complications.
• Supplement your diet. If you’re taking high doses of prednisone, talk to your doctor about taking more calcium and vitamin D supplements to help prevent osteoporosis.
• Keep moving. While you might not feel up to a workout if your joints ache, exercise can help you maintain your range of motion and relieve pain and stiffness.

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