Juvenile Idiopathic Arthritis and other rheumatic diseases in children

JRA – Classification CriteriaJRA – American College of Rheumatology 1970 three types of onset: oligo pauciarticular, polyarticular, & systemic in the first 6 months of developed to achie

JIA and

Other

Rheumatic Diseases in

Children

Norma Liburd, RN-BC, MN

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

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

New classification criteria proposed by the

Pediatric Task Force of the International League

of Associations for Rheumatology (ILAR) in 1997Chronic arthritis in childhood – one of the more frequent chronic illnesses of childhood

An important cause of short and long-term

disability

JRA – Classification Criteria

JRA – American College of Rheumatology 1970 three types of onset: oligo (pauciarticular),

polyarticular, & systemic in the first 6 months of

developed to achieve homogeneity within disease and categories

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

Author Year Published Followup in

years (mean) Poor Function

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)

Diagnostic Studies

Diagnostic Tests

There is no lab test that diagnoses JIA

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

Marginal erosions

Narrowing of

cartilaginous space

EtiologyImmune mediated disease

– Abnormal immunoregulation

– Abnormal cytokine production in the

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

Complex genetic predispositions

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.

3. Polyarthritis (rheumatoid factor negative)

4. Polyarthritis (rheumatoid factor positive)

5. Psoriatic arthritis

6. Enthesitis-related arthritis

7. Undifferentiated arthritis

a. Fits no other category

b. Fits more than one category

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.

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

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

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.

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

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

early recognition)

Associated with CMV, EBV, changes in meds

Mortality 10-20%

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

Five or more joints in the

first 6 months of disease

Asymmetric joint

involvement

Large joints of knees,

wrists, elbows and ankles

Early onset of erosive synovitis

Symmetric joint involvement

Small joints of hands or feet are affectedTMJ: micronathia

Cervical spine may be affected

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 Arthritis in 1 to 4 joints

during the first 6

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 Oligoarticular arthritis fall into this category

Outcome is more typical of RF+ polyarticular

disease

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

Very good in 25% of cases

25% may require surgery for cataracts and/or

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.

Slit Lamp Exam – JIA

Guidelines

Rheumatology & Ophthalmology sections of the

American Academy of Pediatrics, 1993

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

Q 4-6 months for 4 yrs, then 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 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.

Guidelines for ophthalmological screening of

children with JIA

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

Arthritis and enthesitis

Arthritis or enthesitis with at least 2 of the following:

lumbosacral pain

Sacroiliitis with inflammatory bowel disease,

Reiter’s syndrome or acute anterior uveitis in a

first-degree relative.

JRA: Spondyloarthropathy

JIA: Enthesitis related arthritis

Primarily affects boys 8 years and older Affects large joints of lower extremities Heel pain and Achilles tendonitis

NSAIDs DMARDs:

Methotrexate, Plaquenil, Sulfasalazine

Biologic response modifiers

Glucocorticosteroids Miscellaneous

FDA approved for pediatric use

– Aspirin– Tolmetin

– Ibuprofen– Indomethacin– Meloxicam (Mobic)– Celebrex

Common NSAIDS in JIA

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: Biologic Agents:

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

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 Agents:

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

BiologicsActemra (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

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)

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

IV Solumedrol and daily oral Prednisone

systemic flares ~ pericarditis or persistent Sx temporary measure until DMARD is effective

Joint injections - usually under sedation

– Triamcinolone hexacetonide (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

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

Splint fabrication