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.
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 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
Its 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
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 American College of Rheumatology 1970
three types of onset: oligo (pauciarticular),
polyarticular, & systemic in the first 6 months of
onset
JCA Juvenile Chronic Arthritis (European League
Against Rheumatism) 1977
JIA Juvenile 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
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
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 Poor Function
years (mean)
Bunim 1959 10 31%

Laaksonen 1966 >16 48%

Ansell 1976 >15 23%

Hill 1976 (14.5) 33%

Hanson 1977 5-25 (10) 28%

Stoeber 1981 10-22 (15) 41%

Levinson 1991 15-20 17%

Zak 2000 28 11%

 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
Diagnostic Studies
 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
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
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 Criteria
1. Systemic
2. Oligoarthritis
a. Persistent
b. 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 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.
 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
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
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 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 often affected
Morning stiffness, joint
pain
Intermittent low-grade
fever
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
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 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: 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
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 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
Oligoarticular ANA+ <7 Q 3-4 months for 7
years at Dx years, then yearly.
Oligoarticular ANA+ 7 or Q 4-6 months for 7 yrs,
older at Dx then yearly.
Oligoarticular ANA - <7 Q 4-6 months for 4 yrs,
years at Dx then yearly.
Oligoarticular ANA Yearly.
<7 years at Dx
Systemic
 Guidelines for ophthalmological screening of
children with JIA
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.
 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:
Sacroiliac joint tenderness and/or
inflammatory lumbosacral pain
Presence of HLA-B27
Onset of arthritis in a male after age 6 years
Ankylosing spondylitis, Enthesitis Related
Arthritis, Sacroiliitis with inflammatory bowel
disease, Reiters 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
Sacroiliitis (90% of cases)
Iritis (20% of cases) generally acute
process
Low grade fevers
Decreased appetite
Medications
NSAIDs
DMARDs:
Methotrexate, Plaquenil,
Sulfasalazine
Biologic response
modifiers
Glucocorticosteroids
Miscellaneous
NSAIDS
FDA approved for
pediatric use
Aspirin
Tolmetin
Naproxen
Ibuprofen
Indomethacin
Meloxicam (Mobic)
Celebrex
Common NSAIDS in JIA
mg/kg/day Max
Naproxen 10-20 1000
Ibuprofen 30-40 2400
Indomethacin 1.5-3.0 200
Tolmetin 20-30 1800
Meloxican 0.25 15
Piroxicam 0.2-0.3 20
Celecoxib 6-12 400
Nabumetone 30 2000
(Relafen)
ASA 80-100 3200
 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: 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:
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:

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
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.
 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)
 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
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