1. Welcome to the
Club
Elizabeth Candell Chalom, MD
Director, Pediatric Rheumatology
Saint Barnabas Medical Center
Livingston, NJ

2. My Child has a
Rheumatic Disease
WHY????
No One Knows

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. 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. 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. 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. Relative Prevalences of
Chronic Diseases
 JIA 2.0 / 1000
 Childhood Diabetes 1.0 / 1000
 Cystic Fibrosis 0.4 / 1000
(in Caucasians)

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. Eye Disease
All 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. 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. 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. 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. 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. 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. 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. Dermatomyositis
Important 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. Scleroderma
 Systemic Sclerosis
 Localized scleroderma
 Linear scleroderma
 Morphea

18. Treatments for Rheumatologic
Disorders
Medication
Physical / Occupational
Therapy
Surgery

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. 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. 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. 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. Biologics
Genetically 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. 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. 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. Exercise in Rheumatologic
Disorders
 More and more
information has recently
come out showing the
importance of exercise in
rheumatologic diseases

27. Goals of Exercise
Increase flexibility, muscle strength
and endurance
Increase stamina for daily activities
Increase sense of well being

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. 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. 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. 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. 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. 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 visits
It’s not fair!!!

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. Arthritis Foundation
Meeting other children “in the same boat” can
be extremely helpful
 Conferences
 Camps
 JA Activities
Get involved

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!