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INTRODUCTION
EPIDEMIOLOGY
DIAGNOSIS
TREATMENT
CLINICAL OUTCOME
FUTURE
TREATMENTS
ASSUMPTIONS
IN PREPARING TALK
Cause
unknown
Associated with
– abnormal immunoregulation
– abnormal cytokine
production
– ? latent viral infection
JRA- Introduction
Requirements for
diagnosis
combination of data
from
– history
– physical examination
– laboratory testing
JRA- Introduction
5-10% JRA
rheumatoid factor
positive
poly articular
beginning in
adolescence
resembles adult
onset RA
JRA- Epidemiology
U.S.A prevalence
70,000-100,000 case in a
population under 16
35,000-50,000 people over
age16 have active JRA
JRA- Epidemiology
Diagnostic criteria
onset at less than 16 years of age
persistent arthritis in one or more
joints for at least 6 weeks.
exclusion of other types of
childhood arthritis
JRA- Diagnosis
JRA rash
– pale pink
– blanching
– characterised by small macules
on maculopapules
– transient (minutes to a few
hours)
– non pruritic in 95% of cases
– commonly seen on the trunk
JRA- Diagnosis
Other features of
sJRA
• growth delay • anemia
• osteopenia • leucocytosis
• diffuse lymphadenopathy • thrombocytosis
• hepatosplenomegaly • elevated acute
• pericarditis phase reactants
• pleuritis
JRA- Diagnosis
complications of sJRA
– pericadial tamponade
– severe vasculits
po JRA
– two distinct
diseases
• RF positive
• RF negative
JRA- Diagnosis
pa JRA
– eye involvement ( rare)
- very sudden in onset
- painfull red eyes
- chronic complications less likely to
occur
JRA- Diagnosis
JRA- Diagnosis
JRA- Diagnosis
JRA- Diagnosis
JRA- Treatment
Glucocorticoids
Immunosuppressive therapy
Experimental therapy
Orthopedic surgery
Preventive surgery
Reconstructive surgery
JRA- Treatment
Care involves
- family
- interdisciplinary health care
team
JRA- Treatment
treatment
– diagnosis to
satisfy
- patient
- parents
- extended family
- health care team
JRA- Treatment
theraupetic goal
- relieving symptoms
- maintaining joint motion
- maintaining muscle strength
- preventing joint damage
- minimizing joint damage
- maximizing functional status
- promoting positive self image
- encouraging positive/ productive family
dynamics
JRA- Treatment
treatment programme
- physical
- social
- pharmacologic
- surgical
JRA- Treatment
physical
- range of motion
exercises
- splints
- joint protection
techniques
JRA- Treatment
social
- psychosocial adjustement
- school adaptation
- vocational issues
JRA- Treatment
pharmacologic
- articular
- ocular
- other manifestations
JRA- Treatment
NSAIDS
- majority respond in two weeks
- 25% do not respond until 8 to 12 weeks
(average time 4 weeks)
- switch over to another NSAID if one type is
not giving good response
- Aspirin dose 75-90mg/kg/day
- Naproxen 15mg/kg/day
- Ibuprofen 35mg/kg/day
- Indomethacin 2-3mg/kg/day
- Tolmetin sodium 20-30mg/kg/day
JRA- Treatment
injectible gold
- 5mg test dose
- 0.75-1mg/kg weekly for 20 weeks
- maintenance every 2 weeks for 3
months
- every 3 week for 3 months
- every 4 weeks
- 50 –60% of patients improve
- high frequency of side effects
JRA- Treatment
sulphasalazine
- encouraging results
- 40-60mg/kg/day
- A void in sJRA
glucocoticoids
- used in severe and life threatening
complications of sJRA
- used in resistant JRA
- often used in combination with other
drugs
- predisone dose 0.1-1mg/kg/day
JRA- Treatment
Intraarticular steroids
– in pa JRA
JRA- Treatment
IMMUNO SUPPRESSIVE
THERAPY
– chlorambucil
– cyclophosphamide
– azathioprime
JRA- Treatment
IMMUNO SUPPRESSIVE
THERAPY
– Problems
• leucopenia
• bone marrow supression
• malignancy
• mutagenic effects
• sterility and amenorhoea
JRA- Treatment
eternecept
– in patients refractory to or
resistant to methotrexate
– dose 0.4mg/kg/dose (maximum
25mg twice weekly for three
months)
JRA- Treatment
Ocular
– managed by experienced ophthalmologist
- early detection
- topical corticosteroid
- dilating agents
- frequent follow up
• severe cases- systemic/sub-tenon steroid injection
- chlorambucil
- cyclophsophamide
- NSAIDS
JRA- Treatment
surgical
- synovectomy
- tenosynovectomy
- re-constructive surgery
JRA- Outcome