Adult Still’s disease

Date Updated: 05/08/2021

Overview

Adult Still's disease is a rare type of inflammatory arthritis that features fevers, rash and joint pain. Some people have just one episode of adult Still's disease. In other people, the condition persists or recurs.

This inflammation can destroy affected joints, particularly the wrists. Treatment involves medications, such as prednisone, that help control inflammation.

Symptoms

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

Fever. You might have 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. You might have two fever spikes daily, with your temperature returning to normal in between.
Rash. A salmon-pink rash might come and go with the fever. The rash usually appears on your trunk, arms or legs.
Sore throat. This is one of the first symptoms of adult Still's disease. The lymph nodes in your neck might be swollen and tender.
Achy and swollen joints. Your joints — especially your knees and wrists — might be stiff, painful and inflamed. Ankles, elbows, hands and shoulders might also ache. The joint discomfort usually lasts at least two weeks.
Muscle pain. Muscular pain usually ebbs and flows with the fever, but the pain can be severe enough to disrupt your daily activities.

The signs and symptoms of this disorder can mimic those of other conditions, including lupus 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. Also, if you have adult Still's disease and develop a cough, difficulty breathing, chest pain or any other unusual symptoms, call your doctor.

Causes

It's not certain what causes adult Still's disease. Some researchers suspect the condition might be triggered by a viral or bacterial infection.

Risk factors

Age is the main risk factor for adult Still's disease, with incidence peaking twice: once from 15 to 25 years and again from 36 to 46 years. Males and females are equally at risk.

Complications

Most complications from adult Still's disease arise from chronic inflammation of 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 saclike 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.
Macrophage activation syndrome. This rare, but potentially fatal complication of adult Still's disease, can cause low blood cell counts, very high triglyceride levels and abnormal liver function.

Diagnosis

No single test identifies adult Still's disease. Imaging tests can reveal damage caused by the disease, while blood tests can help rule out other conditions that have similar symptoms.

Treatment

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 have side effects.

Nonsteroidal anti-inflammatory drugs (NSAIDs). Over-the-counter NSAIDs, such as ibuprofen (Advil, Motrin IB, others) or naproxen sodium (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 (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), tocilizumab (Actemra) or rituximab (Rituxan).

Lifestyle and home remedies

Here are ways to 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. Although you might not want to work out if your joints ache, exercise is recommended for all types of arthritis. Exercise can help you maintain your range of motion and relieve pain and stiffness.

Preparing for an appointment

You're likely to seek advice from your primary care provider, but he or she might refer you to a specialist in joint diseases (rheumatologist).

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fast for a specific test. Make a list of:

Your symptoms, including when they started and how often they flare
Key medical information, including any other health conditions with which you've been diagnosed
All medications, vitamins and supplements you take, including the doses
Questions to ask your doctor

Take a family member or friend along, if possible, to help you remember the information you get.

For adult Still's disease, basic questions to ask your doctor include:

What's likely causing my symptoms?
What other possible causes are there?
What tests do I need?
Is my condition likely temporary or chronic?
What's the best course of action?
What are the alternatives to the approach you're suggesting?
I have these other health conditions. How can I best manage them together?
Are there restrictions I need to follow?
Should I see a specialist?
Are there brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you questions, such as:

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