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JUVENILE RHEUMATOID ARTHRITIS (JRA)
(JUVENILE CHRONIC ARTHRITIS-JCA)
Omondi Oyoo FACR
DIAGNOSIS AND TREATMENT
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Our task now is
not to fix theblame for the past,
but to fix the
course for the
future
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TOPICS
INTRODUCTION
EPIDEMIOLOGY
DIAGNOSIS
TREATMENT
CLINICAL OUTCOME
FUTURE
TREATMENTS
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ASSUMPTIONS
IN PREPARING TALK
AUDIENCE PREDOMINANTLY NON
RHEUMATOLOGISTS
SINCE THE TALK IS IN THE MORNING, AUDIENCE
IS PHYSICALLY STRONG AND MENTALLY ALERT
TALK WILL BE BOTH QUANTITATIVE AND
QUALITATIVE COVERING BOTH BASICS AND STRESSING KEY CONCEPTS
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JRA- Introduction
most common childhood
arthritis
common childhood
chronic illness
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JRA- Introduction
Requirements for
diagnosiscombination of data
from
history
physical examination
laboratory testing
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JRA- Introduction
Different from adult RA
inclinical course
immunogenetic
associationfunctional out come
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JRA- Introduction
5-10% JRA
rheumatoid factor
positive
poly articular
beginning in
adolescence
resembles adult
onset RA
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JRA- Epidemiology
overall 10-20 cases per 100,000
population
between 57-113/1000 childrenyounger than 16 years in the
U.S.A. (urban white)
26/100,000 urban blacks U.S.ASweden 26/100,000
Finland 18.2 cases/ 1000
population
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JRA- Epidemiology
50% of JRA have disease that
persist into adulthood
age of onset 1-3 years
girls account for majority ofpatients (twice as often as
boys)
44% concordance rate inidentical twins
4% concordance rate in
dizygotic twins
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JRA- Epidemiology
U.S.A prevalence70,000-100,000 case in a
population under 16
35,000-50,000 people over
age16 have active JRA
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JRA- Epidemiology
Comparison with other diseases
same number of children as juvenilediabetes mellitus
4 times more than sickle cell anemia
10 times more than hemophilia,
acute lymphocitic leukemia, chronic
renal failure or muscular dystrophy
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JRA- Diagnosis
AMERICAN COLLEGE OF RHEUMATOLOGY DIAGNOSTIC CRITERIA
FOR CLASSIFICATION OF JUVENILE RHEUMATOID ARTHRITIS
_____________________________________________________________
Age at onset younger than 16years
Arthritis in one or more joints defined as swelling or effusion, or the presence of
two or more of the following signs: limitation of range of motion, tenderness or pain
on motion, and increased heat
Duration of disease ofu 6 weeks
Type of onset of disease during the first 6 months classified as
-Polyarthritis- 5 joints or more
-Oligoarthrits- 4 joints or fewer-Systemic disease with arthritis and intermittent fever
Exclusion of other forms of juvenile arthritis
______________________________________________________________
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JRA- Diagnosis
Four key points
arthritis
swelling effusion
limitation of motion
- tenderness
- pain on motion- joint warmth
(arthralgia is not sufficient to satisfy
this definition )
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JRA- Diagnosis
Four key points(cont)
arthritis must persist for at
least 6 weeks (ACR) (EULAR- 12weeks)
other causes of chronic arthritis
must be excluded.no specific laboratory or other
test can establish the diagnosis
of JRA
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JRA- Diagnosis
Sub division of JRA
Systemic (sJRA) 10%
Polyarticular (po JRA)
30%
Pauciarticular (pa JRA) 60%
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JRA- Diagnosis
Systemic onset JRA
(sJRA)
10% of childhood JRA
peak age 1-6 years
boys and girls equally
affected daily/twice daily
intermittent fever
characteristic JRA rash
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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
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JRA- Diagnosis
Other features of
sJRA growth delay
osteopenia
diffuse lymphadenopathy
hepatosplenomegaly pericarditis
pleuritis
anemia
leucocytosis
thrombocytosis
elevated acutephase reactants
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JRA- Diagnosis
rare features in sJRA
uveitis
positive rheumatoid
factor
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JRA- Diagnosis
complications of sJRA
pericadial tamponade
severe vasculits
prognosis determined by severity
of arthritis (may develop with feverand rash or weeks or months after
onset of fever)
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JRA- Diagnosis
Poly articular onset (po JRA)
arthritis in five or more joints
seen in 30- 40% of patients with
JRA
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JRA- Diagnosis
po JRA two distinctdiseases
RF positive
RF negative
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JRA- Diagnosis
RF positive po JRA
almost always girls later disease onset (u8 years old)
HLA DR 4 positive
symmetric small joint invovement
risk of developing nodules- erosions
- poor functional outcome
resembles adult onset RA
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JRA- Diagnosis
po JRA clinical manifestations fatigue
anorexia
growth retardation
delay in sexual maturation
osteopenia may develop at any age
girls outnumber boys 3 to 1
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JRA- Diagnosis
Pauciarticular JRA (pa JRA) arthritis in four or fever joints
two distinct clinical groups
-early onset
-late onset
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JRA- Diagnosis
Early onset pa JRA
- 1-5 years old- girls : boys 4 : 1
- ANA positive
- chronic eye inflammation (30-50% of
cases)- best overall articular outcome
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JRA- Diagnosis
Early onset pa JRA eye
involvement
- in 30 to 50% of patients- involves anteria chamber
- no or minimal symptoms in 80% of
affected children
- severe changes include- corneal clouding
- cataracts
- glaucoma
- partial or total visual loss
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JRA- Diagnosis
Late onset pa JRA
more common in boys 50% HLA-B27 positive
enthesitis/ tendinitis
arthritis large joints (shoulder hips and knees)- the spine
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JRA- Diagnosis
pa JRA
eye involvement ( rare)
- very sudden in onset
- painfull red eyes
- chronic complications less likely tooccur
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JRA- Diagnosis
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JRA- Diagnosis
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JRA- Diagnosis
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JRA- Treatment
Unique Pediatric Concerns in
treating JRA
Patients are intimate members of
family
- in house nurse, PT, OT, psychologist
- significant impact on sibs and
parental relationship- maternal depression, separation/
divorce, monetary concerns may
distract from medical care
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JRA- Treatment
Unique Pediatric Concerns in treating
JRA (cont)
Patients have a full time career- school!
Growth/nutrition must be monitored
Adolescence changes everything- denial
- sex, drugs, rock & roll
sports rule- proactive enrollment in non-contact sports
- alternative roles
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JRA- Treatment
MANAGEMENT OF JUVENILE RHEUMATIOD ARTHRITIS(cont)
Glucocorticoids
Immunosuppressive therapy
Experimentaltherapy
Orthopedic surgery
Preventive surgery
Reconstructive surgery
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JRA- Treatment
Care involves- family
- interdisciplinary health care
team
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JRA- Treatment
comprehensive care addressing- education
- peer relationship
- self esteem
- social adjustment
- family dynamics- vocational planning
- financial concerns
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JRA- Treatment
treatment
diagnosis to
satisfy- patient
- parents
- extended family
- health care team
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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
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JRA- Treatment
treatment programme- physical
- social
- pharmacologic
- surgical
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JRA- Treatment
physical- range of motion
exercises- splints
- joint protection
techniques
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JRA- Treatment
social
- psychosocial adjustement- school adaptation
- vocational issues
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JRA- Treatment
pharmacologic
- articular- ocular
- other manifestations
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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
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JRA- Treatment
problems associated with
NSAIDS- anorexia
- abdominal pains
- coagulation disorders
- lever function- renal function
- cns symptoms
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JRA- Treatment
Methotrexate(MTX) and NSAIDS
- in two thirds of patients with JRA- 10mg/m2 BSA weekly
- mainly for sJRAor po JRA
- response 70-80%
non responders increase methotrexate dose to
1mg/kg/week (maximum 50mg/week)
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JRA- Treatment
problems with methotrexate- oral ulcers
- nausea
- decreased appetite
- abdominal pains
- pulmonary complications
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JRA- Treatment
oral gold 0.15mg/kg/day
d-penicillamine- 10mg/kg/day
hydroxychloroquine 5-7mg/kg/day
efficacy
similar to
placebo
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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
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JRA- Treatment
sulphasalazine
- encouraging results- 40-60mg/kg/day
- A void in sJRA
intravenous gamma globulins (IVGG) promising results in po JRA
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JRA- Treatment
glucocoticoids
- used in severe and life threateningcomplications of sJRA
- used in resistant JRA
- often used in combination with other
drugs- predisone dose 0.1-1mg/kg/day
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JRA- Treatment
INDICATIONS FOR SYSTEMIC STEROIDS IN sJRA
Macrophage Activation Syndrome
CNS involvement (rare) seizures, lethargy,
meningismus
Stridor with cricoarytenoid arthritis
Interstitial pulmonary disease
Myocarditis
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JRA- Treatment
INDICATIONS FOR SYSTEMIC STEROIDS IN
sJRA(cont)
Moderate to severe pericarditis with
impairement of cardiac function
Secondary amyloidosis
Severe anemia
Failure of standard therapy to relieve
symptoms sufficiently to allow comfortable
function
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JRA- Treatment
Intraarticular steroids
in pa JRA
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JRA- Treatment
IMMUNO SUPPRESSIVE
THERAPY
chlorambucil
cyclophosphamide
azathioprime
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JRA- Treatment
IMMUNO SUPPRESSIVE THERAPY
Used in secondary amyloidosis
- life threatening illness
- un remitting progression of arthritis and
disability
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JRA- Treatment
IMMUNO SUPPRESSIVE
THERAPY
Problems
leucopenia
bone marrow supression
malignancy mutagenic effects
sterility and amenorhoea
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JRA- Treatment
eternecept
in patients refractory to or
resistant to methotrexate
dose 0.4mg/kg/dose (maximum
25mg twice weekly for threemonths)
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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
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JRA- Treatment
other extra articular manifestation
- poor linear growth especially in sJRAand po JRA- growth hormone therapy
protein calorie malnutrition due to
- poor appetite
- catabolic drugs
- physical inactivity
- inflammatory medications
dietary intervention
- adequate caloric and protein intake
- nocturnal enteral nasogastric supplemental feeding
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JRA- Treatment
surgical
- synovectomy- tenosynovectomy
- re-constructive surgery
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JRA- Outcome
satisfactory for properly managed children is
approximately 85%
about 30% of JRA patients have functional
limitation after 10 or more yearsmortality 0.29 1.1 /100 patients (3 14
times greater than the standardized U.S.
children population)
ocular 85% have normal visual acuity
15% with significant visual loss
10% blinded in at least one eye.
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JRA-Future Treatments
Anti TNF -E- standard or
elevated doses
Anti IL 6Anti IL 6 + Anti TNF -E
Stem cell transplant
Pulse therapy to induceremission
- IV steroids + IV Cytoxan + MTX
Other combinations
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The very best
way to predictthe future is to
create it.
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THANK YOU
FOR YOUR
ATTENTION