It's mysterious and it's rare. A form of rheumatoid arthritis, Still's Disease mainly affects children. But there's even a rarer form which strikes adults -- Adult Onset Still's Disease (AOSD).
AOSD is an inflammatory condition that attacks internal organs, joints and other parts of the body. It can appear and disappear suddenly. In very severe cases, AOSD becomes chronic and extremely debilitating, causing terrible pain and stiffness. After many years, the disease cripples vital organs such as the heart and lungs.
There are treatments, mainly anti-inflammatory drugs, that help with most AOSD symptoms. Until recently, we have known little about its causes but medical researchers are starting to learn more about AOSD. With this knowledge comes the promise of new and better treatments.
AOSD gets its name from Dr. Still, a renowned British pediatrician, who first identified the childhood form of the disease. Though rare, it is found worldwide and predominantly affects young adults between 16 and 35.
AOSD does not seem to run in families. The disease can be difficult to diagnose, as it is difficult to distinguish from a number of other conditions that cause unexplained fever as well as other forms of rheumatoid arthritis, as well as from a number of other diseases.
The first sign that someone may be coming down with AOSD is a high spiking fever of 103 degrees or above. The fever usually rises rapidly in the evening and then subsides to or near normal by the next morning. Occasionally, an unusual pattern of two fever spikes in a day is seen in one-fifth of AOSD sufferers.
- High fever lasting one week or longer
- Joint pain lasting two weeks or longer
- Abnormal white blood cell count and other blood problems
- Sore throat
- Swelling of the lymph nodes or spleen
- Liver problems
- Absence of rheumatoid arthritis
- Infections such as hepatitis, rubella, parvovirus,coxsackie, EBV (Epstein-Barr virus) CMV (cytomegalo virus) and HIV (AIDS)
- Infective endocarditis (an infection of the heart tissue)
- Lyme disease
- Crohn's disease
- Cancer, including leukemia and lymphoma
- Connective tissue disease, such as SLE (Lupus)
The abrupt appearance of high fever, sore throat, rash, lymph node swelling and abnormal white blood cell counts suggest some kind of infectious trigger.
While no particular cause of infection has been found, the rubella (German Measles) virus has been detected in many people with AOSD cases. Researchers have also found some association between AOSD and EB, CMV, mumps, parainfluenza, and other viruses.
- Immune Disorder
- Hormonal Influences
Pregnant women are slightly more likely to come down with AOSD or to have a relapse. On the other hand, if the female hormones associated with pregnancy played a role, we would expect AOSD to be more common in one sex or the other. Unlike lupus or rheumatoid arthritis, however, which are more common in women, AOSD seems to affect both sexes equally.
In particularly acute or severe cases, doctors sometimes also prescribe corticosteroids to address heart, blood, and other life-threatening problems that AOSD can cause. Anti-TNF therapy, aimed at a component of the immune system, may be a promising treatment.
Some AOSD sufferers do not respond well to NSAIDs and go on to develop the chronic (long term) form of the disease. Because corticosteroids have serious side effects, they cannot be used to treat AOSD over a period of years. Instead, depending on the individual case, doctors prescribe drugs such as methotrexate, IM gold, D-penicillamine, hydroxychloroquine and azathioprine. For safety reasons, cyclophosphamide is reserved only for the most difficult cases.