What is Adult-Onset Still’s Disease?
Adult-onset Still’s disease, or AOSD, is a rare form of arthritis that affects around 1 in 100,000 adults. Men and women are equally susceptible, but people between the ages of 15 to 25 and 36 to 46 are at highest risk. This condition does not appear to run in families.
Scientists have not pinpointed the cause, but Still’s disease typically strikes after an infection. Some researchers theorize that certain antigens — which are toxins that trigger the body’s production of antibodies — may play a role.
Still’s is inflammatory in nature, similar to rheumatoid arthritis, and it may be a short-term issue or a persistent, chronic one.
The most distinctive characteristic of AOSD is a bumpy, salmon-colored rash, but swelling in the joints, muscle tissue, lymph nodes and organs is common too. Patients also report extreme fatigue.
Diagnosing this disease is no easy feat. Doctors must usually perform multiple tests to rule out unrelated conditions that mimic early symptoms of Still’s. Lyme disease, Crohn’s disease, lymphoma and lupus are examples.
A blood test usually reveals elevated levels of serum ferritin, a blood protein that contains iron. Doctors also listen to the heart and lungs, and imaging tests may reveal telling signs in the liver, spleen or chest cavity.
There is no cure for adult Still’s disease, but it is treatable. The prognosis for most patients is largely favorable, but untreated symptoms could threaten the heart, kidneys or lungs and cause grave complications.
Symptoms of Adult Still’s Disease
Common symptoms include the following:
- Rash
- The first and most marked symptom is a flat or elevated salmon-pink rash. It usually appears on the chest and thighs. The face, arms and lower legs are less likely to be affected. The rash is usually evanescent, meaning that it quickly vanishes and reappears. It may or may not itch.
- Fever
- Fever may persist daily for a week or longer. It typically spikes to 102 F or higher in late afternoon or early evening.
- Sore throat
- This is another early sign. It is accompanied by tender, swollen lymph nodes in the neck, and swallowing is painful.
- Severe muscle aches
- The pain tends to ebb and flow with the fever, but this symptom can be limiting enough to prevent patients from working.
- Swollen, aching joints
- Inflammation causing pain, warmth, swelling and stiffness often persists for two weeks or more. It is most pronounced in the wrists and knees, but it might be problematic in the hands, elbows, shoulders and ankles as well.
- Fatigue
- Extreme fatigue sidelines many workers until they are diagnosed and treated.
Still’s is marked by unpredictability. Several symptoms may appear suddenly, or one symptom at a time might get progressively worse. Some patients experience a single episode and are never bothered by symptoms again. Others have a years-long series of flare-ups and remissions.
Treatment for Adult-Onset Still’s Disease
The importance of treating adult-onset Still’s disease cannot be overstated.
Chronic inflammation can wear down joints. In rare cases, the spleen or lymph nodes may become enlarged. Inflammation of lung tissue may cause fluid to accumulate and interfere with normal breathing. Some patients experience unexplained weight loss or abdominal pain and swelling. One rare complication, macrophage activation syndrome, can lead to serious liver problems.
In short, even mild cases call for prompt treatment.
No one-size-fits-all therapy has been consistently effective for everybody. More often, a variety of drugs taken alone or in combination are prescribed on a case-by-case basis.
Nonsteroidal anti-inflammatory drugs work well to reduce inflammation, and they reduce fever and joint pain as well. Over-the-counter analgesics, like acetaminophen, ibuprofen and high-dose aspirin, further help relieve pain.
Some patients start with high doses of corticosteroids, like prednisone, before gradually tapering off. Using prednisone over long periods may produce unwanted side effects.
Methotrexate, anakinra and other immunosuppressive drugs are commonly used for rheumatic conditions like arthritis. When prescribed for adult Still’s disease, they eliminate the need for long-term corticosteroid use and reduce the risk of associated problems.
Still’s is often cyclical. Patients should not stop taking medications when they feel better without asking the doctor. Exercise, calcium supplements and vitamin D can also help prevent the development of osteoporosis.
