Jia and other rheumatic diseases in children
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JIA and Other Rheumatic Diseases in Children. Norma Liburd , RN-BC , MN. Objectives. Define Juvenile Idiopathic Arthritis (JIA) and discuss the diagnostic criteria. Identify the subtypes of JIA and discuss characteristics of each.

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Jia and other rheumatic diseases in children

JIA and Other Rheumatic Diseases in Children

Norma Liburd,

RN-BC, MN


Objectives

Objectives

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

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

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


A few more objectives

A few more Objectives

  • Discuss three school related problems students with JIA have and intervention strategies for each.

  • Identify the criteria for classification of systemic lupus erythematosus.

  • Name the most common type of juvenile localized scleroderma.

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


Overview of jia

Overview of JIA

  • New classification criteria proposed by the Pediatric Task Force of the International League of Associations for Rheumatology (ILAR) in 1997

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

  • An important cause of short and long-term disability


Chronic arthritis in childhood jia

Chronic arthritis in childhood: JIA

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


Jra incidence prevalence

JRA - Incidence/Prevalence

  • Published series are difficult to interpret due to classification, methodologies, heterogeneity

  • Incidence: (per year)

    1/100,000 in Japan

    20/100,000 in Norway

  • Prevalence:

    • 10 /100,000 in France

    • 400/100,000 in Australia

    • 113/ 100,000

  • Arthritis Foundation:

    300,000 children in the US have chronic arthritis.

    .


Jra classification criteria

JRA – Classification Criteria

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

  • JCAJuvenile Chronic Arthritis (European League Against Rheumatism) 1977

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


Sex ratio

Sex Ratio

  • All types of JIA:

    • Girls: Boys 2:1

  • Oligo JIA:

    • Girls: Boys 3:1

  • JIA with uveitis

    • Girls: Boys 5-6:1

  • Poly JRA:

    • Girls: Boys 3:1

  • Systemic JRA:

    • Girls: Boys approx. 1:1


Jia outcomes mortality

JIA outcomes: Mortality

Disease associated death rate is

< 1% in Europe

< 0.3% in North America

These numbers represent a

4 Fold to 14 fold Increase in Mortality Rate

Compared with General Population

Causes are cardiac, infection & macrophage activation syndrome


Jra outcome functional abilities

JRA outcome: functional abilities


Classification criteria for jia

Age at onset <16 years

Duration of Arthritis: 6 weeks

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

Limitation of ROM

Tenderness or pain on motion

Increased heat

Exclusion of other diseases

Classification Criteria for JIA


Diagnostic studies

Diagnostic Studies


Diagnostic tests

Diagnostic Tests

  • There is no lab test that diagnoses JIA

    The H&P should determine the labs, not the reverse

    • CBC

    • Rheumatoid factor

    • Antinuclear Antibody (ANA) – with titer

    • ESR or CRP

    • Anti-CCP (anti-cyclic citrullinated protein)


Radiologic studies

Radiologic Studies

X-rays

Soft tissue swelling

Osteoporosis

Periosteal new bone formation

Epiphyseal overgrowth

Marginal erosions

Narrowing of cartilaginous

space

Joint subluxation

Bony fusion

Dexascans

Osteopenia

Osteoporosis


Etiology

Immune mediated disease

Abnormal immunoregulation

Abnormal cytokine production in the inflammatory pathway (TNF, IL-6, IL-2R, IL-1alpha)

Complex genetic predispositions

HLA associations

Environmental triggers

Infections

Trauma

Stress

Etiology


Jia and other rheumatic diseases in children

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


Jia and other rheumatic diseases in children

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 criteria

Classification Criteria

  • 1. Systemic2.Oligoarthritis

    a. Persistentb. Extended

    3.Polyarthritis (rheumatoid factor negative)

    4.Polyarthritis (rheumatoid factor positive)

    5. Psoriatic arthritis

    6.Enthesitis-related arthritis

    7. Undifferentiated arthritis

    a. Fits no other categoryb. Fits more than one categoryFrom 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.


Jia subtypes

JIA Subtypes

  • Systemic Onset (5-15%)

  • Polyarticular Onset (20%)

    • Rheumatoid Factor Positive

    • Rheumatoid Factor Negative (85%)

  • Oligoarthritis (50-80%)

  • Juvenile psoriatic arthritis (7%)

  • Enthesitis related arthritis

  • Undifferentiated


Systemic jia

Systemic JIA

  • Definition:

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

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

      • Evanescent, non-fixed, erythematous rash

      • Generalized lymph node enlargement

      • Hepatomegaly and/or splenomegaly

      • Serositis


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 jra

Systemic JRA

  • Rash -

    • Salmon colored

    • Maculopapular – flat to slightly raised

    • Trunk and extremities

    • Migratory

    • Pruritic 5%

    • Fleeting

    • Persistent with fever spike


Overview of systemic jia

Overview of Systemic JIA

  • 10-15% of all JRA patients

  • Broad peak of onset 1-5 years

  • M:F 1:1

  • Variable number of joints

  • Il-6 is elevated and correlates with disease activity

  • Extraarticular symptoms:

    • Fever 100 %

    • Rash 95%

    • Hepatosplenomegaly, 85%

    • Lymphadenopathy 70%

    • Pericarditis 35%

    • Pleuritis 20%


Macrophage activation syndrome

Macrophage Activation Syndrome

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

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

  • Early recognition is life-saving

    • Looks somewhat like a flare up of systemic JRA but is different enough to allow for early recognition)

  • Associated with CMV, EBV, changes in meds

  • Mortality 10-20%


Macrophage activation syndrome1

Macrophage Activation Syndrome

  • Acute onset of fever with

    • Bruising, purpura, mucosal bleeding

    • Enlarged lymph nodes, liver, spleen

    • Elevated AST, ALT, PT, PTT, fibrin D-dimer

    • Elevated ferritin & triglycerides

    • Abrupt fall in WBC & platelets

    • Fall in ESR

    • Fall in fibrinogen, clotting factors

  • Often progresses to fatal DIC, hepatic failure, encephalopathy

  • Treatment: IV steroids, cyclosporin


Polyarticular jia rf negative

PolyarticularJIA - RF negative

  • Five or more joints in the first 6 months of disease

  • Asymmetric joint involvement

  • Large joints of knees, wrists, elbows and ankles often affected

  • Morning stiffness, joint pain

  • Intermittent low-grade fever


Polyarticular rf positive

Polyarticular- RF positive

  • Arthritis affecting 5 or more joints in the first 6 months of disease.

  • Similar to adult RA

  • Females with onset in adolescence

  • Rheumatoid nodules

  • Early onset of erosive synovitis

  • Symmetric joint involvement

  • Small joints of hands or feet are affected

  • TMJ: micronathia

  • Cervical spine may be affected


Rheumatoid nodules

Rheumatoid Nodules

  • Occur in 5-10% of children with JIA

  • Most frequently on elbow

  • Pressure points, digital flexor tendon sheaths, Achilles tendons, bridge of nose in child who wears glasses

  • Firm or hard, usually mobile, nontender.

  • Solitary or multiple, may change in size, may last months to years.


Oligoarticular jia

Oligoarticular JIA

  • Arthritis in 1 to 4 joints during the first 6 months of disease

  • Girls 1 to 4 years

  • Knees, ankles, elbows

  • Painless swelling of joints is common

  • Uveitis: insidious, subacute

  • 15-20% have uveitis


Jia and other rheumatic diseases in children

JIA: Oligo – persistent

  • No more than 4 joints affected throughout the

    disease course

    JIA: Oligo - extended

  • Affects a total of more than 4 joints after the first 6 months of disease. At least 1/3 of children with Oligoarticulararthritis fall into this categoryOutcome is more typical of RF+ polyarticular disease


Uveitis in jia

Uveitis in JIA

  • Intraocular inflammation affects iris and ciliary body

  • Usually insidious and may be asymptomatic

  • Activity of eye does not parallel joint disease

  • Slit lamp exam detects anterior chamber inflammation

  • Girls, ANA + and onset before age 7 at higher risk


Prognosis of uveitis in jia

Prognosis of Uveitis in JIA

  • Very good in 25% of cases

  • 25% may require surgery for cataracts and/or glaucoma

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

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


Uveitis in jia1

Uveitis in JIA

  • Usually occurs after onset of arthritis. Highest risk is within 2 years of onset of arthritis. Majority develop eye disease within 5-7 years after onset

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

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

  • Enbrel.


Jia and other rheumatic diseases in children

OligoarticularANA+<7 years at Dx

Oligoarticular ANA+ 7 or older at Dx

Oligoarticular ANA - <7 years at Dx

Oligoarticular ANA –

<7 years at Dx

Systemic

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

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

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

Yearly.

Slit Lamp Exam – JIA GuidelinesRheumatology & Ophthalmology sections of the American Academy of Pediatrics, 1993


Jia and other rheumatic diseases in children

Guidelines for ophthalmological screening of children with JIA

High risk – screen every 3 months

Moderate risk – screen every 4-6 months

Low risk: screen every 12 months

All 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.


Jia psoriatic arthritis

JIA: Psoriatic Arthritis

  • Arthritis and psoriasis or

  • Arthritis with 2 of the following:

    • Dactylitis - sausage like swelling of toe or finger

    • Nail pitting

    • Psoriasis in a first degree relative (parents, siblings)

  • Slightly more females

  • Symmetrical involving large and small joints


Jra spondyloarthropathy jia enthesitis related arthritis

JRA: SpondyloarthropathyJIA:Enthesitis related arthritis

  • Arthritis and enthesitis

  • Arthritis or enthesitis with at least 2 of the following:

    • Sacroiliac joint tenderness and/or inflammatory lumbosacral pain

    • Presence of HLA-B27

    • Onset of arthritis in a male after age 6 years

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


Jra spondyloarthropathy jia enthesitis related arthritis1

JRA: SpondyloarthropathyJIA:Enthesitis related arthritis

  • Primarily affects boys 8 years and older

  • Affects large joints of lower extremities

  • Heel pain and Achilles tendonitis

  • Sacroiliitis (90% of cases)

  • Iritis (20% of cases) generally acute process

  • Low grade fevers

  • Decreased appetite


Medications

Medications

  • NSAIDs

  • DMARDs: Methotrexate, Plaquenil, Sulfasalazine

  • Biologic response modifiers

  • Glucocorticosteroids

  • Miscellaneous


Nsaids

NSAIDS

  • FDA approved for pediatric use

    • Aspirin

    • Tolmetin

    • Naproxen

    • Ibuprofen

    • Indomethacin

    • Meloxicam (Mobic)

    • Celebrex


Common nsaids in jia

Common NSAIDS in JIA

mg/kg/dayMax

Naproxen 10-20 1000

Ibuprofen30-402400

Indomethacin1.5-3.0200

Tolmetin20-301800

Meloxican0.2515

Piroxicam0.2-0.320

Celecoxib6-12400

Nabumetone302000

(Relafen)

ASA80-1003200


Methotrexate

Methotrexate

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

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

  • Labs every 6 weeks: CBC, CMP

  • No alcohol

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


Meds targeting inflammation

Meds: Targeting inflammation


Meds biologic agents target against cytokines involved in inflammation tnf il 1ra il 6

Meds: Biologic Agents: Target against cytokines involved in inflammation: TNF , IL-1Ra, IL-6

  • Enbrel (Etanercept): approved for JRA

    • 0.4 mg/kg twice per week SQ injections

    • Improvement by third to fourth dose

    • Hold for suspected bacterial infection, varicella

    • Site reactions

    • Binds to TNF


Biologic agents

Biologic Agents:

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

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

  • Improvement can be seen after first dose

  • Labs every 4-8 weeks (CBC, CMP)

  • Not approved for children


Biologic agents1

Biologic Agents:

  • Anakinra (Kineret) – (blocks IL-1 which stimulates synoviocytes and chondrocytes to produce small inflammatory mediators – leading to cartilage destruction and bone erosions.

    • Used in systemic JRA (but not approved)

    • Daily, very painful, SQ injections, rotation of sites is important


Biologics

Biologics

  • Actemra (Tocilizumab) 8 mg/kg

    • ACTEMRA is indicated for the treatment of active systemic juvenile idiopathic arthritis in patients 2 years of age and older who have responded inadequately to 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.


Biologics1

Biologics

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

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

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

  • Humira pen – or prefilled syringe

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

  • Can shorten interval to weekly (with auth)


Biologics2

Biologics

  • Orencia (Abatacept) T-lymphocyte modulator

IV over 30 minutes: at 0, 2 4 weeks, then every 4 weeks

Approved 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


Glucocorticosteroids

Glucocorticosteroids

  • IV Solumedrol and daily oral Prednisone

    • systemic flares ~ pericarditis or persistent Sx

    • temporary measure until DMARD is effective

  • Joint injections - usually under sedation

    • Triamcinolonehexacetonide (Aristaspan)

      • long acting steroid

      • Works best with large joints


  • Miscellaneous treatment

    Thalidomide: 2 mg/kg/day

    Mechanism of action probably by effects on TNF and other inflammatory cytokines

    Very rigorous patient monitoring

    Bone Marrow Transplant

    Experimental for severe autoimmune disease unresponsive to conventional therapy

    Autologous stem cell transplant being evaluated in small number of children

    Infections ~ very risky – high death rate

    Miscellaneous Treatment


    Pt ot overall goals

    PT/OT - Overall goals

    • Maintain or restore functional ROM in joints

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

    • Assist child to perform activities in ways as close to normal as possible

      • so they do not feel “different” from peers.


    Pt ot management in jia

    PT/OT - Management in JIA

    • Splint fabrication


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