1. Group 7
Adrienne Grenier, Koteuaisa Wilson , Maura Comer, Alison Webb

2. Group 7

3. What Is Juvenile Rheumatoid Arthritis?
Arthritis is a disease in a person’s joints that cause swelling and pain where the bones meet each other, resulting in stiffness and loss of motion
Juvenile Rheumatoid Arthritis is arthritis in children under 16 years old with inflammation lasting at least 6 weeks

4. What Is Juvenile Rheumatoid Arthritis?
Juvenile rheumatoid arthritis is different from adult rheumatoid arthritis
Kids have different symptoms
The damage to bones is different

5. How Is Juvenile Rheumatoid Arthritis Different From Adult Rheumatoid Arthritis?
Main Difference: Many people with JRA outgrow the illness, while adults usually have lifelong symptoms.
Studies estimate that by adulthood, JRA symptoms disappear in more than half of all affected children.
Unlike rheumatoid arthritis in an adult, JRA may affect bone development as well as the child's growth.

6. Who Gets JRA?
About 1 child in every 1,000 develops some type of juvenile arthritis.
Approximately 75,000 children in the U.S.
Can affect children at any age, but uncommon in the first six months of life

7. Why Do Kids Get JRA? Cause is unknown JRA is an autoimmune disease
Research suggests a genetic predisposition
Triggered by an environmental factor, such as a virus
JRA is an autoimmune disease
Immune system mistakes healthy cells for harmful ones.
Body releases chemicals to fight healthy cells causing pain and swelling.

8. Types of Juvenile Rheumatoid Arthritis
There are three types JRA.
Pauciarticular: fewer than five joints are affected. Usually knees and large joints.
Polyarticular: affects five or more joints. Joints on both sides of body are affected. Smaller joints like hands or toes.
Systemic JRA: affects many parts of the body. Heart, lungs and liver could be affected as well.

9. Signs/symptoms Limping Stiffness when awakening
Reluctance to use an arm or leg
Reduced activity level
Persistent fever
Joint swelling

10. Signs/symptoms

11. Pathophysiology
Chronic inflammation of synovium is characterized by B lymphocyte infiltration and expansion.
Macrophages and T-cell invasion are associated with the release of cytokines, which evoke synoviocyte proliferation.

12. Pathophysiology Continued…
2001 study by Scola et al :
Found synovium to contain m-RNA for vascular endothelial growth factor, angiopoietin 1, and their respective receptors
This suggests that induction of angiogenesis by products of lymphocytic infiltration may be involved in persistence of disease.
The resulting thickened pannus causes joint destruction. In many patients, predominance of cytokines associated with tissue destruction, including interleukin-6 and TNF, suggests the possibility of improved responsiveness to specific biologic agents targeting these factors.

13. Healthy Joint Vs. Damaged Joint

14. Active Learning: X-Ray of Patient
One is normal, and one is a patient with JRA:
Can you determine the differences?

15. How Is JRA Diagnosed? Complete health history
Physical examination to look for joint inflammation, rashes, nodules, signs of internal organ inflammation and/or eye problems
Laboratory tests to help rule out other diseases
Erythrocyte sedimentation rate
Hemoglobin and blood count testing
Urinalysis
X-rays
Tests of joint, blood and tissue fluids to check for infections or inflammation

16. Treatment Options
Goal: Control symptoms, prevent joint damage, and maintain function
1. Non-steroidal Anti-Inflammatory Drugs (NSAIDS)
Motrin or Advil
2. Disease Modifying Drugs (DMARDS)
Hydroxychloroquine: Plaquenil
Sulfasalazine: Azulfidine
Methotrexate: Rheumatrex

17. Medication Options
JRA is treated similarly to adult RA, although many drugs used to treat RA are not approved for children.
Aspirin, in large doses, is generally the first line of defense.
If the disease is progressive, anti-rheumatic drugs, such as methotrexate, injectable gold, can be used .
The new biologic agent, etanercept (Enbrel), was recently approved for treating JRA. Glucocorticoids, such as prednisone, are usually reserved for children with internal organ or eye inflammation because of the drugs' potential, at high doses, to stunt growth.

18. Structure of Medications
Plaquenil
Rheumatrex
Azulfidine

19. Medications Continued…
Juvenile Rheumatoid Arthritis
Medications Continued…
Drug Type/Name(s)
Dosage
Possible Side Effects
Cautions
Nonsteroidal Anti-Inflammatory Drugs (Naiads) (ibuprofen, naproxen, tolmentin, aspirin, choline magnesium, trisalicylate, indomethacin)
Taken one to four times per day in pill or liquid form. Must be taken three to four weeks to determine if helping to control pain and inflammation. 
Possible side effects include occasional stomach pains, nausea and vomiting, anemia, headache, blood in the urine, fluid retention, thinning and scarring of the skin (especially with naproxen), difficulty concentrating, stomach ulcer.
Aspirin Possible side effects include pains or stomach bleeding. Toxic reactions can occur from too high a dose built up in your child's system (rapid or deep breathing, ringing in the ears, decrease in hearing, drowsiness, nausea, vomiting, irritability, unusual behavior and black stools). A rare complication called Reye's syndrome can occur in children who have the chicken pox or the flu and are taking aspirin.
Young children should not suck or chew on aspirin as this may damage the chewing surfaces of the teeth and irritate the gums. Instead, try crushing the dose and having the child swallow it in a small amount of a favorite food such as applesauce or yogurt.
The different NSAIDs should never be combined without doctor's instructions.
Disease-Modifying Anti-Inflammatory Drugs(DMARDS)  (hydroxychloroquine,  sulfasalazine, penicillamine, gold compounds)
Hydroxychloroquine (Plaquenil) Given in pill form. May take 3 to 6 months to work. 
Sulfasalazine (Azulfadine) Given in pill form. Takes 6-12 weeks to work.
Gold Compounds (Auranofin, Ridaura, Myochrysine, Solganol) Oral form taken daily. Injections usually given weekly for 5 or 6 months, then once or twice a month for as long as necessary. Take 3 to 6 months to work. 
Penicillamina (DePen, Cuprimine) Given in pill form. Takes up to 6 months to work. 
Hydroxychloroquine (Plaquenil) Side effects may include upset stomach, skin rash. Rare complication, eye damage. 
Sulfasalazine (Azulfadine) Side effects may include stomach uoset, achiness, diarrhea, dizziness, headache, light sensitivity, itching, appetite loss, liver abnormalities, lowered blood count, nausea, vomiting or rash.
Gold Compounds (Auranofin, Ridaura, Myochrysine, Solganol) Side effects may include skin rash, mouth sores, kidney problems, a low blood count, anemia. 
Penicillamina (DePen, Cuprimine) Side effects may include diarrhea, skin rash, hives or itching, low blood counts, mouth sores, nausea or vomiting, stomach pain, loss of taste or appetite, swollen glands, unusual bleeding or bruising, weakness. 
Hydroxychloroquine (Plaquenil) Child should have his/her eyes checked every six months by an opthalmologist familiar with this medication. 
Sulfasalazine (Azulfadine) Can sometimes have more side effects in children with systemic onset JRA.

20. Surgery Rarely used in the early course of disease Function:
Relieve pain
Release joint contractures
Replace a damaged joint

21. Exercise
Target:
Shoulder
Neck
Lower leg
Chest
Hip
Knee

22. Frequency In the U.S. Internationally 10-20 cases per 100,000 children
Pauciarticular and polyarticular disease occur more frequently in girls
Both sexes are affected with equal frequency in systemic-onset disease
Internationally
Occurs more frequently in certain populations (e.g., Native Americans) from areas like British Columbia and Norway

23. Mortality
Less than 1%
Often associated with the evolution of disease to manifestations of other rheumatic diseases

24. Morbidity Morbidity: Psychological Morbidity:
Relates to adverse effects of medications, particularly NSAIDS
Abdominal pain due to gastritis or ulcer disease, hepatotoxicity, renal toxicity
Psychological Morbidity:
Situational depression
Problems functioning in school

25. Is There a Bright Side? There are numerous resources for parents:
Websites
Support systems for children
Ongoing research to improve quality of life
Five-year-old Katie Tortorice leads an active, healthy life despite having juvenile rheumatoid arthritis (JRA).

26. Resources: Paper Media: Web:
Miterski B, Drynda S, Boschow G, Klein W, Oppermann J, Kekow J, Epplen J Complex genetic predisposition in adult and juvenile rheumatoid arthritis BMC Genetics 2004, 5:2 1-14
Web: