Still's Disease

Still's disease is rare illness characteristic of many symptoms, but foremost chronic or long -- term arthritis. 1 Children born with Still's disease have a form of juvenile idiopathic arthritis (JIA). The term juvenile idiopathic ("unknown cause") arthritis derived as comprise between American term juvenile rheumatoid arthritis and European classification juvenile chronic arthritis. 2 In the United States, juvenile idiopathic arthritis affects approximately 25,000 - 50,000 children. 5 "Arthritis is the inflammation of the synovium (the lining tissues) of the joint." Arthritis persistently causes swelling and pain varying degree. Joints affected include the knees, ankle, wrist and small joints in the hands and feet. 3 Common symptoms Still's disease include: Rapid change in body temperature from extreme high fever (102 degrees F) to normal or below, occurring at nearly the same time every day, along with extreme fatigue. The skin appears on and off salmon colored (95% patients 5) and no apparent itching. Patients experience poor appetite, nausea and weight loss. Other known factors include swelling of the lymph glands, enlargement of the spleen and liver, and sore throat. "Some patients develop inflammation around the heart (pericarditis) and lungs (pleuritis), with occasional fluid accumulation around heart (pericardial effusion) and lungs (pleural effusion)."1 Also, breathing deeply (pleuisy) causes pain. 8

Juvenile idiopathic arthritis occurs as early as six weeks of age, but rarely develops before age of six months. The severity of the disease peaks between 1 - 3 years old and between 8 - 12 years old. 6 Different forms of JIA may accompany five (more or less) joints and may or may not include systemic symptoms. 16 Twenty percent of Juvenile idiopathic arthritis (children) patients develop uveitis, serious inflammation of structure within the eye. If uveitis is undiagnosed or untreated could lead to blindness. Steroid eye drops prescribed to treat eye inflammation. 11 About seventy-five percent of Juvenile idiopathic arthritis patients eventually enter remission at puberty with minimal loss and deformity. 12 A pediatric orthopedist or orthopedic surgeon qualified to diagnosis or evaluate juvenile idiopathic arthritis besides a rheumatologist.13

Still's disease named after the English physician Sir George Frederic Still (1861 - 1941), first diagnosed the disease. In 1971, English physician Bywaters, diagnosed the disease less common to as adult - onset Still's disease (AOSD). 4 Majority of adults diagnosed with AOSD between 20 and 35 years of age at the onset of symptoms. 5 Each year less than 1 out of 100,000 people develop adult Still's disease. More women than men develop AOSD. 8

Blood tests confirm the diagnosis of Still's disease:

Erthroycyte Sedimentation Rate (ESR) will be above normal.

Complete Blood Count (CBC) increased number of white blood cells and lower red blood cells

"Liver function tests will show high levels of high AST/ALT"

"Fibrinogen (A protein important for blood clotting 7) level will be high"

Protein found inside cells that stores iron (Ferriton) very high.

"Rheumatoid factor and ANA (antibodies) test will be negative"

Abdominal ultrasound, x-ray, CT scan of the abdomen, joints, chest, liver and spleen confirm the presence of inflammation. "Adult Still's disease can only be diagnosed after other diseases are ruled out." 8 Other types of disease that mimic the symptoms of Adult Still's disease include: Rheumatic diseases, including rheumatoid arthritis, infectious mononucleosis, and a type of cancer called lymphoma. 9

Medication available only to treat the symptoms of adult Still's Disease: Aspirin (Salicylates) and nonsteriod - anti-inflammatory medication (NSAID's) including ibuprofen are prescribed. "Prednisone may be used for more severe cases." Inflammation of adult Still's disease may be reduced by suppressing the immune system by prescribed medication: Methotrexate and Enbrel (etanercept) ("A study by Children's Hospital Medical Center in Cincinnati, Ohio, released in 1999, showed the drug was effective in 75% of children with severe JRA. The drug eases joint pain, reduces swelling, and improves mobility 11) are Tumor necrosis factor (TNF) antagonists. 9 According to British Pediatric Rheumatology (BPRG), etanercept treatment should be withdrawal if severe side effects occur (Headache, gastrointestinal symptoms, rash, irritation and redness at the injection site, and runny nose and cough (upper respiratory infection) 15) or no response to treatment within six months. 14 Etanercept treatment is very expensive, and unknown if the drug has long term dangerous side effects. 15 Experimental drugs been successful in small groups of people: Cyclosporine (Sandimmune), intravenous immunoglobulin (IVIg), anakinra (Kineret) and thalidomide (Thalomid). 9 Alternative treatment option detoxify the body or reduce juvenile idiopathic arthritis symptoms include eating fruits, vegetables (carrots, celery, potatoes, cabbage, lemons, cherries, cucumbers, beets, garlic and radishes). Aromatherapy preparations utilize cypress, fennel, and lemon. Massage oils include chamomile, camphor, juniper, rosemary and benzoin. Also, acupuncture, acupressure and body work. 11 Very rarely surgery is an option, only when medical and physiotherapy fails and severe deformity has developed. 13

"Studies show that in about 20% of patients, all symptoms go away in a year and never come back. In about 30% of patients, all symptoms go away but then come back several times over the next years ". 8 People diagnosed with adult Still's disease fall into one of three groups: Recovery, Occasional flare-ups, and Chronic arthritis. 9

Cause of Still's disease is uncertain. Probability the disease is linked to genetic factors, viral or bacterial infections, abnormal immune responses (infections attack joint tissues), emotional stress and trauma. 6 Very rare the disease affects two children in the same family. 16

Founded in 1999, the International Still's Disease Foundation, providing support, information and a chance to meet others with this disease. Through the organization support group members able to E-mail other members from a mailing list. "It gives you a way to ask questions, tell your story, or if you need a friend." Join the mailing list send an email to: stillsdisease-request@belle.pcpros.net10

1.) Still's Disease (Systemic-Onset Juvenile Rheumatoid Arthritis - http://www.medicinenet.com/stills_disease/article.htm

2.) Juvenile Idiopathic Arthritis - http://www.innvista.com/HEALTH/ailments/arthritis/juvenidi.htm

3.) Juvenile idiopathic arthritis - http://en.wikipedia.org/wiki/Juvenile_idiopathic_arthritis

4.) Still's disease - http://en.wikipedia.org/wiki/Still's_disease

5.) Still's disease (cont.) - http://www.medicinenet.com/stills_disease/page2.htm

6.) Juvenile Idiopathic Arthritis - http://www.innvista.com/HEALTH/ailments/arthritis/juvenidi.htm

7.) Fibrinogen - http://www.drkaslow.com/html/fibrinogen.html

8.) Adult Still's disease - http://www.nlm.nih.gov/medlineplus/ency/article/000450.htm

9.) Adult Still's disease - http://www.mayoclinic.com/health/adult-stills-disease/DS00792/DSECTION=2

10.) International Still's Disease Foundation - http://stilligans.tripod.com/

11.) Juvenile arthritis - http://www.healthatoz.com/healthatoz/Atoz/common/standard/transform.jsp?requestURI=/healthatoz/Atoz/ency/juvenile_arthritis.jsp

12.) Juvenile rheumatoid arthritis - http://www.nlm.nih.gov/medlineplus/ency/article/000451.htm

13.) Juvenile Idiopathic Arthritis - http://www.bchealthguide.org/kbase/topic/major/hw104391/descrip.htm

14.) Arthritis (juvenile idiopathic - etanercept - http://www.nice.org.uk/guidance/index.jsp?action=byID&r=true&o=11446

15) Etanercept for juvenile rheumatoid arthritis - http://www.webmd.com/rheumatoid-arthritis/etanercept-for-juvenile-rheumatoid-arthritis

16.) JUVENILE IDIOPATHIC ARTHRITIS - http://www.printo.it/pediatric-rheumatology/information/UK/1.htm