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Adult Onset Still's Disease
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Adult Onset Still's Disease

 
Drs. Lichauco and Sinha are Fellows in Rheumatology, Department of Medicine, Montefiore Medical Center and the Albert Einstein College of Medicine, New York.


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.

Symptoms of AOSD
Common symptoms of AOSD include those on the following list. It is important to remember that there is no definite pattern that AOSD symptoms follow. In some cases, only one or two symptoms occur immediately; others may follow later. Aside from fever and joint pain, which are almost always among the first signs of AOSD, some of the symptoms on this list may not appear for weeks, or even at all.

Fever

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.

Rash
The typical Still's rash is salmon pink in color and comes and goes unpredictably, although it sometimes occurs at the same time as high fever. It is most common on the chest and thigh, although it may also appear on the face, hands and feet. In some cases, the rash becomes itchy.

Joint and Muscle Pain
At some point, every AOSD sufferer experiences intense joint pain, often in the hand. This can be accompanied by severe muscle pain as well. Both may become worse during a fever spike.

Sore Throat
Most AOSD sufferers come down with a sore throat that is usually described as a severe, constant burning pain in the area around the pharynx.(2)

Heart and Lung Symptoms
These include pain, difficulty breathing and other problems that can be mistaken for an infection of tissue within the heart.

Abdominal Pain
This can be caused by swollen lymph nodes, as well as enlargement of the spleen or liver. These symptoms are also sometimes attributed to other causes.

Abnormal White Blood Cell Count and Other Blood Abnormalities
These can be easily detected using a variety of blood tests. Most patients with AOSD have greatly elevated white blood cell counts which may lead their doctors to suspect an underlying infection or even leukemia. However, the cultures of the blood and other sites are always negative and the blood and bone marrow do not show the abnormal cells found in leukemia. A group of tests referred to as "acute phase reactants" are almost always abnormally elevated in AOSD. The two most commonly associated with AOSD are an elevated sedimentation rate (ESR) and a high blood ferritin level.

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Readers Comments
(12) Comments have been made

yen
my older bro had basically all of the symptoms about two weeks ago.. he's 24 years of age.
Posted Thu, Oct. 6, 2011 at 12:12 am EDT
 
Anonymous
where is the picture of the rash
Posted Fri, Sep. 23, 2011 at 1:32 pm EDT
 
Debbie
Does anyone have a "remedy" for the fatique? I have had AOSD for a little over 2 years now and it just seems to be getting worse instead of better. Being tired ALL the time is starting to mess with my work and I can't afford to quit working. Any suggestions or medications that will help? Thank you, Debbie
Posted Thu, May. 26, 2011 at 6:21 pm EDT
 
Tom
I have had 2 attacks of ASOD, the first when I was 14 at which point I recovered before a diagnosis was made. The second time was when I was 18 and I have a much more severe attack. I was in hospital for almost 6 weeks, including a 3 day stay in intensive care and 12 days in an isolation unit whilst a diagnosis was being made. My blood pressure at one point dropped to 48/31, which is why I ended up in intensive care. However, with great care from the team looking after me at the Norfolk and Norwich Hospital in Norfolk (England) I started to recover whilst on a cocktail of up to 13 different drugs at one time - although no one is actually sure if any of these drugs where actually helping or if I just happened to be recovering at my own pace. After 9 months of intensive physio and treatment I was able to start working, and I was able to stop taking a medication about a year after that. @Upendra, I am sorry to here that your sister is suffering, unfortunately this is a not a disease that is easy to overcome. There is no guaranteed method to treat it and in some cases it is a chronic condition. The best advice I can give is to take it easy, do what the doctors tell you, and if they give you steroids, try not to eat to much!
Posted Thu, Feb. 10, 2011 at 4:55 am EST
 
Upendra
My sister is suffering from AOSD. Please tell me how to overcome this disease.
Posted Tue, Jan. 18, 2011 at 10:24 am EST
 
Tammy
I was diagnosed just about a year ago. I had a rough few months and then with the use of prednisone and methotrexate started to come around. I still get sore but not nearly as bad. I'm off prednisone now and currently take methotrexate, folic acid and plaquenil. Additionally, I also use fish oil, Vit. D. and calcium.
Posted Sat, Jan. 15, 2011 at 2:48 pm EST
 
Anonymous
Harry: please write directly to the editor for an answer to this question.
Posted Fri, Nov. 12, 2010 at 8:31 am EST
 
bidou
(United States) Hi, This is a question for the webmaster/admin here at www.thedoctorwillseeyounow.com. May I use some of the information from your post above if I provide a backlink back to this site? Thanks, Harry
Posted Thu, Nov. 11, 2010 at 8:07 am EST
 
Arun Dubey
My uncle is suffering from this disease for the past year and a half. After several medical treatments there is no relief. Is it curable?
Posted Tue, Sep. 14, 2010 at 7:34 am EDT
 
ken gusdal
I got Stills when i was 18, and it lasted for a couple years. It was the worst thing I ever went through in my life, and I really felt like i was not going to make it through the ordeal. I am now 44 yrs. old. The lasting effects for me are an egg-sized lump just in front of my left ankle and loss of motion in my wrists (they no longer bend back). Both give me some pain in the winter months and when the weather changes. My only advice, stay positive no matter how bad things are. It helps tremendously. I am now looking for some way to have the lump on my ankle removed as it is increasing in size. Any insight would be greatly appreciated if anyone has had this done.
Posted Thu, Aug. 12, 2010 at 1:38 am EDT
 
MIGUEL
My mom has had this for about 1 1/2 years already. Is it ok if she takes prednisone medication?
Posted Mon, Jun. 28, 2010 at 2:39 pm EDT
 
vikash anand
I really want to know the medication of this disease "AOSD.' It's urgent. My uncle is suffering from this for 5 months.
Posted Tue, May. 4, 2010 at 10:05 am EDT










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