JIA and JIA and Other Other

Rheumatic Rheumatic Diseases in Diseases in

ChildrenChildren

Norma Liburd, Norma Liburd,

RN-BC, MNRN-BC, MN

ObjectivesObjectivesDefine Juvenile Idiopathic Define Juvenile Idiopathic Arthritis (JIA) and discuss Arthritis (JIA) and discuss the diagnostic criteria.the diagnostic criteria.

Identify the subtypes of JIA Identify the subtypes of JIA and discuss characteristics and discuss characteristics of each.of each.

Name at least one NSAID, Name at least one NSAID, one biologic and one one biologic and one DMARD used in the DMARD used in the treatment of JIA.treatment of JIA.

A few more ObjectivesA few more ObjectivesDiscuss three school related problems Discuss three school related problems students with JIA have and intervention students with JIA have and intervention strategies for each.strategies for each.

Identify the criteria for classification of Identify the criteria for classification of systemic lupus erythematosus. systemic lupus erythematosus.

Name the most common type of juvenile Name the most common type of juvenile localized scleroderma.localized scleroderma.

Discuss the criteria for diagnosis of juvenile Discuss the criteria for diagnosis of juvenile dermatomyositis, and treatment approachesdermatomyositis, and treatment approaches

Overview of JIAOverview of JIA

New classification criteria proposed by the New classification criteria proposed by the Pediatric Task Force of the International League Pediatric Task Force of the International League of Associations for Rheumatology (ILAR) in of Associations for Rheumatology (ILAR) in 19971997

Chronic arthritis in childhood – one of the more Chronic arthritis in childhood – one of the more frequent chronic illnesses of childhood.frequent chronic illnesses of childhood.

An important cause of short and long-term An important cause of short and long-term disability disability

Chronic arthritis Chronic arthritis in childhood: JIAin childhood: JIA

It’s not a single disease, but a group of It’s not a single disease, but a group of related, genetically heterogeneous, related, genetically heterogeneous, phenotypically diverse phenotypically diverse immunoinflammatory disorders affecting immunoinflammatory disorders affecting joints and other structures, possibly joints and other structures, possibly activated by contact with an external activated by contact with an external antigen or antigens.antigen or antigens.

JRA - Incidence/PrevalenceJRA - Incidence/Prevalence

Published series are difficult Published series are difficult to interpret due to to interpret due to classification, methodologies, classification, methodologies, heterogeneityheterogeneityIncidence: (per year)Incidence: (per year)

1/100,000 in Japan 1/100,000 in Japan 20/100,000 in Norway20/100,000 in Norway

Prevalence:Prevalence:– 10 /100,000 in France 10 /100,000 in France – 400/100,000 in Australia400/100,000 in Australia– 113/ 100,000113/ 100,000

Arthritis Foundation: Arthritis Foundation: 300,000 children in the US 300,000 children in the US have chronic arthritis.have chronic arthritis.

..

JRA – Classification CriteriaJRA – Classification CriteriaJRAJRA – American College of Rheumatology 1970 – American College of Rheumatology 1970 three types of onset: oligo (pauciarticular), three types of onset: oligo (pauciarticular), polyarticular, & systemic in the first 6 months of polyarticular, & systemic in the first 6 months of onsetonset

JCAJCA Juvenile Chronic ArthritisJuvenile Chronic Arthritis (European League (European League Against Rheumatism) 1977Against Rheumatism) 1977

JIAJIA Juvenile Idiopathic ArthritisJuvenile Idiopathic Arthritis proposed by the proposed by the Pediatric Task force of the International League of Pediatric Task force of the International League of Associations for Rheumatology ILAR (1993) – Associations for Rheumatology ILAR (1993) – developed to achieve homogeneity within disease developed to achieve homogeneity within disease and categories. and categories.

Sex Ratio Sex Ratio All types of JIA:All types of JIA:– Girls: Boys 2:1Girls: Boys 2:1Oligo JIA: Oligo JIA: – Girls: Boys 3:1Girls: Boys 3:1

JIA with uveitisJIA with uveitis– Girls: BoysGirls: Boys 5-6:1 5-6:1

Poly JRA:Poly JRA:– Girls: Boys 3:1Girls: Boys 3:1

Systemic JRA:Systemic JRA:– Girls: Boys approx. 1:1Girls: Boys approx. 1:1

JIA outcomes: MortalityJIA outcomes: Mortality

Disease associated death rate isDisease associated death rate is< 1% in Europe< 1% in Europe< 0.3% in North America< 0.3% in North America

These numbers represent a These numbers represent a

4 Fold to 14 fold Increase in Mortality Rate4 Fold to 14 fold Increase in Mortality RateCompared with General Population Compared with General Population

Causes are cardiac, infection & macrophage activation syndromeCauses are cardiac, infection & macrophage activation syndrome

JRA outcome: functional abilitiesJRA outcome: functional abilities

AuthorAuthor Year PublishedYear Published Followup in Followup in years (mean)years (mean)

Poor FunctionPoor Function

BunimBunim 19591959 1010 31%31%

LaaksonenLaaksonen 19661966 >16>16 48%48%

AnsellAnsell 19761976 >15>15 23%23%

HillHill 19761976 (14.5)(14.5) 33%33%

HansonHanson 19771977 5-25 (10)5-25 (10) 28%28%

StoeberStoeber 19811981 10-22 (15)10-22 (15) 41%41%

LevinsonLevinson 19911991 15-2015-20 17%17%

ZakZak 20002000 2828 11%11%

Classification Criteria for JIAClassification Criteria for JIA

Age at onset <16 yearsAge at onset <16 years

Duration of Arthritis: 6 weeksDuration of Arthritis: 6 weeks

Arthritis in one or more joints defined as swelling Arthritis in one or more joints defined as swelling or effusion, or presence of two or more of the or effusion, or presence of two or more of the following signs: (in 1 or more joints)following signs: (in 1 or more joints)– Limitation of ROMLimitation of ROM

– Tenderness or pain on motionTenderness or pain on motion

– Increased heatIncreased heat

Exclusion of other diseasesExclusion of other diseases

Diagnostic StudiesDiagnostic Studies

Diagnostic TestsDiagnostic Tests

There is no lab test that diagnoses JIAThere is no lab test that diagnoses JIA

The H&P should determine the labs, not The H&P should determine the labs, not the reversethe reverse– CBC CBC – Rheumatoid factor Rheumatoid factor – Antinuclear Antibody (ANA) – with titerAntinuclear Antibody (ANA) – with titer– ESR or CRPESR or CRP– Anti-CCP (Anti-CCP (anti-cyclic citrullinated protein)anti-cyclic citrullinated protein)

Radiologic StudiesRadiologic StudiesX-rays X-rays

Soft tissue swellingSoft tissue swellingOsteoporosisOsteoporosis

Periosteal new bone Periosteal new bone formationformation

Epiphyseal overgrowthEpiphyseal overgrowthMarginal erosionsMarginal erosions

Narrowing of Narrowing of cartilaginouscartilaginous

spacespaceJoint subluxationJoint subluxation

Bony fusionBony fusion

DexascansDexascansOsteopeniaOsteopenia

OsteoporosisOsteoporosis

EtiologyEtiologyImmune mediated diseaseImmune mediated disease– Abnormal immunoregulationAbnormal immunoregulation– Abnormal cytokine production in the Abnormal cytokine production in the

inflammatory pathway (TNF, IL-6, IL-2R, inflammatory pathway (TNF, IL-6, IL-2R, IL-1alpha)IL-1alpha)

Complex genetic predispositions Complex genetic predispositions – HLA associationsHLA associations

Environmental triggers Environmental triggers – InfectionsInfections– TraumaTrauma– StressStress

Synovial lining is a thin membrane enclosing the joint space. The joint space contains fluid that bathes the joint and reduces friction on motion.

With onset of inflammation, the synovial lining thickens and secretes more fluid, which may remain in the joint and cause swelling. The inflamed lining produces warmth, swelling, and pain.

As inflammation progresses, the synovial lining grows over the cartilage and starts to erode it. As inflammation continues, changes include marked erosion of cartilage, cystic changes and thinning of the bone.

Classification CriteriaClassification Criteria1.1. Systemic Systemic2.2. Oligoarthritis Oligoarthritis

a.a. Persistent Persistentb.b. Extended Extended

3.3. Polyarthritis (rheumatoid factor negative) Polyarthritis (rheumatoid factor negative)

4.4. Polyarthritis (rheumatoid factor positive) Polyarthritis (rheumatoid factor positive)

5.5. Psoriatic arthritis Psoriatic arthritis

6.6. Enthesitis-related arthritis Enthesitis-related arthritis

7.7. Undifferentiated arthritis Undifferentiated arthritis a.a. Fits no other category Fits no other categoryb.b. Fits more than one category Fits more than one category

From Petty RE, Southwood TR, Baum J et al: Revision of the proposed classification criteria for From Petty RE, Southwood TR, Baum J et al: Revision of the proposed classification criteria for juvenile idiopathic arthritis: Durban, 1997, J Rheumatol 25:199-1994, 1998.juvenile idiopathic arthritis: Durban, 1997, J Rheumatol 25:199-1994, 1998.

JIA SubtypesJIA Subtypes

Systemic Onset (5-15%)Systemic Onset (5-15%)

Polyarticular Onset (20%)Polyarticular Onset (20%)– Rheumatoid Factor PositiveRheumatoid Factor Positive– Rheumatoid Factor Negative (85%)Rheumatoid Factor Negative (85%)

Oligoarthritis (50-80%)Oligoarthritis (50-80%)

Juvenile psoriatic arthritis (7%)Juvenile psoriatic arthritis (7%)

Enthesitis related arthritis Enthesitis related arthritis

UndifferentiatedUndifferentiated

Systemic JIASystemic JIADefinition:Definition:

– Arthritis with, or preceded by, daily fever Arthritis with, or preceded by, daily fever of at least 2 weeks’ durationof at least 2 weeks’ duration

– Fevers are quotidian (daily) for at least 3 Fevers are quotidian (daily) for at least 3 days and is accompanied by one or more days and is accompanied by one or more of the following:of the following:

Evanescent, non-fixed, erythematous rashEvanescent, non-fixed, erythematous rash

Generalized lymph node enlargementGeneralized lymph node enlargement

Hepatomegaly and/or splenomegalyHepatomegaly and/or splenomegaly

SerositisSerositis

Quotidian fever Quotidian fever Intermittent fever of systemic JIA in a 3-year-old girl. The fever spikes usually occurred daily in the late evening to early morning (quotidian pattern), returned to normal or below normal, and were accompanied by severe malaise, tachycardia, and rash.

Systemic JRASystemic JRA

Rash - Rash - – Salmon coloredSalmon colored– Maculopapular – Maculopapular –

flat to slightly raisedflat to slightly raised– Trunk and Trunk and

extremitiesextremities– MigratoryMigratory– Pruritic 5%Pruritic 5%– FleetingFleeting– Persistent with Persistent with

fever spikefever spike

Overview of Systemic JIAOverview of Systemic JIA10-15% of all JRA patients10-15% of all JRA patientsBroad peak of onset 1-5 yearsBroad peak of onset 1-5 yearsM:F 1:1M:F 1:1Variable number of jointsVariable number of jointsIl-6 is elevated and correlates with disease activity Il-6 is elevated and correlates with disease activity

Extraarticular symptoms: Extraarticular symptoms: – Fever 100 %Fever 100 %– Rash 95%Rash 95%– Hepatosplenomegaly, 85%Hepatosplenomegaly, 85%– Lymphadenopathy 70%Lymphadenopathy 70%– Pericarditis 35%Pericarditis 35%– Pleuritis 20%Pleuritis 20%

Macrophage Activation SyndromeMacrophage Activation Syndrome

Rare devastating complication of systemic JIA. Rare devastating complication of systemic JIA. Etiology is uncertain.Etiology is uncertain.

Demonstration of macrophages ingesting other Demonstration of macrophages ingesting other hematopoietic cells in marrow is diagnostichematopoietic cells in marrow is diagnostic

Early recognition is life-saving Early recognition is life-saving – Looks somewhat like a flare up of systemic JRA but is different enough to allow for Looks somewhat like a flare up of systemic JRA but is different enough to allow for

early recognition)early recognition)

Associated with CMV, EBV, changes in meds Associated with CMV, EBV, changes in meds

Mortality 10-20%Mortality 10-20%

Macrophage Activation SyndromeMacrophage Activation Syndrome

Acute onset of fever withAcute onset of fever with– Bruising, purpura, mucosal bleedingBruising, purpura, mucosal bleeding– Enlarged lymph nodes, liver, spleenEnlarged lymph nodes, liver, spleen– Elevated AST, ALT, PT, PTT, fibrin D-dimerElevated AST, ALT, PT, PTT, fibrin D-dimer– Elevated ferritin & triglyceridesElevated ferritin & triglycerides– Abrupt fall in WBC & plateletsAbrupt fall in WBC & platelets– Fall in ESRFall in ESR– Fall in fibrinogen, clotting factorsFall in fibrinogen, clotting factors

Often progresses to fatal DIC, hepatic Often progresses to fatal DIC, hepatic failure, encephalopathyfailure, encephalopathyTreatment: IV steroids, cyclosporinTreatment: IV steroids, cyclosporin

Polyarticular JIA - Polyarticular JIA - RF negativeRF negativeFive or more joints in the Five or more joints in the first 6 months of diseasefirst 6 months of diseaseAsymmetric joint Asymmetric joint involvementinvolvementLarge joints of knees, Large joints of knees, wrists, elbows and wrists, elbows and ankles often affectedankles often affectedMorning stiffness, joint Morning stiffness, joint painpainIntermittent low-grade Intermittent low-grade feverfever

Polyarticular Polyarticular - RF positive- RF positiveArthritis affecting 5 or more joints in the Arthritis affecting 5 or more joints in the first 6 months of disease. first 6 months of disease.

Similar to adult RASimilar to adult RA

Females with onset in adolescenceFemales with onset in adolescence

Rheumatoid nodulesRheumatoid nodules

Early onset of erosive synovitisEarly onset of erosive synovitis

Symmetric joint involvementSymmetric joint involvement

Small joints of hands or feet are affectedSmall joints of hands or feet are affected

TMJ: micronathiaTMJ: micronathia

Cervical spine may be affectedCervical spine may be affected

Rheumatoid NodulesRheumatoid NodulesOccur in 5-10% of children Occur in 5-10% of children with JIAwith JIAMost frequently on elbowMost frequently on elbowPressure points, digital flexor Pressure points, digital flexor tendon sheaths, Achilles tendon sheaths, Achilles tendons, bridge of nose in tendons, bridge of nose in child who wears glasseschild who wears glassesFirm or hard, usually mobile, Firm or hard, usually mobile, nontender. nontender. Solitary or multiple, may Solitary or multiple, may change in size, may last change in size, may last months to years.months to years.

Oligoarticular JIAOligoarticular JIAArthritis in 1 to 4 joints Arthritis in 1 to 4 joints during the first 6 during the first 6 months of diseasemonths of disease

Girls 1 to 4 yearsGirls 1 to 4 years

Knees, ankles, elbowsKnees, ankles, elbows

Painless swelling of Painless swelling of joints is commonjoints is common

Uveitis: insidious, Uveitis: insidious, subacutesubacute

15-20% have uveitis15-20% have uveitis

JIA: Oligo – persistentJIA: Oligo – persistentNo more than 4 joints affected throughout theNo more than 4 joints affected throughout thedisease coursedisease course

JIA: Oligo - extendedJIA: Oligo - extendedAffects a total of more than 4 joints after the first 6 Affects a total of more than 4 joints after the first 6 months of disease. months of disease. At least 1/3 of children with Oligoarticular arthritis At least 1/3 of children with Oligoarticular arthritis fall into this categoryfall into this categoryOutcome is more typical of RF+ polyarticular Outcome is more typical of RF+ polyarticular diseasedisease

Uveitis in JIAUveitis in JIAIntraocular Intraocular inflammation affects inflammation affects iris and ciliary bodyiris and ciliary bodyUsually insidious and Usually insidious and may be asymptomaticmay be asymptomaticActivity of eye does Activity of eye does not parallel joint not parallel joint diseasediseaseSlit lamp exam Slit lamp exam detects anterior detects anterior chamber inflammationchamber inflammationGirls, ANA + and Girls, ANA + and onset before age 7 at onset before age 7 at higher riskhigher risk

Prognosis of Uveitis in JIAPrognosis of Uveitis in JIAVery good in 25% of casesVery good in 25% of cases

25% may require surgery for cataracts and/or 25% may require surgery for cataracts and/or glaucomaglaucoma

50% require prolonged treatment for moderate to 50% require prolonged treatment for moderate to severe chronic inflammation; however, the severe chronic inflammation; however, the prognosis is generally goodprognosis is generally good

Complications: cataracts Complications: cataracts 20%, glaucoma 20%, 20%, glaucoma 20%, band keratopathy 16% band keratopathy 16% (end stage scarring)(end stage scarring)

Uveitis in JIAUveitis in JIAUsually occurs after onset of arthritis. Highest Usually occurs after onset of arthritis. Highest risk is within 2 years of onset of arthritis. Majority risk is within 2 years of onset of arthritis. Majority develop eye disease within 5-7 years after onsetdevelop eye disease within 5-7 years after onset

65% have bilateral involvement, unilateral may 65% have bilateral involvement, unilateral may progress to bilateralprogress to bilateral

Treatment includes topical steroids, SQ Treatment includes topical steroids, SQ Methotrexate, IV Remicade; SQ Humira and Methotrexate, IV Remicade; SQ Humira and Enbrel.Enbrel.

Slit Lamp Exam – JIA Slit Lamp Exam – JIA GuidelinesGuidelines

Rheumatology & Ophthalmology sections of the Rheumatology & Ophthalmology sections of the American Academy of Pediatrics, 1993American Academy of Pediatrics, 1993

Oligoarticular ANA+Oligoarticular ANA+ <7 <7 years at Dxyears at Dx

Oligoarticular ANA+ 7 or older Oligoarticular ANA+ 7 or older at Dxat Dx

Oligoarticular ANA -Oligoarticular ANA - <7 years at <7 years at DxDx

Oligoarticular ANA –Oligoarticular ANA –

<7 years at Dx<7 years at Dx

SystemicSystemic

Q 3-4 months for 7 Q 3-4 months for 7 years, then yearly.years, then yearly.

Q 4-6 months for 7 yrs, Q 4-6 months for 7 yrs, then yearly.then yearly.

Q 4-6 months for 4 yrs, Q 4-6 months for 4 yrs, then yearly.then yearly.

Yearly.Yearly.

JIA Onset ANA Onset < 7 yrs Onset ≧ 7 years

Oligo Positive Every 3-4 months Every 4-6 months

Oligo Negative Every 4-6 months Every 4-6 months

Polyarthritis Positive Every 3-4 months Every 4-6 months

Polyarthritis Negative Every 4-6 months Every 4-6 months

Systemic Neg or pos Every 12 months Every 12 months

High risk – screen every 3 monthsModerate risk – screen every 4-6 monthsLow risk: screen every 12 monthsAll patients considered to be at low risk 7 yr after onset of arthritis; should have yearly

ophthalmological exams indefinitely.All patients are considered to be at low risk 4 years after onset of arthritis, should have yearly

ophthalmological exams indefinitely.All high risk patients are considered to be at medium risk 4 years after onset of arthritis.Modified from Yancy C, et.al, The Guidelines of the Rheumatology and ophthalmology sections of the

AAP. Pediatrics 92:295-296, 2003.

Guidelines for ophthalmological screening of children with JIA

JIA: Psoriatic ArthritisJIA: Psoriatic ArthritisArthritis and psoriasis orArthritis and psoriasis orArthritis with 2 of the following:Arthritis with 2 of the following:– Dactylitis - sausage like Dactylitis - sausage like

swelling of toe or fingerswelling of toe or finger– Nail pittingNail pitting– Psoriasis in a first degree Psoriasis in a first degree

relative (parents, siblings)relative (parents, siblings)Slightly more femalesSlightly more females Symmetrical involving large Symmetrical involving large and small jointsand small joints

JRA: SpondyloarthropathyJRA: SpondyloarthropathyJIA:JIA: Enthesitis related arthritisEnthesitis related arthritisArthritis and enthesitisArthritis and enthesitis

Arthritis or enthesitis with at least 2 of the following:Arthritis or enthesitis with at least 2 of the following:– Sacroiliac joint tenderness and/or inflammatory Sacroiliac joint tenderness and/or inflammatory

lumbosacral painlumbosacral pain– Presence of HLA-B27Presence of HLA-B27– Onset of arthritis in a male after age 6 yearsOnset of arthritis in a male after age 6 years– Ankylosing spondylitis, Enthesitis Related Ankylosing spondylitis, Enthesitis Related

Arthritis, Sacroiliitis with inflammatory bowel Arthritis, Sacroiliitis with inflammatory bowel disease, Reiter’s syndrome or acute anterior disease, Reiter’s syndrome or acute anterior uveitis in a first-degree relative.uveitis in a first-degree relative.

JRA: SpondyloarthropathyJRA: SpondyloarthropathyJIA:JIA: Enthesitis related arthritisEnthesitis related arthritis

Primarily affects boys 8 years and olderPrimarily affects boys 8 years and older

Affects large joints of lower extremitiesAffects large joints of lower extremities

Heel pain and Achilles tendonitis Heel pain and Achilles tendonitis

Sacroiliitis (90% of cases)Sacroiliitis (90% of cases)

Iritis (20% of cases) generally acute Iritis (20% of cases) generally acute processprocess

Low grade feversLow grade fevers

Decreased appetite Decreased appetite

MedicationsMedications

NSAIDs NSAIDs

DMARDs: DMARDs: Methotrexate, Plaquenil, Methotrexate, Plaquenil, Sulfasalazine Sulfasalazine

Biologic response Biologic response modifiersmodifiers

GlucocorticosteroidsGlucocorticosteroids

MiscellaneousMiscellaneous

NSAIDSNSAIDS

FDA approved for FDA approved for pediatric usepediatric use– AspirinAspirin– TolmetinTolmetin– NaproxenNaproxen– IbuprofenIbuprofen– IndomethacinIndomethacin– Meloxicam (Mobic)Meloxicam (Mobic)– CelebrexCelebrex

Common NSAIDS in JIACommon NSAIDS in JIA

mg/kg/daymg/kg/day MaxMaxNaproxen Naproxen 10-20 10-20 10001000IbuprofenIbuprofen 30-4030-40 24002400IndomethacinIndomethacin 1.5-3.01.5-3.0 200200TolmetinTolmetin 20-3020-30 18001800MeloxicanMeloxican 0.250.25 1515PiroxicamPiroxicam 0.2-0.30.2-0.3 2020CelecoxibCelecoxib 6-126-12 400400NabumetoneNabumetone 3030 20002000 (Relafen)(Relafen)ASAASA 80-10080-100 32003200

MethotrexateMethotrexate

Standard dose: 10-15 mg/m2 or 0.3-0.6 Standard dose: 10-15 mg/m2 or 0.3-0.6 mg/kg/week, mg/kg/week, subQsubQ

Improvement seen in 6-8 weeks, but may Improvement seen in 6-8 weeks, but may take up to 6 months. take up to 6 months.

Labs every 6 weeks: CBC, CMPLabs every 6 weeks: CBC, CMP

No alcoholNo alcohol

Used for treatment of uveitis (4-6 months Used for treatment of uveitis (4-6 months to determine efficacy)to determine efficacy)

Meds: Targeting inflammationMeds: Targeting inflammation

Meds: Biologic Agents: Meds: Biologic Agents: Target against cytokines involved in Target against cytokines involved in

inflammation: TNF , IL-1Ra, IL-6inflammation: TNF , IL-1Ra, IL-6

Enbrel Enbrel (Etanercept): (Etanercept): approved for JRAapproved for JRA– 0.4 mg/kg twice per 0.4 mg/kg twice per

week SQ injectionsweek SQ injections– Improvement by third Improvement by third

to fourth doseto fourth dose– Hold for suspected Hold for suspected

bacterial infection, bacterial infection, varicellavaricella

– Site reactionsSite reactions– Binds to TNF Binds to TNF

Biologic Agents: Biologic Agents:

Remicade (Infliximab) Remicade (Infliximab) - - infusion, risk of infusion, risk of anaphylaxis, dose may need to be increased anaphylaxis, dose may need to be increased depending on response, used in refractory depending on response, used in refractory uveitis as welluveitis as well

3 mg/kg IV weeks 0, 2 and 6 (may 3 mg/kg IV weeks 0, 2 and 6 (may dose to dose to 10 mg/kg)10 mg/kg)

Improvement can be seen after first doseImprovement can be seen after first dose

Labs every 4-8 weeks (CBC, CMP)Labs every 4-8 weeks (CBC, CMP)

Not approved for childrenNot approved for children

Biologic Agents: Biologic Agents:

Anakinra (Kineret) – Anakinra (Kineret) – (blocks IL-1 which (blocks IL-1 which stimulates synoviocytes and chondrocytes stimulates synoviocytes and chondrocytes to produce small inflammatory mediators – to produce small inflammatory mediators – leading to cartilage destruction and bone leading to cartilage destruction and bone erosions. erosions. – Used in systemic JRA (but not approved)Used in systemic JRA (but not approved)– Daily, Daily, very painfulvery painful, SQ injections, rotation of , SQ injections, rotation of

sites is importantsites is important

BiologicsBiologicsActemra (Tocilizumab) 8 mg/kgActemra (Tocilizumab) 8 mg/kg– ACTEMRA is indicated for the treatment of active ACTEMRA is indicated for the treatment of active

systemic juvenile idiopathic arthritis in patients 2 years systemic juvenile idiopathic arthritis in patients 2 years of age and older who have responded inadequately to of age and older who have responded inadequately to previously therapy with NSAIDS and steroids.previously therapy with NSAIDS and steroids.

--Given every 2 weeks by IV, over one hour. --Dosing interval can be shortened to every week if condition warrants.

BiologicsBiologics

Humira (adalimumab) Humira (adalimumab) TNF blocker: approved TNF blocker: approved for children ages 4 to 17for children ages 4 to 17

Dose: 15mg (33 lbs) to <30 kg (66 lbs): 20 mg Dose: 15mg (33 lbs) to <30 kg (66 lbs): 20 mg every other weekevery other week

Dose: 30kg or more: 40 mg every other weekDose: 30kg or more: 40 mg every other week

Humira pen – or prefilled syringeHumira pen – or prefilled syringe

Painful injections, but can add lidocaine to buffer Painful injections, but can add lidocaine to buffer the pain (Hershey study).the pain (Hershey study).

Can shorten interval to weekly (with auth)Can shorten interval to weekly (with auth)

BiologicsBiologicsOrencia (Abatacept) Orencia (Abatacept) T-lymphocyte modulatorT-lymphocyte modulator

IV over 30 minutes: at 0, 2 4 weeks, then every 4 weeksApproved for children 6 and older as monotherapy or with methotrexate<75 Kg: 10 mg/Kg If over 75 Kg: follow adult dosing

Approved for adults: weekly SQ self injection

GlucocorticosteroidsGlucocorticosteroids

IV Solumedrol and daily oral PrednisoneIV Solumedrol and daily oral Prednisone

systemic flares ~ pericarditis or persistent Sxsystemic flares ~ pericarditis or persistent Sx

temporary measure until DMARD is effectivetemporary measure until DMARD is effective

Joint injectionsJoint injections - usually under sedation - usually under sedation– Triamcinolone hexacetonide (Aristaspan)Triamcinolone hexacetonide (Aristaspan)

long acting steroidlong acting steroid

Works best with large jointsWorks best with large joints

Miscellaneous TreatmentMiscellaneous Treatment

Thalidomide: 2 mg/kg/dayThalidomide: 2 mg/kg/day– Mechanism of action probably by effects on TNF Mechanism of action probably by effects on TNF

and other inflammatory cytokinesand other inflammatory cytokines– Very rigorous patient monitoringVery rigorous patient monitoring

Bone Marrow TransplantBone Marrow Transplant– Experimental for severe autoimmune disease Experimental for severe autoimmune disease

unresponsive to conventional therapyunresponsive to conventional therapy– Autologous stem cell transplant being evaluated Autologous stem cell transplant being evaluated

in small number of childrenin small number of children– Infections ~ very risky – high death rateInfections ~ very risky – high death rate

PT/OT - Overall goalsPT/OT - Overall goals

Maintain or restore Maintain or restore functional ROM in jointsfunctional ROM in joints

Strengthen muscles Strengthen muscles surrounding affected joints surrounding affected joints - to enable joints to - to enable joints to remain in a functional remain in a functional positionposition

Assist child to perform Assist child to perform activities in ways as close activities in ways as close to normal as possible to normal as possible – so they do not feel so they do not feel

“different” from peers.“different” from peers.

PT/OT - Management in JIAPT/OT - Management in JIASplint fabricationSplint fabrication