Download What Is Juvenile Rheumatoid Arthritis?

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BACKGROUND
Chronic Arthritis in Childhood
characterized as
Juvenile Rheumatoid Arthritis
JRA or JIA
Age of onset < 16 years of age.
2
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.
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,.
Overview of JIA
Chronic arthritis in childhood – one of
the more frequent chronic illnesses
of childhood.
 An important cause of short and
long-term disability
JRA - Incidence/Prevalence
 Incidence: (per year)
1/100,000 in Japan
20/100,000 in
Norway
 Prevalence:
 10 /100,000 in
France
 400/100,000 in
Australia

What Is Juvenile Rheumatoid
Arthritis?
Arthritis is a disease in a person’s
joints that cause swelling and pain
where the bones meet each other,
resulting in stiffness and loss of
motion
Juvenile Rheumatoid Arthritis is
arthritis in children under 16 years old
with inflammation lasting at least 6
weeks
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
Etiology
 Immune mediated disease
 Abnormal immunoregulation
 Abnormal cytokine production in the
inflammatory pathway (TNF, IL-6, IL-2R, IL1alpha)
 Complex genetic predispositions
 HLA associations
 Environmental triggers
 Infections
 Trauma
 Stress
Who Gets JRA?
 About 1 child in every 1,000 develops
some type of juvenile arthritis.
 Approximately 75,000 children in the
U.S.
 Can affect children at any age, but
uncommon in the first six months of life
 Peak of Age” 1-3 and 8-12 years
Why Do Kids Get JRA?
 Cause is unknown
 Research suggests a genetic predisposition
 Triggered by an environmental factor, such as a virus
 JRA is an autoimmune disease
 Immune system mistakes healthy cells for harmful
ones.
 Body releases chemicals to fight healthy cells causing
pain and swelling.
CLASSIFICATION OF JRA
 ACR Criteria
 Age at onset: < 16 years of age;
 Arthritis - swelling or effusion or the presence of 2 or more of
the following signs:
 Limitation of range of motion,
 Tenderness or pain on motion and
 Increased heat in one or more joints;
 Duration of disease > 6 weeks;
 Onset type is defined by the type of disease in the first 6
months:
 Oligoarticular (Pauciarticular) < 5 inflamed joints;
 Polyarticular: > 5 inflamed joints;
 Systemic onset: arthritis with characteristic fever.
 Exclusion of other forms of childhood arthritis.
Modified from JT Cassidy, JT Levinson, RM Laxer, CB Lindsley. Textbook of Pediatric Rheum. 2005
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BACKGROUND
 Pathogenesis and Etiology of JRA: Multi-factorial
 Genetic, Hormonal, Immunologic.
 Heterogeneity of JRA
 At least 3 primary types of onset of JRA:
 Pauciarticular (Oligoarticular)
 Polyarticular and
 Systemic
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Types of Juvenile Rheumatoid
Arthritis
 There are three types JRA.
 Pauciarticular: fewer than five joints are affected.
Usually knees and large joints.
 Polyarticular: affects five or more joints. Joints on
both sides of body are affected. Smaller joints like
hands or toes.
 Systemic JRA: affects many parts of the body. Heart,
lungs and liver could be affected as well.
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 3year-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%
Signs/symptoms
 Limping
 Stiffness when awakenin
 Reduced activity level
 Persistent fever
 Joint swelling
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
Oligoarticular
 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
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)
JRA by the Type-of-Onset
Characteristic Pauciarticular
Polyarticular
Systemic
60 (5:1)
30 (3:1)
10 (1:1)
<4
>5
Variable
Age at onset
Early
childhood,
peak 1-2 yr
Thru
childhood,
peak 1-3 yr
Thru
childhood,
no peak
Systemic
involvement
None;
uveitis (++)
Mild;
unremitting
articular
involvement
Systemic selflimited;
chronic
destructive
arthritis ~50%
5-15%
5%
% Cases (F:M)
# Joints
Chronic Uveitis
JT Cassidy, RE Petty, RM Laxer, CB Lindsley. Textbook of Pediatric Rheumatology, 2005
Rare
32
Extra-Articular Manifestations of JRA
Pauciarticular
Fever
Rheumatoid rash
Rheumatoid nodules
Hepatosplenomegaly
Lymphadenopathy
Chronic uveitis
Pericarditis
Pleuritis
Abdominal pain
0%
0
0
0
0
20
0
0
0
JT Cassidy, RE Petty. Textbook of Pediatric Rheumatology, 2001
Polyarticular
Systemic
30%
2
10
10
5
5
5
1
1
100%
95
5
85
70
1
35
20
10
33
How Is Juvenile Rheumatoid
Arthritis Different From Adult
Rheumatoid Arthritis?
 Main Difference: Many people with JRA outgrow
the illness, while adults usually have lifelong
symptoms.
 Studies estimate that by adulthood, JRA
symptoms disappear in more than half of all
affected children.
 Unlike rheumatoid arthritis in an adult, JRA may
affect bone development as well as the child's
growth.
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
Osteopenia
Osteoporosis
CLINICAL MANIFESTATIONS of JRA
37
How Is JRA Diagnosed?
 Complete health history
 Physical examination to look for joint inflammation, rashes,
nodules, signs of internal organ inflammation and/or eye
problems
 Laboratory tests to help rule out other diseases
 Erythrocyte sedimentation rate
 Hemoglobin and blood count testing
 Urinalysis
 X-rays
 Tests of joint, blood and tissue fluids to check for infections or
inflammation
PROGNOSIS OF JRA
 Pauciarticular JRA
 Boys may be affected in older childhood or adolescence;
this may represent an early manifestation of a
spondyloarthropathy.
 Leg length discrepancy from asymmetric knee synovitis
and bone growth may cause flexion contractures, gait
abnormalities and long-term growth abnormalities.
 Eye involvement as anterior uveitis, may lead to scarring
or blindness in ~ 15-20% of children.
46
PROGNOSIS OF JRA
 Polyarticular JRA and Systemic JRA
 Active arthritis into adulthood: 50% to 70% of polyarticular or
systemic onset JRA;
 Long-term disabilities: 30% to 40% of children
 May need major surgery (joint replacement).
 Radiographic joint damage within 2 years;
 Mortality rate: 0.4% to 2% (greater risk with systemic JRA than
with polyarticular JRA).
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Active Learning:
X-Ray of Patient
 One is normal, and one is a patient with JRA:
 Can you determine the differences?
Treatment Options
 Goal: Control symptoms, prevent joint damage,
and maintain function
 1. Non-steroidal Anti-Inflammatory Drugs (NSAIDS)
 Motrin or Advil
 2. Disease Modifying Drugs (DMARDS)
 Hydroxychloroquine: Plaquenil
 Sulfasalazine: Azulfidine
 Methotrexate: Rheumatrex
Medications
 NSAIDs
 DMARDs: Methotrexate,
Plaquenil, Sulfasalazine
 Biologic response
modifiers
 Glucocorticosteroids
 Miscellaneous
Evolving Treatment of JRA
Since the 1990s and
into the 2000s…
Paradigm shift….
The trend in managing JRA is much more aggressive treatment
earlier in the disease course with the goal of preventing joint
damage and slowing progressive articular damage.
51
Surgery
 Rarely used in the early course of disease
 Function:
 Relieve pain
 Release joint contractures
 Replace a damaged joint
Exercise
 Target:
 Shoulder
 Neck
 Lower leg
 Chest
 Hip
 Knee