Juvenile Idiopathic

Arthrithis
Introduction
https://www.youtube.com/watch?v=EEcstaUcXX4
Definition
Juvenile Idiopathic Arthritis (JIA)
Most common chronic rheumatic disease of childhood.
A type of arthritis that causes joint inflammation and stiffness for more than six weeks in a
child aged 16 or younger. It includes groups of diseases that share specific characteristics.

There are 3 main types of JRA:

Pauciarticular /OligoarticularJRA (common)

affects 4 or fewer joints

Polyarticular JRA:
affects 5 or more joints

Systemic JRA (rare)

the symptoms are not usually related tojoints; high fever, loss of appetite, weight loss,
swollen lymph nodes, hepatomegaly, splenomegaly, anemia.
Epidemiology

JIA is the most common chronic rheumatologic disease

of childhood prevalence of 1:1000

Two peaks; one at 1-3 yrs & one at 8-12 yrs

Girls are affected more commonly than boys

JIA Subgroups
JIA subtype % Age Girls% Typical Pattern HLA
B27
Systemic 4-7 4-7 48-70 Any or none 4-11
arthritis
Oligoarthriti 46- 4-5 66-78 Large joints, 11-17
s 54 asymmetric
RF-negative 13- 4-7 76-80 Any, often 10-11
polyarthriti 21 symmetric
s
RF-positive 1-4 11- 83-92 typically symmetric 8-18
polyarthriti 12 arthritis
s
Psoriatic 3-7 8-10 57-69 Spine, lower 8-21
arthritis extremities, distal
interphalangeal
joints
5
Enthesitis- 4-14 10- 9-38 Spine, sacroiliac, 67-76
Pathophysiology
The etiology and pathogenesis of JIA are not completely
understood. Genetic susceptibility plays a major role,
but there is significant overlap between loci associated
with JIA and those associated with other autoimmune
diseases (humoral and cell-mediated immunity)
Presentation
History findings in children with JIA may include the following:

Arthritis present for at least 6 weeks before diagnosis (mandatory for diagnosis of
JIA)

Either insidious or abrupt disease onset, often with morning stiffness or gelling
phenomenon and arthralgia during the day

Complaints of joint pain or abnormal joint use and may have a history of school
absences or limited ability to participate in physical education classes

Spiking fevers occurring once or twice each day at about the same time of day

Evanescent rash on the trunk and extremities

Psoriasis or more subtle dermatologic manifestations

Systemic JIA
The child appears systemically
ill

Arthralgia is often present

Generalized myalgia

Rash: salmon-pink, macular

rash
(often linear) predominantly on
the trunk and the extremities, Systemic juvenile idiopathic arthritis
(JIA) rash
associated
8
with fever spikes
 Systemic JIA
Physical Exam
Characteristics:

Spiking Fever

Hepatospelomegaly

Generalised Rash

9
 Polyarticular JIA
Physical
Characteristics:

Involvement >4
joints

Small joints

Symmetric/Asymm
etric
10
Radiograph of Hands

11
Radiograph of Chest
Hepatosplenomegaly is
often present
Lymphadenopathy is
sometimes present,
especially the axillary
lymph nodes
Muscle tenderness to
palpation may be
observed
Child with pericardial effusion due
Serositis, including pleural to systemic onset juvenile
idiopathic arthritis
and pericardial effusions, 12
may be present
Oligoarticular JIA
Physical Characteristics :

4 or fewer joints (and in

many cases, only 1 joint) are
affected

Large, weight-bearing joints

e.g. knees and ankles are
affected
Eighteen-month-old girl with arthritis
in her right knee. Note the flexion
Asymmetrical 13
Oligoarticular JIA

14
Oligoarticular JIA
Muscle atrophy, often of extensor
muscles

Flexion contractures in the knees and,

less commonly, the wrists are found

Involvement of a few small joints in

the hands is atypical and suggests
eventual development of polyarticular
JIA or psoriatic arthritis.
Dactylitis, or diffuse tenosynovitis of a
finger or toe, also called a "sausage
digit," is more typical of psoriatic
arthritis or enthesitis-related arthritis.

15
Oligoarticular JIA

Anterior uveitis is present in as

many as 20% of children with
oligoarticular and polyarticular
JIA, especially those who are
antinuclear antibody (ANA)
positive

Asymptomatic

16
Oligoarticular JIA - Radiograph of
knees

17
Juvenile psoriatic arthritis
Physical
Characteristics:

Joints: Arthralgia .
Affects especially
distal Interphalangeal
joints

Skin: Plaques

Nails: Pitting
18
Workup and Diagnosis
History and clinical examination
Laboratory evaluation
Acute phase reactants
ANA/Rheumatoid factor
Fluid analysis
Imaging
Radiographs
MRI/ CT/USG

19
Diagnosis
Lab Invest:
Inflammatory markers: Erythrocyte sedimentation rate (ESR) or CRP level (elevated) can be used to monitor disease progression, complement
Complete blood count (CBC) and metabolic panel: throbocytosis, leukocytosis, Lymphopenia (emigration of activated lymphocytes out of the circulation into
synovium)
Liver function tests and assessment of renal function with serum creatinine levels
A complete blood count, liver function tests (to exclude the possibility of viral or autoimmune hepatitis), and assessment of renal function with serum
creatinine levels should be
done before starting treatment with nonsteroidal anti-inflammatory drugs (NSAIDs), methotrexate (MTX), or tumor necrosis factoralpha inhibitors
Antinuclear antibody (ANA) testing: increased risk of anterior uveitis
Rheumatoid factor (RF) and anticyclic citrullinated peptide (CCP) antibody
Additional studies: Total protein, albumin, fibrinogen, ferritin, D-dimer, angiotensin-converting enzyme (ACE), antistreptolysin 0 (AS0), anti-DNAse B,
urinalysis

When only a single joint is affected, radiography is important to exclude other diseases.

Basic radiographic changes in JIA include the following:

Soft tissue swelling
Osteopenia or osteoporosis
Joint-space narrowing
Bony erosions
Intra-articular bony ankylosis
Periosteitis
Growth disturbances
Epiphyseal compression fracture
Joint subluxation
Synovial cysts
Treatment Medical
Management
Steroids intra-articular /oral

Non steroidal anti inflammatory drugs

DMARDS Methotrexate, sulfasalazine,

leflunomide, cyclosporine,thalidomide

Biologicals TNF alpha blockers, IL-1, IL-6

blockers, costimulation inhibitors
21
,Rituximab, IVIg.
Supportive care
Physical therapy

Occupational therapy

Psychological development and growth

Peer group relationship and schooling
Nutrition
Parent counseling

Coordinated care
Pediatric rheumatologist
Opthalmology consultations

22
Surgical management
Need for surgical therapy may be
decreasing as a result of improved medical
management

Procedures that can be done:

Epiphysiodesis

Soft tissue surgery

Arthroplasty
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Complications
Growth retardation and osteopenia

24
TMJ involvement with growth
failure of lower jaw

25
Other Complications
Localised growth disturbances
Sexual maturation, sleep disturbance and fatigue
Macrophage activation syndrome
Amylodosis
Drugs related side effects

26
Prognosis
Common misconception - childhood
arthritis would disappear in
adulthood

Clinical remission in 40-60% (over 10

28 yrs)
Systemic-onset JIA 0-50%
Oligoarthritiscular JIA 50- 80%
Polyarticular JIA 20-30%
ERA 0- 30% 27