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Juvenile rheumatoidJuvenile rheumatoidarthritis (JRA)arthritis (JRA)
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BACKGROUNDBACKGROUND
Chronic Arthritis in Childhood isChronic Arthritis in Childhood is
characterized ascharacterized as
Juvenile Rheumatoid ArthritisJuvenile Rheumatoid Arthritis
(JRA)(JRA)
Age of onset < 16 years of age. Age of onset < 16 years of age.
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BACKGROUNDBACKGROUND
Pathogenesis and Etiology of JRA: MultiPathogenesis and Etiology of JRA: Multi--factorialfactorial
Genetic, Hormonal, ImmunologicGenetic, Hormonal, Immunologic
PathogenesisPathogenesis
Characterized by chronic inflammation of theCharacterized by chronic inflammation of the
synovium;synovium;
Presence of articular cartilage damage;Presence of articular cartilage damage;
Accompanied by extra Accompanied by extra--articular systemicarticular systemic
manifestations.manifestations.
Heterogeneity of JRAHeterogeneity of JRA
At least 3 primary types of onset of JRA: At least 3 primary types of onset of JRA:
Pauciarticular (Oligoarticular)Pauciarticular (Oligoarticular)
Polyarticular andPolyarticular and
SystemicSystemic
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BACKGROUNDBACKGROUND
PathogenesisPathogenesis (Continued)(Continued)
GeneticGenetic
Basis of immune distinction between self and nonBasis of immune distinction between self and non--self is the major histocompatibility complex (MHC)self is the major histocompatibility complex (MHC)that in humans is called the human leukocytethat in humans is called the human leukocyteantigen (HLA).antigen (HLA).
HLA system comprises a family of polymorphicHLA system comprises a family of polymorphicgenes located on the short arm of chromosome 6.genes located on the short arm of chromosome 6.
Polymorphisms of JRA suggest a nonPolymorphisms of JRA suggest a non--mendelianmendelianinheritance.inheritance.
Hormonal FactorsHormonal Factors
Differences in the sex ratio of JRA subtype onsetDifferences in the sex ratio of JRA subtype onset
PrePre--adolescent or postadolescent or post--adolescent peaksadolescent peaks
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BACKGROUNDBACKGROUND Immune MechanismsImmune Mechanisms
Disease process involves loss of tolerance towardsDisease process involves loss of tolerance towards
autoauto--antigensantigens pp chronic synovitis;chronic synovitis;
Production of autoProduction of auto--antibodies:antibodies:
Anti Anti--nuclear antibodies (ANA): associated withnuclear antibodies (ANA): associated with
increased risk of iridocyclitis (eye inflammation);increased risk of iridocyclitis (eye inflammation);
Rheumatoid factors (RF): autoRheumatoid factors (RF): auto--antibodies directedantibodies directed
against the Fc fragment of IgG (associated withagainst the Fc fragment of IgG (associated with
~10% of polyarticular JRA);~10% of polyarticular JRA);
Complement activation by circulating immuneComplement activation by circulating immunecomplexes may also contribute to the diseasecomplexes may also contribute to the disease
process.process.
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BACKGROUNDBACKGROUND
Immune MechanismsImmune Mechanisms (Continued)(Continued) CytokinesCytokines:: act on the immune system and other cellsact on the immune system and other cells
to initiate and sustain inflammation:to initiate and sustain inflammation:
Intercellular mediators:Intercellular mediators: InterleukinInterleukin--1 (IL1 (IL--1), IL1), IL--6,6,
and tumor necrosis factor and tumor necrosis factor--alpha (TNFalpha (TNF--ww););
Immunomodulatory cytokinesImmunomodulatory cytokines produced by Tproduced by T--
cellscellspp Interferon gamma (IFNInterferon gamma (IFN--), IL), IL--4, IL4, IL--2.2.
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CLASSIFICATION OF JRACLASSIFICATION OF JRA
ACR ( ACR ( American College of Rheumatology) American College of Rheumatology) CriteriaCriteria
Age at onset: < 16 years of age; Age at onset: < 16 years of age;
Arthritis Arthritis -- swelling or effusion or the presence of 2 or moreswelling or effusion or the presence of 2 or moreof the following signs:of the following signs:
Limitation of range of motion,Limitation of range of motion,
Tenderness or pain on motion andTenderness or pain on motion and Increased heat in one or more joints;Increased heat in one or more joints;
Duration of diseaseDuration of disease >> 6 weeks;6 weeks;
Onset type is defined by the type of disease in the first 6Onset type is defined by the type of disease in the first 6
months:months: Oligoarticular (Pauciarticular)Oligoarticular (Pauciarticular) < 5 inflamed joints;< 5 inflamed joints;
Polyarticular:Polyarticular: >> 5 inflamed joints;5 inflamed joints;
Systemic onset:Systemic onset: arthritis with characteristic fever.arthritis with characteristic fever.
Exclusion of other forms of childhood arthritis.Exclusion of other forms of childhood arthritis.
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General history of JRA includes the following:General history of JRA includes the following:
Disease onset is either insidious or abrupt, with morningDisease onset is either insidious or abrupt, with morning
stiffness and arthralgia during the day.stiffness and arthralgia during the day.
Their abilities to participate in physical education classesTheir abilities to participate in physical education classes
may reflect severity of the disease.may reflect severity of the disease.
Limping may be observed in individuals with more severeLimping may be observed in individuals with more severe
JRA; however, the presence of limping also raises theJRA; however, the presence of limping also raises thepossibility of trauma or another orthopedic problem.possibility of trauma or another orthopedic problem.
A preceding illness raises the possibility of infectious trigger A preceding illness raises the possibility of infectious trigger
of JRA or postinfectious arthritis.of JRA or postinfectious arthritis.
Very severe joint pain raises isn¶t typical for JRA and showsVery severe joint pain raises isn¶t typical for JRA and showsthe possibility of acute rheumatic fever (also suggested bythe possibility of acute rheumatic fever (also suggested by
migratory but not additive arthritis, with fevers).migratory but not additive arthritis, with fevers).
Weight loss without diarrhea may be observed in individualsWeight loss without diarrhea may be observed in individuals
with active JRA and sometimes associated with anorexia.with active JRA and sometimes associated with anorexia.
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CLINICAL MANIFESTATIONS of JRACLINICAL MANIFESTATIONS of JRA
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Characteristic
% Cases (F:M)
# Joints
Age at onset
Systemicinvolvement
Chronic Uveitis
RF/ANA
Prognosis
Polyarticular
30 (3:1)
> 5
Thruchildhood,peak 1-3 yr
Mild;unremitting
articularinvolvement
5%
10%/40-50%
Guarded tomoderately
good
Pauciarticular
60 (5:1)
< 4
Earlychildhood,peak 1-2 yr
None;uveitis (++)
5-15%
Rare/75-85%
Excellentexcept for
eyesight
Systemic
10 (1:1)
Variable
Thruchildhood,
no peak
Systemic self-limited; chronic
destructivearthritis ~50%
Rare
Rare/10%
Moderate topoor
JRA by the TypeJRA by the Type--of of--OnsetOnset
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ExtraExtra--Articular Manifestations of JRA Articular Manifestations of JRA
FeverRheumatoid rash
Rheumatoid nodulesHepatosplenomegalyLymphadenopathyChronic uveitisPericarditis
PleuritisAbdominal pain
Polyarticular
30%2
101055511
Pauciarticular
0%0
000
20000
Systemic
100%95
585701
352010
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Systemic Systemic- -onset JRAonset JRA is characterizedis characterized
by spiking fevers, typically occurring severalby spiking fevers, typically occurring several
times each daytimes each day Evanescent salmonEvanescent salmon--pink rash, often linear, is foundpink rash, often linear, is found
on the trunk and the extremities; this rash is worseon the trunk and the extremities; this rash is worsewith fever.with fever.
Hepatosplenomegaly.Hepatosplenomegaly. Lymphadenopathy.Lymphadenopathy.
Muscle tenderness to palpation.Muscle tenderness to palpation.
Ocular: Photophobia, in uveitis (usuallyOcular: Photophobia, in uveitis (usually
asymptomatic on onset), and synechiae (irregular asymptomatic on onset), and synechiae (irregular iris perimeter resulting from postinflammatoryiris perimeter resulting from postinflammatoryadhesions of iris to lens) may be foundadhesions of iris to lens) may be found
Cardiovascular: myocarditis occurs in individualsCardiovascular: myocarditis occurs in individuals
with systemic JRA.with systemic JRA.
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Pau ci ar tic ular for mPau ci ar tic ular for m is characterized by arthritisis characterized by arthritis
affecting 4 or fewer joints.affecting 4 or fewer joints.
Typically, large weightTypically, large weight--bearing joints, knees, andbearing joints, knees, andankles are affected.ankles are affected.
Involvement of a few small joints in the hands isInvolvement of a few small joints in the hands is
atypical and suggests eventual development of atypical and suggests eventual development of
polyarticular JRA.polyarticular JRA. Muscle atrophy, often of extensor musclesMuscle atrophy, often of extensor muscles
(vastus lateralis, quadriceps when knee affected)(vastus lateralis, quadriceps when knee affected)
is found.is found.
Flexion contractures in the knees and, lessFlexion contractures in the knees and, less
commonly, the wrists are found.commonly, the wrists are found.
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Picture 1. Patient with active pauciarticular disease.Picture 1. Patient with active pauciarticular disease. ((significantsignificant
suprapatellar swelling (effusion)suprapatellar swelling (effusion),, loss of natural contour medial toloss of natural contour medial to
the patellathe patella))..
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P ol y ar tic ular for mP ol y ar tic ular for m affects at least 5 jointsaffects at least 5 joints
Both large and small joints can be involved,Both large and small joints can be involved,often in symmetric bilateral distribution.often in symmetric bilateral distribution.
Severe limitations in motion are usuallySevere limitations in motion are usually
accompanied by weakness and decreasedaccompanied by weakness and decreased
physical function.physical function.
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Picture 2. Patient with active polyarticular arthritis.Picture 2. Patient with active polyarticular arthritis. ((swelling of allswelling of all
proximal interphalangeal jointsproximal interphalangeal joints,, boney overgrowthboney overgrowth,, lack of distallack of distal
interphalangeal joint involvement. The patient has interosseusinterphalangeal joint involvement. The patient has interosseus
muscle wasting and subluxation and ulnar deviation of the wristsmuscle wasting and subluxation and ulnar deviation of the wristsare presentare present))..
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Laboratory studiesLaboratory studies should include the following:should include the following:
oo Erythrocyte sedimentation rate (ESR)Erythrocyte sedimentation rate (ESR)
oo CBC with differential and platelet countCBC with differential and platelet count
oo Alanine aminotransferase (ALT) test Alanine aminotransferase (ALT) test
oo Urinalysis with microscopic examinationUrinalysis with microscopic examination
oo
Antinuclear antibody Antinuclear antibodyoo Rheumatoid factor Rheumatoid factor
oo Total protein, albumin, fibrinogen, DTotal protein, albumin, fibrinogen, D--dimer dimer (for (for systemic JRA)systemic JRA)
oo
Imaging Studies:Imaging Studies: radiography of affectedradiography of affected joints, bone scanning, MRI, CT scanning of joints, bone scanning, MRI, CT scanning of long bones,long bones, eechocardiographychocardiography
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Picture 3. Wrist radiographs of the patient with active polyarticular arthritis shownPicture 3. Wrist radiographs of the patient with active polyarticular arthritis shown
in Image 2.in Image 2. ((severe loss of cartilage in the intercarpal spaces and the radiocarpalsevere loss of cartilage in the intercarpal spaces and the radiocarpal
space of the right wristspace of the right wrist,, large erosion is present in the articular surface of thelarge erosion is present in the articular surface of the
ulnar epiphysis. The view of the left wrist shows boney ankylosis involving theulnar epiphysis. The view of the left wrist shows boney ankylosis involving the
lateral 4 carpal bones with sparing of the pisiform. Erosions are present in thelateral 4 carpal bones with sparing of the pisiform. Erosions are present in the
distal radius and ulna.distal radius and ulna.
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PROGNOSIS OF JRAPROGNOSIS OF JRA
Pauciarticular JRAPauciarticular JRA
BoysBoys may be affected in older childhood or may be affected in older childhood or
adolescence; this may represent an earlyadolescence; this may represent an early
manifestation of a spondyloarthropathy.manifestation of a spondyloarthropathy.
Leg length discrepancyLeg length discrepancy from asymmetric kneefrom asymmetric knee
synovitis and bone growth may cause flexionsynovitis and bone growth may cause flexioncontractures, gait abnormalities and longcontractures, gait abnormalities and long--term growthterm growth
abnormalities.abnormalities.
Eye involvementEye involvement as anterior uveitis, may lead toas anterior uveitis, may lead to
scarring or blindness in ~ 15scarring or blindness in ~ 15--20% of children.20% of children. Active arthritis into adulthood Active arthritis into adulthood in 40% to 50% of in 40% to 50% of
patients.patients.
Radiographic joint damageRadiographic joint damage within 5 years.within 5 years.
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PROGNOSIS OF JRAPROGNOSIS OF JRA
Polyarticular JRA and Systemic JRAPolyarticular JRA and Systemic JRA Active arthritis into adulthood Active arthritis into adulthood: 50% to 70% of polyarticular : 50% to 70% of polyarticular
or systemic onset JRA;or systemic onset JRA;
LongLong--term disabilitiesterm disabilities: 30% to 40% of children: 30% to 40% of children Unemployment: 25% to 50% of adult JRA patients;Unemployment: 25% to 50% of adult JRA patients;
May need major surgeryMay need major surgery (joint replacement).(joint replacement).
Radiographic joint damageRadiographic joint damage within 2 years;within 2 years;
Mortality rateMortality rate: 0.4% to 2% (greater risk with systemic JRA: 0.4% to 2% (greater risk with systemic JRAthan with polyarticular JRA).than with polyarticular JRA).
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Traditional Approach to theTraditional Approach to the
Treatment of JRATreatment of JRA
Cytotoxic Drugs
Disease Modifying
Anti-Rheumatic Drugs(DMARDs)
Intra-Articular/Oral Corticosteroids
Non-Steroidal Anti-Inflammatory Drugs (NSAIDs)
Before the 1990s «Pyramid Approach
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Treatments with Indications for JRATreatments with Indications for JRA
NonNon--Selective NSAIDsSelective NSAIDs
Aspirin, tolmetin sodium, ibuprofen, naproxen Aspirin, tolmetin sodium, ibuprofen, naproxen
NaproxenNaproxen [Tablets and Suspension][Tablets and Suspension]
Indicated for patients 2 years and older with juvenileIndicated for patients 2 years and older with juvenile
arthritis.arthritis.
Daily dose: approximately 10 mg/kg/day as a BIDDaily dose: approximately 10 mg/kg/day as a BIDdose (5 mg/kg given twicedose (5 mg/kg given twice--aa--day). Total daily dose isday). Total daily dose is
not to exceed 15 mg/kg/day.not to exceed 15 mg/kg/day.
Adverse events: gastrointestinal, central nervous Adverse events: gastrointestinal, central nervous
system (headache, dizziness, drowsiness, vertigo),system (headache, dizziness, drowsiness, vertigo),rash (ecchymoses, purpura), pruritus, sweating,rash (ecchymoses, purpura), pruritus, sweating,
special senses (tinnitus, visual disturbances, hearingspecial senses (tinnitus, visual disturbances, hearing
disturbances), cardiovascular (edema, palpitations)disturbances), cardiovascular (edema, palpitations)
prolonged bleeding times.prolonged bleeding times.
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Treatments with Indications for JRATreatments with Indications for JRA
NonNon--Selective NSAIDs/COXSelective NSAIDs/COX--2 Selective Inhibitors2 Selective Inhibitors
MOBIC (meloxicam)MOBIC (meloxicam) [Tablets and Suspension][Tablets and Suspension]
Indicated for the relief of the signs and symptoms of Indicated for the relief of the signs and symptoms of
pauciarticular and polyarticular course JRA in patients 2 yrspauciarticular and polyarticular course JRA in patients 2 yrs
and older.and older.
0.125 mg/kg once daily up to a maximum of 7.5 mg.0.125 mg/kg once daily up to a maximum of 7.5 mg. Adverse events: abdominal pain/upper, vomiting, diarrhea, Adverse events: abdominal pain/upper, vomiting, diarrhea,
headache, infection (rhinitis), cough, pyrexia, rash. urticaria,headache, infection (rhinitis), cough, pyrexia, rash. urticaria,
slight increases in systolic blood pressure.slight increases in systolic blood pressure.
VIOXX (rofecoxib)VIOXX (rofecoxib) [Tablets and Suspension][Tablets and Suspension]
Withdrawn from the global market September 2004.Withdrawn from the global market September 2004. Indicated for the relief of the signs and symptoms of juvenileIndicated for the relief of the signs and symptoms of juvenile
rheumatoid arthritis in patients 2 years and older.rheumatoid arthritis in patients 2 years and older.
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Treatment of JRATreatment of JRA
CorticosteroidsCorticosteroids
Used for uncontrolled or lifeUsed for uncontrolled or life--threatening systemicthreatening systemicdisease;disease;
Treatment of chronic uveitis as local ophthalmicTreatment of chronic uveitis as local ophthalmicdrops; or drops; or
IntraIntra--articular agents (Pauciarticular agents (Pauci-- and polyarticular JRA)and polyarticular JRA)
IntermediateIntermediate--acting corticosteroids: Prednisone;acting corticosteroids: Prednisone;methylmethyl--prednisolone (Intravenous pulse therapy for prednisolone (Intravenous pulse therapy for severely active JRA).severely active JRA).
Prednisone lowPrednisone low--dose as 0.1 to 0.2 mg/kg; higher dose as 0.1 to 0.2 mg/kg; higher--dose 0.25 to 1.0 mg/kg/day (maximum single dosedose 0.25 to 1.0 mg/kg/day (maximum single dose
40 mg)40 mg) Adverse events: hypertension, iatrogenic Adverse events: hypertension, iatrogenic
Cushing¶s syndrome, growth suppression,Cushing¶s syndrome, growth suppression,fractures, cataracts, increased susceptibility tofractures, cataracts, increased susceptibility toinfection.infection.
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Treatment of JRATreatment of JRA DMARDs and Biologic DMARDsDMARDs and Biologic DMARDs
Methotrexate (MTX):Methotrexate (MTX): used when NSAIDs fail to bringused when NSAIDs fail to bringrelief.relief.
Indicated for polyarticular JRA. MTX is the mostIndicated for polyarticular JRA. MTX is the mostwidelywidely used DMARD for JRA treatment.used DMARD for JRA treatment.
Starting dose 7.5 mg/mStarting dose 7.5 mg/m22 per week; maximum dose of per week; maximum dose of
15 mg/m15 mg/m22
per week.per week. Methotrexate compared to leflunomide (Lef): 240 JRAMethotrexate compared to leflunomide (Lef): 240 JRA
pts, 16pts, 16--week DB + 6 mo Ext + optional 30 mo Ext inweek DB + 6 mo Ext + optional 30 mo Ext inJRA; JRA Definition of ImprovementJRA; JRA Definition of Improvement >> 30% (JRA DOI30% (JRA DOI>> 30): 89% MTX compared to 68% Lef.30): 89% MTX compared to 68% Lef.
Adverse events: stomatitis, leukopenia, nausea/ Adverse events: stomatitis, leukopenia, nausea/abdominal pain, gastrointestinal bleeding, anorexia,abdominal pain, gastrointestinal bleeding, anorexia,malaise, fatigue, chills and fever, headache, alopecia,malaise, fatigue, chills and fever, headache, alopecia,rash, decreased resistance to infection, elevatedrash, decreased resistance to infection, elevatedhepatic enzymes.hepatic enzymes.
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Treatment of JRATreatment of JRA
DMARDs and Biologic DMARDsDMARDs and Biologic DMARDs (Continued)(Continued)
SulfasalazineSulfasalazine
Indicated for polyarticular JRA who have respondedIndicated for polyarticular JRA who have responded
inadequately to salicylates or other noninadequately to salicylates or other non--steroidalsteroidal
antianti--inflammatory drugs.inflammatory drugs.
Children 6 yrs and older: 40Children 6 yrs and older: 40 -- 60 mg/kg/day divided60 mg/kg/day divided
into 3 to 6 doses.into 3 to 6 doses.
Maintenance dose: 30 mg/kg/day divided into 4Maintenance dose: 30 mg/kg/day divided into 4
doses.doses.
Adverse events: anorexia, headache, vomiting, Adverse events: anorexia, headache, vomiting,gastric distress, rash, urticaria, hemolytic anemia.gastric distress, rash, urticaria, hemolytic anemia.
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Treatment in JRATreatment in JRA
DMARDs and Biologic DMARDsDMARDs and Biologic DMARDs (Continued)(Continued)
ENBREL (etanercept):ENBREL (etanercept): a cytokine antagonista cytokine antagonist Indicated for moderate to severe polyarticular course JRAIndicated for moderate to severe polyarticular course JRA
patients 4 to 17 years of age who had an inadequate responsepatients 4 to 17 years of age who had an inadequate response
to one or more DMARDs.to one or more DMARDs.
Dosage: 0.4 mg/kg/week (maximum 25 mg/ dose given twiceDosage: 0.4 mg/kg/week (maximum 25 mg/ dose given twice
weekly) as subcutaneous injection preweekly) as subcutaneous injection pre--filled syringe, 72filled syringe, 72--96 hrs.96 hrs.apart.apart.
Adverse events: headache, nausea, abdominal pain, and Adverse events: headache, nausea, abdominal pain, and
vomiting. Infection was reported in 43 of 69 (62%) of JRAvomiting. Infection was reported in 43 of 69 (62%) of JRA
patients during the 3patients during the 3--month (openmonth (open--label phase). Serious AEslabel phase). Serious AEs
reported in the study: varicella, gastroenteritis, depression/reported in the study: varicella, gastroenteritis, depression/personality disorder, cutaneous ulcer, esophagitis/ gastritis,personality disorder, cutaneous ulcer, esophagitis/ gastritis,
group A streptococcal septic shock, Type 1 diabetes, soft tissuegroup A streptococcal septic shock, Type 1 diabetes, soft tissue
and postand post--operative wound infectionoperative wound infection..
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Treatment in JRATreatment in JRA
DMARDs indicated for RA without an indication for JRADMARDs indicated for RA without an indication for JRA Hydroxychloroquine, injectable gold, leflunomide andHydroxychloroquine, injectable gold, leflunomide and
dd--penicillamine.penicillamine.
Other Immunomodulatory or Cytotoxic DrugsOther Immunomodulatory or Cytotoxic Drugs
Indicated in RA without a JRA indication:Indicated in RA without a JRA indication:
Azathioprine Azathioprine
Cyclosporine ACyclosporine A
Without a RA or a JRA indication:Without a RA or a JRA indication:
ChlorambucilChlorambucil
ThalidomideThalidomide
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Treatment of JRA in 2008Treatment of JRA in 2008
Pauciarticular Pauciarticular
25% to 33% will respond to NSAIDs;25% to 33% will respond to NSAIDs;
Patients not responsive to NSAIDS after 4Patients not responsive to NSAIDS after 4 -- 6 weeks6 weeks
with flexion contractures or leg length discrepancywith flexion contractures or leg length discrepancy pp
intraintra--articular corticosteroids.articular corticosteroids. Patients with extended pauciarticular JRA or smallPatients with extended pauciarticular JRA or small
joint involvement joint involvement pp treat as polyarticular JRA.treat as polyarticular JRA.
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Treatment of JRA in 2008Treatment of JRA in 2008
Polyarticular Polyarticular RF (RF (--) or (+), NSAID (symptom control) alone is usually) or (+), NSAID (symptom control) alone is usually
not as effective as a NSAID + DMARD.not as effective as a NSAID + DMARD.
NSAID trial for several weeksNSAID trial for several weeks pp add oral MTX.add oral MTX.
If oral MTX is not effectiveIf oral MTX is not effective pp parenteral route MTX.parenteral route MTX. If NSAID + MTX (oral or parenteral) is not effectiveIf NSAID + MTX (oral or parenteral) is not effectivepp
antianti--TNF medication.TNF medication.
No current evidence whether a combination of MTX +No current evidence whether a combination of MTX +
antianti--TNF medication are more effective than only antiTNF medication are more effective than only anti--TNF medicationTNF medication..
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Treatment of JRA in 2008Treatment of JRA in 2008
SystemicSystemic NSAIDs 2 to 3 weeks with cautionNSAIDs 2 to 3 weeks with cautionpp risk of risk of
Disseminated Intravascular Coagulation (DIC),Disseminated Intravascular Coagulation (DIC),
(macrophage activation syndrome);(macrophage activation syndrome);
Intravenous pulse methylprednisolone;Intravenous pulse methylprednisolone; Oral corticosteroidsOral corticosteroids pp
Lowest effective dose;Lowest effective dose;
Steroid sparingSteroid sparing pp immunomodulatory approach isimmunomodulatory approach is
under evaluation for steroid sparing effects.under evaluation for steroid sparing effects.
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ComplicationsComplications:: SystemicSystemic--onset JRAonset JRA
PericarditisPericarditis
Hemolytic anemiaHemolytic anemia Disseminated intravascular coagulopathy.Disseminated intravascular coagulopathy.
Macrophage activation syndromeMacrophage activation syndrome
Endarteritis resulting in circulatory compromise of the digitsEndarteritis resulting in circulatory compromise of the digitswith threatened autoamputationwith threatened autoamputation
Pauciarticular JRAPauciarticular JRA Knee flexion contractures:Knee flexion contractures:
Uveitis (Uveitis (Picture 7Picture 7).).
Leg length discrepancy (can result from neovascularizationLeg length discrepancy (can result from neovascularizationof growth plates of an affected knee)of growth plates of an affected knee)
Polyarticular JRAPolyarticular JRA Skeletal abnormalitiesSkeletal abnormalities -- ((Picture 5Picture 5--66).).
Cervical spine involvementCervical spine involvement
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Picture 5. Patient with inactive polyarticular arthritis. LongPicture 5. Patient with inactive polyarticular arthritis. Long--term sequelae of term sequelae of
polyarticular disease includes joint subluxation (note both wrists and thumbs),polyarticular disease includes joint subluxation (note both wrists and thumbs),
joint contractures (at proximal interphalangeal joints and distal interphalangeal joint contractures (at proximal interphalangeal joints and distal interphalangeal
joints), boney overgrowth), and finger deformities (swan joints), boney overgrowth), and finger deformities (swan--neck or boutonniereneck or boutonniere
deformities).deformities).
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Picture 6. Hand and wrist radiographs of the patient with inactive polyarticular Picture 6. Hand and wrist radiographs of the patient with inactive polyarticular
arthritis shown in Image 5. Longarthritis shown in Image 5. Long--term sequelae of polyarticular disease includesterm sequelae of polyarticular disease includes
periarticular osteopenia, generalized increase in the size of epiphyses,periarticular osteopenia, generalized increase in the size of epiphyses,
accelerated bone age, narrowed joint space, boutonniere deformities (at left thirdaccelerated bone age, narrowed joint space, boutonniere deformities (at left thirdand fourth interphalangeal joints), and medial subluxation of the firstand fourth interphalangeal joints), and medial subluxation of the first
metacarpophalangeal joints bilaterallymetacarpophalangeal joints bilaterally..
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Picture 7. Sequelae of chronic anterior uveitis. Note the posterior synechiaePicture 7. Sequelae of chronic anterior uveitis. Note the posterior synechiae
(weblike attachments of the pupillary margin to the anterior lens capsule) of the(weblike attachments of the pupillary margin to the anterior lens capsule) of the
right eye secondary to chronic anterior uveitis. This patient has a positiveright eye secondary to chronic anterior uveitis. This patient has a positive
antinuclear antibodies and initially had a pauciarticular course of her arthritis.antinuclear antibodies and initially had a pauciarticular course of her arthritis.
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