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JIA 101: Welcome to the Club


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JIA 101: Welcome to the Club

  1. 1. Welcome to the Club Elizabeth Candell Chalom, MDDirector, Pediatric Rheumatology Saint Barnabas Medical Center Livingston, NJ
  2. 2. My Child has aRheumatic Disease WHY???? No One Knows
  3. 3. Genetic??? Autoimmune/rheumatologic diseases tend to run in families, but are not strictly genetic. Identical twin studies show less than 50% concordance rates for most rheumatic disorders. Genetic predisposition to autoimmune diseases.
  4. 4. Diagnosis Rheumatologic diseases are very difficult to diagnose!! Very often they are diagnoses of exclusion. They must be watched over time -- almost never diagnosed at the first visit. Very frustrating for parent, patient and physician.
  5. 5. Unpredictable Course The natural course of rheumatologic disorders is one of exacerbations and remissions. Children can have great days followed by terrible days, which are then followed by great days. Medicines which seem to work well at one point may later seem to have no effect.
  6. 6. Unpredictable Prognosis Prognosis is very variable, with a wide range of possibilities. Although certain labs (RF, HLA typing) increase the likelihood of a given prognosis, there is still a great deal of variation. Few physicians will commit and give a prognosis.
  7. 7. Relative Prevalences of Chronic Diseases JIA 2.0 / 1000 Childhood Diabetes 1.0 / 1000 Cystic Fibrosis 0.4 / 1000 (in Caucasians)
  8. 8. PATHOGENESIS The key is inflammation Inflammation in the joints: thickening of the synovium (cells lining the joint) and increased joint fluid (swelling)  Over a long period of time may lead to joint damage Inflammation in the muscles (myositis), blood vessels (vasculitis), around the heart or lungs (serositis).
  9. 9. Eye DiseaseAll children with rheumatic disorders should have their eyes checked frequently. Uveitis can be seen in JIA. ---Often asymptomatic, until vision loss occurs. Lupus,Vasculitis, Behcet’s, etc. can affect the eyes. Medications (steroids, plaquenil) can affect the eyes.
  10. 10. Eye Exams Children with oligioiarticular JIA, or ANA+ polyarticular JIA should have their eyes checked every 3 months. Children with ANA- polyarticular JIA and lupus should have eye exams every 6 months. Children taking steroids (JIA, lupus, dermatomyositis, etc.) should have eye exams every 6 months.
  11. 11. Diagnostic Criteria for JIA Age at onset < 16 years Presence of arthritis (joint swelling, loss of motion) Duration of 6 weeks or longer Onset type classified in the first 6 months Exclusion of other forms of juvenile arthritis
  12. 12. Types of JIA Oligioarticular JIA: affects less than 5 joints Polyarticular JIA: affects 5 or more joints ERA/Sponduloarthropathies Psoriatic Arthritis Systemic onset JIA: high, spiking fevers; rash; can have swollen glands, heart involvement, liver involvement.
  13. 13. Definition of SLE Multisystem, autoimmune disease. Antibodies attack various components of the cell nucleus. Wide variety of clinical manifestations. Four of the following 11 criteria, developed by the American College of Rheumatology, must be met to classify a patient with systemic lupus erythematosis (SLE):
  14. 14. Criteria for SLE Malar (butterfly) rash  Arthritis: pain & swelling in the joints Discoid rash  Neurological disease: Photosensitivity seizures or hallucinations Oral or nasal painless  Serositis: fluid around the ulcers heart or lungs Blood disease: anemia  Autoantibodies to either: (↓hemoglobin) or ↓white dsDNA or Sm nuclear blood cells or ↓platelets antigen, or antiphosphllipid Kidney disease: protein antibodies or blood in the urine  Positive ANA
  15. 15. Dermatomyositis Inflammation of the muscles can cause significant weakness Rash: most often over the knuckles, around the eyes, and on the chest. Can also see on elbows and knees Labs often show ↑CPK and ↑ aldolase (muscle enzymes)
  16. 16. DermatomyositisImportant to stretch but not to overuse muscles early on When muscles are inflamed, they can become tight and stiff. Stretching helps prevent contractures (permanent shortening of the muscles). Overuse and stressing the muscles can increase inflammation
  17. 17. Scleroderma Systemic Sclerosis Localized scleroderma  Linear scleroderma  Morphea
  18. 18. Treatments for Rheumatologic DisordersMedicationPhysical / Occupational TherapySurgery
  19. 19. Medications NSAIDS: Decrease pain and inflammation  Naprosyn, Relafen, Daypro, Voltaren, Indocin, etc.  Cox-2 inhibitors: Celebrex Meloxicam (mostly)  1st line treatment, take 4-6 weeks for full effect Main side effect: GI upset Can affect: liver, kidneys, cell counts
  20. 20. Medications (cont.) Steroids  Often needed to control systemic symptoms (fever, rash, myositis, etc.)  Sometimes needed to control joint symptoms  Can be taken by mouth, given intravenously, or injected into the joints  Systemic steroids work well but cause many significant side effects  Joint injections cause minimal side effects
  21. 21. Medications (continued) DMARDs (disease modifying anti-rheumatic drugs)  Methotrexate- most common second line agent for inflammatory arthritis and dermatomyositis  Sulfasalazine- especially good for ERA/spondyloarthropathies  Hydroxychloroquine-good for skin disease in lupus and dermatomyositis, helps prevent lupus flares  Cellcept- good for lupus, especially renal disease
  22. 22. What’s New in Rheumatic Medications Almost all DMARDS were borrowed from other specialties Gold for pulmonary TB Antimalarials (Plaquenil) Sulfasalazine for IBD Penacillamine for Wilsons and Cystinuria Methotrexate and Cytoxan for cancer
  23. 23. BiologicsGenetically engineered to specifically inhibit inflammatory cells or proteins TNF inhibitors (Enbrel, Remicaide, Humira) IL-1 inhibitors (Kineret,Ilaris) IL-6 Inhibitor (Actemra) Co-stimulation Blocker (Orencia) B-cell inhibitors (Rituxan, Benlysta)
  24. 24. Biologics (cont.) All are degraded by the acid in the stomach, so none can be taken by mouth. Some must be given IV, others can be given SQ
  25. 25. New Medications or Diets Almost every week, a new drug or vitamin is described as the “cure” for arthritis. Most of the time, there is no scientific evidence to support these claims. When controlled studies are actually done, very few of these new “cures” show any efficacy. Holistic therapies have not been able to replace conventional treatments.
  26. 26. Exercise in Rheumatologic Disorders  More and more information has recently come out showing the importance of exercise in rheumatologic diseases
  27. 27. Goals of ExerciseIncrease flexibility, muscle strength and enduranceIncrease stamina for daily activitiesIncrease sense of well being
  28. 28. Conditioning ProgramsConditioning programs have been shown to decrease the number of swollen joints, increase range of motion, and decrease perception of pain in children with arthritis
  29. 29. Why Children with Arthritis Don’t ExerciseFear of discomfortFear of not playing sports wellParental overprotection  Fear of injury or disease exacerbation
  30. 30. What is a Reasonable Compromise? While joints/muscles are actively inflamed, gentle exercise/stretching are encouraged. Non-contact sports are allowed, as long as the child understands he/she should not overdo it. If a joint begins hurting during exercise, he/she is overdoing it.
  31. 31. Tips for Exercise in Children with Arthritis Stretch  especially hip flexors, hamstrings, dorsiflexors Strengthen  helps support the joints Aerobic conditioning  need to start conditioning earlier
  32. 32. Day to Day Problems with Chronic Illness Compliance  Once children start feeling better, they think they don’t need medications anymore  Side effects of medications: • Injections can be painful • Steroids cause weight gain • Methotrexate can cause nausea Anger
  33. 33. I Don’t Want to be Sick!Children don’t want to be different from their friends Most friends don’t take medications every day or every week Most friends don’t have days when they feel lousy for seemingly no reason Most friends don’t need frequent lab tests and doctor’s visitsIt’s not fair!!!
  34. 34. What Parents Can Do Acknowledge that it is not fair, but be positive Allow children to take as much control over their illness as possible Treat child with rheumatic disorder the same as siblings Yes you can!  Emphasize what they can do, not what they can’t do  Allow them to try, even if you think they may fail
  35. 35. Arthritis FoundationMeeting other children “in the same boat” can be extremely helpful Conferences Camps JA ActivitiesGet involved
  36. 36. Problems with Rheumatic Disorders We still don’t understand rheumatic disorders We don’t know the causes Our medications help control symptoms, but there can be side effects We NEED a cure!