A Case of Idiopathic Juvenile Arthritis

A Case Of FeverA Case Of Fever

DR.G.BALAJIDR.G.BALAJI

DR.G.SUNDARAMURTHY’S DR.G.SUNDARAMURTHY’S UNITUNIT

historyhistory

• 14 year boy presented with fever of 1 14 year boy presented with fever of 1 month duration. month duration.

• fever low grade, with no rigors, more in fever low grade, with no rigors, more in the evenings.the evenings.

• No h/o cough, expectorations, dyspnea, No h/o cough, expectorations, dyspnea, chest pain, palpitations.chest pain, palpitations.

• No h/o weight loss, night sweats.No h/o weight loss, night sweats.

• No h/o dysuria, hematuria, pain abdomenNo h/o dysuria, hematuria, pain abdomen

• No h/o loose stools, abdominal pain, constipation, No h/o loose stools, abdominal pain, constipation, weight loss.weight loss.

• h/o myalgia, joint pains.h/o myalgia, joint pains.• No h/o rashes, bleeding from gums, or any other No h/o rashes, bleeding from gums, or any other

mucosal bleeds.mucosal bleeds.• No h/o seizures, altered sensorium.No h/o seizures, altered sensorium.• No h/o any skin lesions.No h/o any skin lesions.Past history:Past history:• No similar illness.No similar illness.• No h/o transfusions, tattooing.No h/o transfusions, tattooing.• Patient was treated by a private practitioner, but Patient was treated by a private practitioner, but

fever tend to recur.fever tend to recur.

• While in hospital the patient While in hospital the patient complained of severe pain in the complained of severe pain in the knee and left ankle pain. knee and left ankle pain.

• The patient gave h/o pain and The patient gave h/o pain and swelling of joints on & off for past 6 swelling of joints on & off for past 6 months for which he was treated with months for which he was treated with analgesics – self medication. The analgesics – self medication. The pain disappear but to re appear later.pain disappear but to re appear later.

• Family history:Family history:

• No h/o PT,BA, DM, SHT, psoriasisNo h/o PT,BA, DM, SHT, psoriasis

• Personal history:Personal history:

• Nil significantNil significant

On examinationOn examination

• Pt conscious, orientedPt conscious, oriented

• Febrile, temp- 100 F.Febrile, temp- 100 F.

• pallor+pallor+

• No icterus, cyanosis, clubbing, pedal No icterus, cyanosis, clubbing, pedal edema, LNE.edema, LNE.

• Pulse- 82/min , regular.Pulse- 82/min , regular.

• BP- 110/70 mm hg.BP- 110/70 mm hg.

• Cvs- s1, s2 heard, no murmurs.Cvs- s1, s2 heard, no murmurs.• RS- nvbs heard, no added soundsRS- nvbs heard, no added sounds• p/a- soft, non tender,p/a- soft, non tender,• Spleen palpable- 2 cm below left Spleen palpable- 2 cm below left

costal margin, soft, non tender.costal margin, soft, non tender.• Liver not palpable, Liver not palpable, • No free fluid.No free fluid.• Cns- no focal deficitsCns- no focal deficits

• On examination of joints:On examination of joints:• The left ankle , left knee , PIP join of The left ankle , left knee , PIP join of

right middle finger joints swollen.right middle finger joints swollen.• Warmth +Warmth +• Tenderness in the joint line+Tenderness in the joint line+• Movements restricted due to pain in Movements restricted due to pain in

the above joints.the above joints.• Other joints appeared normalOther joints appeared normal

• Summary:Summary:

14 year old boy with14 year old boy with

• Low grade feverLow grade fever

• AnemiaAnemia

• SplenomegalySplenomegaly

• Poly arthritisPoly arthritis

A case of feverA case of fever

DR.G.BALAJIDR.G.BALAJI

PROF.DR.G.SUNDARAMURTY’S PROF.DR.G.SUNDARAMURTY’S UNITUNIT

Diferential diagnosisDiferential diagnosis

Septic arthritisSeptic arthritisTuberculosisTuberculosisHemarthrosis.Hemarthrosis.Juvenile idiopathic arthritisJuvenile idiopathic arthritisSLESLETrauma.Trauma.Psoriatic arthritis.Psoriatic arthritis.Enteropathic arthritis-IBDEnteropathic arthritis-IBD

Treatment givenTreatment given

• Iv fluidsIv fluids

• Cetriaxone- ivCetriaxone- iv

• Tab.Artemether- lumefantrine.Tab.Artemether- lumefantrine.

• Paracetamol.Paracetamol.

• NSAID- IndomethacinNSAID- Indomethacin

INVESTIGATIONSINVESTIGATIONS

CBC:CBC:• Hb- 9 gramsHb- 9 grams• TC-4600 cells/cummTC-4600 cells/cumm• DC- N-55,L-45,DC- N-55,L-45,• ESR- 25/45ESR- 25/45• Platelets- 1.5 lakhPlatelets- 1.5 lakh• Rbc-4 million.Rbc-4 million.Rft:Rft:• Urea- 35 mgUrea- 35 mg• Creatinine- 1.0 mgCreatinine- 1.0 mg

Smear for mp/mf- negativeSmear for mp/mf- negative• Widal – negative studyWidal – negative study• Mantoux- negativeMantoux- negative• Chest xray- normal.Chest xray- normal.• MSAT for leptospirosis- negativeMSAT for leptospirosis- negative• Peripheral smear- normocytic normochromic Peripheral smear- normocytic normochromic

anemia, no immature cells seen. anemia, no immature cells seen. • Bone marrow- normal study, no blasts seen.Bone marrow- normal study, no blasts seen.• PT, APTT- Normal.PT, APTT- Normal.

• HIV – negativeHIV – negative• HBS Ag- negativeHBS Ag- negative• anti HCV- negativeanti HCV- negative• Stool examination- occult blood negative.Stool examination- occult blood negative.• Urine culture- no organism grownUrine culture- no organism grown• Blood culture- no organism grown.Blood culture- no organism grown.• RA factor- negative.RA factor- negative.• ANA- positiveANA- positive• Anti DS DNA- negativeAnti DS DNA- negative

• Repeat CBC- Repeat CBC-

• TC-5100 cellsTC-5100 cells

• DC- N- 52, L-45.DC- N- 52, L-45.

• ESR-30/55.ESR-30/55.

• Aspiration from joint – serous fluid+, no pus.Aspiration from joint – serous fluid+, no pus.

• CRP – positive.CRP – positive.

• Anti CCP –POSITIVE.Anti CCP –POSITIVE.

• Serum uric acid- 4.5 mg/dlSerum uric acid- 4.5 mg/dl

IN view ofIN view of

• Age < than 16 yearsAge < than 16 years

• Arthritis, 3 joints involved,Arthritis, 3 joints involved,

• Duration of symptoms> than 6 months.Duration of symptoms> than 6 months.

• Low grade fever, anemia, spleno Low grade fever, anemia, spleno megaly, megaly,

• ANA , anti CCP , CRP postivityANA , anti CCP , CRP postivity

• Final diagnosisFinal diagnosis

• JUVENILE IDIOPATHIC ARTHRITIS.JUVENILE IDIOPATHIC ARTHRITIS.

Juvenile idiopathic arthritisJuvenile idiopathic arthritis

JUVENILE IDIOPATHIC ARTHRITISJUVENILE IDIOPATHIC ARTHRITISAmerican College of Rheumatology Revised American College of Rheumatology Revised CriteriaCriteria

• age of onset < 16 yearsage of onset < 16 years

• arthritis of one or more jointsarthritis of one or more joints

• duration of disease duration of disease >> 6 weeks 6 weeks

• other conditions which present with other conditions which present with arthritis in childhood must be arthritis in childhood must be excludedexcluded

JUVENILE IDIOPATHIC JUVENILE IDIOPATHIC ARTHRITISARTHRITISEpidemiologyEpidemiology

• described in all races and geographic described in all races and geographic areasareas

• incidence: 6 – 19.6 cases/100,000 incidence: 6 – 19.6 cases/100,000 childrenchildren

• prevalence: 16-150/100,000prevalence: 16-150/100,000

• females predominate 2:1females predominate 2:1

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISEtiology and PathogenesisEtiology and Pathogenesis

• unknownunknown

• combination of factorscombination of factors– environment (infection, trauma, stress)environment (infection, trauma, stress)– autoimmunityautoimmunity– immunogeneticimmunogenetic

PATHOGENESISPATHOGENESIS

• The etiology of JRA is unknownThe etiology of JRA is unknown

• Chronic inflammation of synovium is Chronic inflammation of synovium is characterized by B lymphocyte characterized by B lymphocyte infiltration and expansion. infiltration and expansion. Macrophages and T-cell invasion are Macrophages and T-cell invasion are associated with the release of associated with the release of

cytokines, which evoke synoviocyte cytokines, which evoke synoviocyte proliferation.proliferation.

PATHOGENESISPATHOGENESIS

• Studies have suggested exaggerated Studies have suggested exaggerated immune reactivity of several types of immune reactivity of several types of cells in predisposed children, may be cells in predisposed children, may be in response to certain viral infectionsin response to certain viral infections

• There is recruitment of T cells for There is recruitment of T cells for antigens in the joint synoviumantigens in the joint synovium

• This is made possible by certain HLA This is made possible by certain HLA types DR4 (poly) DR8 and DR5 (pauci)types DR4 (poly) DR8 and DR5 (pauci)

PATHOGENESISPATHOGENESIS

• In many patients, predominance of cytokines In many patients, predominance of cytokines associated with tissue destruction, including associated with tissue destruction, including interleukin-6 and TNF, suggests the possibility interleukin-6 and TNF, suggests the possibility of improved responsiveness to specific of improved responsiveness to specific biologic agents targeting these factors.biologic agents targeting these factors.

• A 2001 study by Scola et al found synovium A 2001 study by Scola et al found synovium to contain messenger RNA for vascular to contain messenger RNA for vascular endothelial growth factor, angiopoietin 1, and endothelial growth factor, angiopoietin 1, and their respective receptors, suggesting that their respective receptors, suggesting that induction of angiogenesis by products of induction of angiogenesis by products of lymphocytic infiltration may be involved in lymphocytic infiltration may be involved in persistence of diseasepersistence of disease

EXAMINATIONEXAMINATION

• A detailed physical examination is a critical A detailed physical examination is a critical tool in diagnosing JRA.tool in diagnosing JRA.

• Physical findings are important to provide Physical findings are important to provide criteria for diagnosis and to detect criteria for diagnosis and to detect abnormalities suggestive of other possible abnormalities suggestive of other possible diagnosesdiagnoses

• Arthritis on examination is defined as either Arthritis on examination is defined as either joint swelling (although trauma can also joint swelling (although trauma can also cause swelling and may need to be excluded) cause swelling and may need to be excluded) or the combination of limited motion with pain or the combination of limited motion with pain (on motion or to palpation).(on motion or to palpation).

• The hips and small joints in the spine, The hips and small joints in the spine, when affected by synovitis, do not when affected by synovitis, do not demonstrate swelling but demonstrate demonstrate swelling but demonstrate the combination of loss of motion and the combination of loss of motion and painpain

• In individuals with pauciarticular JRA, 4 or In individuals with pauciarticular JRA, 4 or fewer joints are affected; often, only a fewer joints are affected; often, only a single joint is affected.single joint is affected.

• Typically, large weight-bearing joints, Typically, large weight-bearing joints, knees, and ankles are affected.knees, and ankles are affected.

• In persons with polyarticular JRA, 5 or In persons with polyarticular JRA, 5 or more joints are affected.more joints are affected.

• Weight-bearing joints are affected, and Weight-bearing joints are affected, and symmetric involvement of small joints in symmetric involvement of small joints in the hands is foundthe hands is found

• Pain with decreased range of motion in the Pain with decreased range of motion in the cervical spine is sometimes found.cervical spine is sometimes found.

• Involvement of a few small joints in the Involvement of a few small joints in the hands is atypical and suggests eventual hands is atypical and suggests eventual development of polyarticular JRAdevelopment of polyarticular JRA

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISOnset TypesOnset Types

pauciarticular (pauciarticular (<< 4 joints) 4 joints)

polyarticular (polyarticular (>> 5 joints) 5 joints)

systemic (arthritis with fever and systemic (arthritis with fever and rash)rash)

ILAR Proposed Classification ILAR Proposed Classification CriteriaCriteriaJuvenile Idiopathic Arthritis Juvenile Idiopathic Arthritis

(JIA)(JIA)

systemicsystemicpolyarticular RF+polyarticular RF+polyarticular RF-polyarticular RF-oligoarticularoligoarticular

persistentpersistentextendedextended

psoriatic arthritispsoriatic arthritisenthesitis-related arthritisenthesitis-related arthritisundifferentiated arthritisundifferentiated arthritis

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISClinical features: pauciarticular Clinical features: pauciarticular diseasedisease• 40-60% of patients with JRA.40-60% of patients with JRA.• Preferentially affect girls.Preferentially affect girls.• insidious onsetinsidious onset• morning irritability/stiffness subtlemorning irritability/stiffness subtle• systemic symptoms: usually absentsystemic symptoms: usually absent• large joints (rarely hip), asymmetric large joints (rarely hip), asymmetric

involvement.involvement.• Strong association between HLA A2. HLA Strong association between HLA A2. HLA

DRB1.DRB1.• M/C- knees and ankles.M/C- knees and ankles.• Uveitis- m/c extra articular manifestation.Uveitis- m/c extra articular manifestation.

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISLaboratory Studies: Pauciarticular Laboratory Studies: Pauciarticular DiseaseDisease

• CBC: normalCBC: normal• ESR: usually normalESR: usually normal• ANA: frequently positiveANA: frequently positive• RF: usually negativeRF: usually negative• synovial fluid: inflammatorysynovial fluid: inflammatory• x-ray findings: soft tissue swelling, x-ray findings: soft tissue swelling,

periarticular osteoporosis, growth periarticular osteoporosis, growth disturbance, loss of joint spacedisturbance, loss of joint space

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISClinical features: polyarticular Clinical features: polyarticular diseasedisease• 30-40% of patients with JRA30-40% of patients with JRA

• morning irritability/stiffness more morning irritability/stiffness more prominentprominent

• systemic symptoms: mild to moderatesystemic symptoms: mild to moderate

• large and small joints including cervical large and small joints including cervical spine, symmetric involvementspine, symmetric involvement

• uveitis 5%uveitis 5%

• subtypessubtypes

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISLaboratory features: polyarticular Laboratory features: polyarticular diseasedisease

• WBC WBC , Hb , Hb , platelets WNL to , platelets WNL to • ESR ESR to to • ANA may be positiveANA may be positive• RF may be positiveRF may be positive• Synovial fluid: inflammatorySynovial fluid: inflammatory• X-ray findings: soft tissue swelling, X-ray findings: soft tissue swelling,

periarticular osteoporosis, joint space periarticular osteoporosis, joint space narrowing, erosionsnarrowing, erosions

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISClinical features: systemic diseaseClinical features: systemic disease

• 10-20% of patients with JRA10-20% of patients with JRA

• prominent systemic symptoms: fever, prominent systemic symptoms: fever, rash, lymphadenopathy, rash, lymphadenopathy, hepatosplenomegaly, pericarditis, hepatosplenomegaly, pericarditis, pleuritispleuritis

• arthritis may be absent for months to arthritis may be absent for months to yearsyears

• uveitis uncommonuveitis uncommon

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISLaboratory studies: systemic Laboratory studies: systemic diseasedisease

• WBC WBC , Hgb , Hgb , platelets , platelets to to , , • ESR ESR to to • ANA and RF usually negativeANA and RF usually negative

• x-rays : soft tissue swellingx-rays : soft tissue swelling

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISExtra-articular ManifestationsExtra-articular Manifestations

• generalized or local growth disturbancesgeneralized or local growth disturbances• delayed pubertydelayed puberty• pericarditis, myocarditis, rarely pericarditis, myocarditis, rarely

endocarditisendocarditis• pleural effusion, pleural effusion, • pneumonitis, pulmonary fibrosis- pneumonitis, pulmonary fibrosis-

CAPLAN’s syndromeCAPLAN’s syndrome• hepatitishepatitis• Renal involvement is rareRenal involvement is rare

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISTreatmentTreatment

• supportive not curativesupportive not curative

• involves multidisciplinary team approachinvolves multidisciplinary team approach

• goals:goals:– to suppress articular and/or systemic inflammation to suppress articular and/or systemic inflammation

with as little risk as possiblewith as little risk as possible– to maintain function/prevent disabilitiesto maintain function/prevent disabilities– to foster normal psychological and social to foster normal psychological and social

developmentdevelopment

• heterogenity of disease mandates heterogenity of disease mandates individualizationindividualization

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISTreatment: physical measuresTreatment: physical measures

heat:heat: analgesiaanalgesiamuscle relaxationmuscle relaxation

splinting: splinting: provide joint restprovide joint restmaintain functional positionmaintain functional positioncorrect deformitiescorrect deformities

exercise:exercise: passive, active assisted andpassive, active assisted andactive range of motionactive range of motiongeneral conditioninggeneral conditioning

restrest

Pharmacologic TherapyPharmacologic Therapy

NSAIDsNSAIDs

• GlucocorticoidsGlucocorticoids

• DMARDDMARD

• Anticytokine therapy.Anticytokine therapy.

• Chemo theraputic agents.Chemo theraputic agents.

• Autologous stem cell transplant in Autologous stem cell transplant in severe systemic JIA. severe systemic JIA.

NSAIDNSAID

• Naproxen, ibuprofen, diclofenac, and Naproxen, ibuprofen, diclofenac, and indomethacin.indomethacin.

• Aspirin is not the drug of first choice Aspirin is not the drug of first choice because of the increased frequency of because of the increased frequency of gastric toxicity and hepatotoxicity when gastric toxicity and hepatotoxicity when compared to other NSAID medicationscompared to other NSAID medications

• Predicting which individual patient will Predicting which individual patient will respond to a particular NSAID is not respond to a particular NSAID is not possible; sometimes, after 1-2 months of possible; sometimes, after 1-2 months of treatment, those persons who do not treatment, those persons who do not respond may benefit from changing to a respond may benefit from changing to a different NSAID.different NSAID.

NSAIDsNSAIDs

• Pros:Pros:– Analgesic, Analgesic,

Antipyretic, Anti-Antipyretic, Anti-inflammatoryinflammatory

• Cons:Cons:– Don’t alter disease Don’t alter disease

progressionprogression– Ineffective in Erosive Ineffective in Erosive

diseasedisease

• GI/UlcersGI/Ulcers• HepatotoxicityHepatotoxicity• NephrotoxicityNephrotoxicity• AINAIN• Bleeding – Bleeding –

antiplateletantiplatelet• RashRash• Aseptic meningitisAseptic meningitis

• DMARD:DMARD:

• GOLD saltsGOLD salts

• D- PencillamineD- Pencillamine

• sulfasalazinesulfasalazine

MethotrexateMethotrexate

• may affect immune functionmay affect immune function

• Dose10-25 mg/m2/wk PO/IM/SC as a Dose10-25 mg/m2/wk PO/IM/SC as a single dose or divided into 2 doses qwk; single dose or divided into 2 doses qwk; many pediatric rheumatologists many pediatric rheumatologists increase dose (not to exceed 30 mg/m2, increase dose (not to exceed 30 mg/m2, approximately equivalent to 1 mg/kg); approximately equivalent to 1 mg/kg); administer with folic acid 1-2 mg PO qd administer with folic acid 1-2 mg PO qd or folinic acid 2.5-5 mg PO qwkor folinic acid 2.5-5 mg PO qwk

MethotrexateMethotrexate

• Dihydrofolate reductase Dihydrofolate reductase inhibitorinhibitor– Well tolerated, Well tolerated,

Mono/ComboMono/Combo– Onset: 6-12 weeksOnset: 6-12 weeks

• Metabolism: LiverMetabolism: LiverClearance: KidneysClearance: Kidneys

• Monitoring:Monitoring:– Baseline:CXR, PFTs, HIV, Baseline:CXR, PFTs, HIV,

HBV/HCVHBV/HCV– CBC, LFTs Q4-8 weeksCBC, LFTs Q4-8 weeks– Caution with CRICaution with CRI

• NauseaNausea• Mucosal ulcerationsMucosal ulcerations• Fatigue & Flu-like Fatigue & Flu-like

symptomssymptoms• BM ToxicityBM Toxicity• HepatotoxicityHepatotoxicity

• Treat with Folic acid, 1 Treat with Folic acid, 1 mg/dmg/d

CorticosteroidsCorticosteroids

• Decrease cytokinesDecrease cytokines

• Slow Joint Slow Joint Inflammation.Inflammation.

• Intra lesional Intra lesional triamcinalone triamcinalone hexaceetonidehexaceetonide

• InsomniaInsomnia• Emotional labilityEmotional lability• Fluid retentionFluid retention• Weight gainWeight gain• HTNHTN• HyperglycemiaHyperglycemia• OsteoporosisOsteoporosis

– Bisphosphonates: >5mg/d for Bisphosphonates: >5mg/d for >3months>3months

• CataractsCataracts• Avascular necrosisAvascular necrosis• MyopathyMyopathy• PsychosisPsychosis

LeflunomideLeflunomide

• Inhibits dihydrooratate Inhibits dihydrooratate dehydrogenasedehydrogenase– Dec. activated T-cells Dec. activated T-cells – Onset: rapidOnset: rapid

Efficacy: ≤6 weeksEfficacy: ≤6 weeks

• Monitoring:Monitoring:– CBC, LFTsCBC, LFTs

• Derm - rash, alopeciaDerm - rash, alopecia

• DiarrheaDiarrhea

• BM toxicityBM toxicity

• HepatotoxicityHepatotoxicity

AzathioprineAzathioprine

• Corticosteroid-Corticosteroid-sparingsparing

• Monitoring:Monitoring:– CBC Q1-2 monthsCBC Q1-2 months– AST/ALTAST/ALT

• Infection Infection

• BM ToxicityBM Toxicity

• HepatitisHepatitis

• MalignancyMalignancy

CyclophosphamideCyclophosphamide

• Alkylating agentAlkylating agent

• Monitoring:Monitoring:– CBC, UA monthlyCBC, UA monthly– Yearly UA +/- CytologyYearly UA +/- Cytology

• AlopeciaAlopecia• NauseaNausea• InfectionInfection• BM suppression BM suppression

pancytopeniapancytopenia• Infertility – pretreat Infertility – pretreat

women with women with LeuprolideLeuprolide

• Renal: hemorrhagic Renal: hemorrhagic cystitis, bladder cystitis, bladder malignancy – treat malignancy – treat with mesna.with mesna.

Anticytokine therapyAnticytokine therapy

• Anti-TNF alpha agentsAnti-TNF alpha agents– EtanerceptEtanercept– InfliximabInfliximab– AdalimumabAdalimumab– IL-1 receptor antagonist (Anakinra).IL-1 receptor antagonist (Anakinra).– Abatacept- CTLA-4 IGAbatacept- CTLA-4 IG

TNF-a InhibitorsTNF-a Inhibitors

• Anti-inflammatoryAnti-inflammatory• Block TNF-α Block TNF-α

(proinflammatory (proinflammatory cytokine)cytokine)

• Etanercept, Etanercept, Adalimumab (SQ), Adalimumab (SQ), Infliximab (IV)Infliximab (IV)

• Combo therapy with Combo therapy with MTXMTX

• Injection site Injection site reactionreaction

• InfectionInfection

• Reactivated TBReactivated TB

• InfliximabInfliximab• infusion reactioninfusion reaction

• PancytopeniaPancytopenia

• Autoantibody/SLE-Autoantibody/SLE-likelike

• Exacerbate CHFExacerbate CHF

• Malignancy – Malignancy – lymphomalymphoma

EtanerceptEtanercept• Acts by binding and inhibiting TNF, a cytokine that Acts by binding and inhibiting TNF, a cytokine that

contributes to inflammatory and immune response.contributes to inflammatory and immune response.• Contraindications Documented hypersensitivity; Contraindications Documented hypersensitivity;

sepsissepsis• Interactions -Do not administer within 3 months of Interactions -Do not administer within 3 months of

live virus vaccines live virus vaccines • Precautions Caution in impaired renal function and Precautions Caution in impaired renal function and

asthma; discontinue administration if a serious asthma; discontinue administration if a serious infection develops; adverse effects may include infection develops; adverse effects may include injection site pain, localized erythema, rash, URI injection site pain, localized erythema, rash, URI symptomatology, GI upset, nausea, vomiting, symptomatology, GI upset, nausea, vomiting, rhinitis, and coughrhinitis, and cough

Disease CourseDisease Course

• Long RemissionLong Remission– 10%10%

• Intermittent DiseaseIntermittent Disease– 15-30%15-30%

• Progressive DiseaseProgressive Disease

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISPrognosisPrognosis

• chronic disease which cannot be curedchronic disease which cannot be cured

• characterized by flares and remissionscharacterized by flares and remissions

• after 10 years or more:after 10 years or more:– 31%-55% persistent active disease31%-55% persistent active disease– 31% (9% - 48%) Steinbrocker Class III and IV31% (9% - 48%) Steinbrocker Class III and IV

JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITISPoor Prognostic SignsPoor Prognostic Signs

• pauciarticularpauciarticular– long duration of active diseaselong duration of active disease– conversion to polyarticular disease (30%)conversion to polyarticular disease (30%)– chronic uveitischronic uveitis

• polyarticularpolyarticular– long duration of active diseaselong duration of active disease– articular erosionsarticular erosions– RF positivity/rheumatoid nodulesRF positivity/rheumatoid nodules

• systemicsystemic– conversion to polyarticular disease (25-50%)conversion to polyarticular disease (25-50%)

• References:References:

• KELLY’S RHEUMATOLOGY.KELLY’S RHEUMATOLOGY.

• HARRISONS.HARRISONS.

• Thank youThank you