Juvenile Arthritis

1. Juvenile Arthritis This aint your Grandma�s arthritis!

2. Diagnosis What happened to the R in JRA? We no longer refer to Juvenile arthritis as �rheumatoid� The official name is Juvenile idiopathic arthritis or JIA Unlike adults, few children have a positive rheumatoid factor The causes are largely unknown, thus the term �idiopathic� is more accurate

3. What is arthritis? Arthritis is defined as: Pain (arthralgia) Swelling Limited range of motion Joint pain is a common complaint in children Injury, overuse, infection Growing pains, hypermobility, mechanical issues

4. How is JIA diagnosed? History & Physical Exam JIA is a diagnosis of exclusion Less than 16 years of age Arthritis of unknown etiology Lasts longer than 6 weeks Lab testing There is no single test for JA < 10% of kids have a + RF, � 80% RF + in adults with RA Radiology studies Can be helpful in the diagnosis Help rule out injury

5. Prevalence of JIA There are about 20-150 cases of JIA per 100,000 children. � It is the most common childhood rheumatic disease. � Most children will not achieve long-term remission, so this is truly a chronic condition for most people with JIA. � JIA is a disease characterized by remission and flare.

6. Types of JIA There are eight categories: Systemic Oligo (or pauci meaning few) Oligo extended Polyarticular RF negative Polyarticular RF positive Psoriatic Enthesis related Unclassifiable

7. Systemic JIA Accounts for about 10% of all JIA cases.� Severe inflammation throughout the body Spiking fever, rash, lymphadenopathy, hepatosplenomegaly. � Affects boys and girls equally.� Can have serious effects: macrophage activation syndrome Outcomes: 50% good clinical outcome, 50% go on to have progressive complications. �

8. Oligo Oligoarticular, Oligo extended Few joints 60% of JIA 1-4 joints in 1st 6 months Extended means > 4 joints after 1st 6 months young child <6 years, affects girls more often If ANA positive, ? risk of eye inflammation (uveitis) Usually better prognosis

9. Polyarticular Poly JIA RF positive/RF negative Many joints- 5 or more in 1st 6 months More common in late childhood 25% of all JIA, more common in girls Affects large and small joints, including neck and jaw May have a lower risk of uveitis, but still need routine eye exams RF positive have more aggressive, more adult like arthritis

10. Psoriatic Preceded by, or follows development of psoriasis. Pitting or ridges on the fingernails �sausage digits� may be seen Rash is usually scaly red blotches Appears behind ears, on eyelids, knees, scalp or on the genitals Treatment of rash and treatment of arthritis are the same

11. Enthesis Related Inflammation of the entheses (places where tendons attach to bones) More common in boys Arthritis can be mild, 4 or fewer joints in 50% Arthritis can frequently move to the spine Often test positive for HLA B 27 May develop into spondyloarthropathies: Ankylosing spondylitis, arthritis assoc with IBD, reactive arthritis. �

12. Treatment JIA is a complex autoimmune disease Treatment is often complex There is no quick fix, and often drug therapies require weeks to months to get to full effectiveness Drug side effects can make day to day school life difficult Because JIA is an autoimmune disease, treatment often lowers the immune system

13. First Line Treatments-NSAIDs Ibuprofen, meloxicam, Celebrex, naproxen, indomethacin, nabumetone Stomach pain, heart burn, nausea, ulcer May need a PPI Must take with food OK to use acetaminophen Must avoid OTC NSAIDs Warning signs: bloody emesis, black tarry stool

14. DMARDS Disease modifying anti-rheumatic drugs Most common methotrexate Azathiprine, cyclosporin, plaquenil (hydroxychloroquine), leflunomide and sulfasalzine are also used. None produce immediate relief-1-3 months for relief to start Corticosteroids are often used as a bridge All decrease immune system-diligent evaluation of infection!

15. Methotrexate MTX may be added if NSAID not sufficient Given once weekly PO or SQ NO LIVE VIRUS VACCINES Anti-nausea med/ small snacks or sips of water to help with nausea May experience fatigue-rest break or nap can help Adolescents: must avoid alcohol and pregnancy!! TWO FORMS OF BIRTH CONTROL IF SEXUALLY ACTIVE! Warning signs: new infection beyond the common cold that includes a fever of 101 or more-must be evaluated by pediatrician!

16. Biologics Orencia, Humira, Enbrel, Remicade, Kineret, Rituxan, Actemra Biologics decrease inflammation by targeting certain cells in the immune system Anti TNF , interleukin-1, interleukin-6 Given by injection daily, weekly, Q 2 weeks, or infusion Monthly or Q 8 weeks. Decrease immune system-diligent evaluation of infection! No Live virus vaccines! Side effects: injection site reaction, headache, URI�s

17. Corticosteroids Very effective at treating inflammation, but are limited by side effects Prednisone oral dosing: taken daily, at lowest effective dose, for shortest possible time IV solumedrol given in high doses to treat flares Steroid joint injections: less systemic side effects, can work for up to 3-12 months

18. PT/OT Physical and occupational therapy are vital for many kids with JIA Unfortunately, appointments are often only available during school hours Kids with JIA are encouraged to stay active Kids with JIA are often deconditioned PE is encouraged, with self limitation accommodation. Kids are encouraged to be as active as they can be, but should be allowed to limit their own activity during a flare Encourage them to use activities learned at PT when not able to participate in PE activities

19. A Parent�s Perspective Chris Nieto

20. Implications For the School Nurse Medication side effects can be a challenge GI: stomach pain, nausea Allow child to eat a small snack, or sip on water, use anti-nausea meds, PPI Headache Side effect of MTX and biologics Ok to use acetaminophen while on NSAIDS/MTX/biologics Fatigue Allow rest period, or nap for young child Infection Diligent evaluation of infection with fever of 101 or more for kids on Biologics, steroids, or DMARDs like methotrexate

21. Vaccines Influenza vaccine is recommended for all patients taking biologics& DMARDs Avoid �Flu Mist�, must take injection No live virus vaccines for kids on Methotrexate or biologics Children on methotrexate must notify rheumatologist for exposure to active varicella infection

22. Mobility During a flare joints become inflamed, painful and child may develop a limp Allow student to leave class 5 minutes early, or arrive 5 minutes late to avoid crowded hallways Allow student to use elevator Allow student to park, or be dropped off close to the building Allow student to group classes close together Students with knee or hip arthritis may have difficulty sitting �crisscross applesauce� PE is important as are recreational activities-allow the child to limit their own activity- this is part of learning autonomy

23. Stiffness Morning stiffness can last minutes to hours-may cause them to be late to school PE may be better scheduled later in the day Students may be scheduled to start classes later Allow use of heat or ice pack Arrange for short bus trip due to morning stiffness Allow student to sit at the side or back of the room to allow them to get up to stretch Excuse student when late to school due to morning stiffness

24. Social Issues Many kids with arthritis don�t want others to know, and don�t want to be seen as different Often kids with arthritis can feel isolated Families can arrange for someone to share with the class if the student feels comfortable with this. Teachers should have same expectation for accomplishment and success, it just may take longer for a child with arthritis

25. Difficulty Writing Kids with finger and wrist involvement may have difficulty with writing assignments and tests. They may need longer time to finish writing assignments and tests May need assistance with note taking May need to have shorter homework assignments if appropriate Using foam grips for pencil/pens and gel or felt tip pens which are easier to use May need to use keyboard, or iPad

26. Fatigue Fatigue is one of the common complaints for kids with arthritis Not only is it a symptom of the disease, it is also a common side effect of many of the treatments Kids may need to have a rest period or nap for younger children May not be able to make up all of the work they have missed- may need shortened assignments May need to arrive late, or leave early

27. Flares and Illness Arthritis is characterized by periods of remission when the child is feeling well, followed by periods of flare. When the child develops an infection, they may also experience a flare of arthritis symptoms. May need to miss school during a flare, and are prone to more frequent infections: Excuse absences without a doctors note each time

28. Pain management Chronic pain is something kids with JIA have to learn to deal with Exercise: stretching and moving can help Medications can be used such as acetaminophen, or RX meds Heat or ice can be helpful, topical creams Distraction techniques: light massage, positive thinking, doing something they enjoy

29. References Wagner-Weiner, L. (2008). Pediatric rheumatology for the adult rheumatologist. JCR: Journal of Clinical Rheumatology,14(2), 109-119. Hashkes, P.J., Laxer, L.M. (2005). Medical treatment of juvenile idiopathic arthritis. JAMA, 294 (13), 1673-1684. Huff, C. (2008) Raising a child with arthritis: a parent�s guide (R. Vehe, MD, ed.). Atlanta, Georgia: The Arthritis Foundation.