JIA, JRA, Pediatric rheumatic disease, juvenile rheumatoid arthritis

1. Dr. Virendra Kumar Gupta
MD Pediatrics,MIAP
Fellowship In pediatric Gastroentero-Hepatology & Liver Transplantation
Assistant Professor
Institute of Paediatric Gastroenterology
Nims University Jaipur

2.  It’s not a single disease, but a group of
related, genetically heterogeneous,
phenotypically diverse immunoinflammatory
disorders affecting joints and other
structures, possibly activated by contact with
an external antigen or antigens.
 Also named-
Juvenile Rheumatoid Arthritis (JRA)
Juvenile chronic Arthritis(JCA)

3.  unknown
 combination of factors
◦ environment (infection, trauma, stress)
◦ autoimmunity
◦ immunogenetic

4.  Arthritis
 Chronic synovial inflammation leading to
bone/joint erosion
 Morning stiffness, limp, or falling often
 Easy fatigability
 Joint swelling
 Minimal pain
 Joint NEVER red or exquisitely tender
 Alteration of activities
 Loss of function

5.  Age: < 16y at time of onset
 Duration: at least 6 weeks
 Arthritis in one or more joints
 Exclusion of other rheumatologic d/o
Subgroup named after 6 months
◦ Pauciarticular(Oligoarticular): 4 or fewer joints
◦ Polyarticular: 5 or more joints
◦ Systemic: arthritis with fever

6.  Oligoarticular
◦ Persistent
◦ Extended (>4 joints after 6 months)
 RF Positive Polyarticular
 RF Negative Polyarticular
 Systemic Onset
 Psoriatic Arthritis
 Enthesitis-related Arthritis
 Other

7. Juvenile Idiopathic Arthritis (JIA)
1-oligoarticular
persistent
extended
2-polyarticular RF+
3-polyarticular RF-
4-systemic
5-psoriatic arthritis
6-enthesitis-related arthritis
7-undifferentiated arthritis

8. ◦ 4 or fewer joints
 Large joints: knees,
ankles, wrists
 NOT HIP
◦ Serology
 Positive ANA
 Negative RF
◦ Main morbidity
 ASYMPTOMATIC
ANTERIOR UVEITIS
 (assoicated with
positive ANA)
 Can lead to blindness

11.  5 or more involved joints
◦ Small and large joints
◦ PIP, MCP, wrist
 Rheumatoid nodules
 ANA may be positive
 RF may be + or –
◦ If + then worse prognosis

14.  Males = Females
 Quotidian fever
 Arthritis
 Visceral involvement
◦ HSM
◦ LAD
◦ Serositis
 Leukocytosis
 Rash
◦ Evanescent, salmon
colored
 ANA and RF negative

15. •“Fleeting salmon-color rash”
•Macular or wheal-like
•Not pruritic
•Irregular
•May coalesce with fever

16.  Enthesitis-related JIA
 Enthesis: insertion of ligaments and tendons
into bone
 Asymmetrical arthritis affected 4 or fewer
joints
 Male predominance

17.  Enthesitis of axial skeleton and sacroiliac joints.
◦ Present with back pain
◦ Loss of lumbosacral mobility
 Oligoarthritis of joints of lower extremities
 Common presentation
◦ Male with back pain, morning stiffness that is relieved w/
exercise
 Labs
◦ HLA-B27 positive
◦ Increased ESR
◦ ANA and RF are NEGATIVE
 Radiology
◦ Bamboo Spine
 Treatment
◦ NSAIDS, Sulfasalazine, Mtx

19.  generalized or local growth disturbances
 delayed puberty
 pericarditis, myocarditis, rarely endocarditis
 pleural effusion,
 pneumonitis, pulmonary fibrosis- CAPLAN’s
syndrome
 hepatitis
 Renal involvement is rare

20.  supportive not curative
 involves multidisciplinary team approach
 goals:
◦ to suppress articular and/or systemic
inflammation with as little risk as possible
◦ to maintain function/prevent disabilities
◦ to foster normal psychological and social
development
 heterogenity of disease mandates
individualization

21. heat: analgesia
muscle relaxation
splinting: provide joint rest
maintain functional position
correct deformities
exercise: passive, active assisted and
active range of motion
general conditioning
rest

22.  NSAID’S
 Glucocorticoids
 DMARD
 Anticytokine therapy.
 Chemo theraputic agents.
 Autologous stem cell transplant in severe
systemic JIA.

23.  Pros:
◦ Analgesic, Antipyretic,
Anti-inflammatory
 Cons:
◦ Don’t alter disease
progression
◦ Ineffective in Erosive
disease
• GI/Ulcers
• Hepatotoxicity
• Nephrotoxicity
• AIN
• Bleeding –
antiplatelet
• Rash
• Aseptic meningitis

24. Medications Doses
(mg/kg)
Side effects
Aspirin 50-120 Stomack pain, vomiting,
gastrointestinal
bleedings, headache,
blood in the urine, fluid
retention, thinning and
scarring of the skin
(especially with
naproxen), stomach
ulcer (aspirin).
Ibuprofen 10-30
Tolmentin 10-15
Naproxen 5-20

25. Medications Doses
(mg/kg)
Side effects
Hydroxychlo-
roquine
(Plaquenil)
5-7 Upset stomach, skin
rash and a eye
damage. A child who
takes this drug should
have his/her eyes
examined at least
every six months by
an ophthalmologist
Sulfasalazine
(Azulfadine)

26. Medications Doses(weekly,
depending from
body weight )
Side effects
Auranofin,
Ridaura,
Myochrysine
Solganol
20 kg – 10 mg
30 kg – 20 mg
40 kg – 30 mg
50 kg – 40 mg
> 50 kg – 50 mg
Skin rash,
mouth sores,
kidney
problems, a low
blood count or
anemia

27. Medications Week of
treatme
nt
Doses
(mg/
kg)
Side effects
Penicillamine
(DePen,
Cuprimine)
0-2
2-4
4-6
6-8
10-14
25-50
50-100
100
100-150
150-200
Diarrhea, skin rash,
low blood counts,
nausea or vomiting,
stomach pain, loss
of appetite, swollen
glands, unusual
bleeding or bruising

28. Medications Doses Side effects
Methotrexate
(Rbeumatrex)
Azathioprine
(Imuran)
Cyclophospha
mide
(Cytoxan)
Typically 7.5 to
25 mg a week
Loss of appetite,
nausea or
vomiting, skin
rash, unusual
bleeding or
bruising,
tiredness or
weakness,
sterility.

29.  Biologic Agents, which blocks the protein
TNF
Etanercept (Enbrel)
Infliximab (Remicade)
 Glucocorticoid Drugs (Dexamethasone,
Methylprednisolone, Cortef, Prednisolone
and Prednisone)
 Analgesics (acetaminophen [Tylenol,
Panadol], tramadol [Ultram])

30.  Therapeutic exercises
 Sports and Recreational Activities
 Splints

31.  Morning Stiffness Relief
 Diet
 Eye Care
 Dental Care
 Surgery

32.  Long Remission
◦ 10%
 Intermittent Disease
◦ 15-30%
 Progressive Disease

33.  chronic disease which cannot be cured
 characterized by flares and remissions
 after 10 years or more:
◦ 31%-55% persistent active disease
◦ 31% (9% - 48%) Steinbrocker Class III and IV

34.  pauciarticular
◦ long duration of active disease
◦ conversion to polyarticular disease (30%)
◦ chronic uveitis
 polyarticular
◦ long duration of active disease
◦ articular erosions
◦ RF positivity/rheumatoid nodules
 systemic
◦ conversion to polyarticular disease (25-50%)