Juvenile Rheumatoid Arthritis (JRA)

8/8/2019 Juvenile Rheumatoid Arthritis (JRA)

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Juvenile rheumatoidJuvenile rheumatoidarthritis (JRA)arthritis (JRA)



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.




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




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



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.




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.



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.



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.



CLINICAL MANIFESTATIONS of JRACLINICAL MANIFESTATIONS of JRA



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



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



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.



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.



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))..



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.



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))..



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



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.



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.



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).



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



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.



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.




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.



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.




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.



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..



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



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.




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..




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.



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



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).



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..



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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Juvenile Rheumatoid Arthritis (JRA)