Upload
veronica-hodge
View
213
Download
0
Embed Size (px)
Citation preview
JRA is the most frequent chronic arthritis of children.
The evidence of its was discovered from remained
skeleton from 1000 years ago .
It was prescribed at first in the 1800s, and its criteria established in 1973 .
1
Historically, until recently, the terms JRA (in USA),and JCA ( In Europe) ,were used; but recent literatures use JIA instead of them because of :
Only 5-10% of JRA patients havecharacteristic of adult-RF positive RA, so usage ofrheumatoid term may be confusing.
The JRA classification criteria focuses the patternof onset, not the course and progression.
2
Epidemiology
. The true prevalence of JRA is not determined, but
estimates about 1 to 2 per 1000.
. The incidence is about 10 to 20 cases per 100000 children.
. It was reported from all of the world and all ethnics.
. Prevalence and in incidence in Iran were not precisely
defined.
3
Classically ,JRA subdivided to :
1. Oligoarthritis
2. polyarthritis
3. Systemic onset-disease
Its diagnosis requires to
. Onset before 16th birthday
. Objective arthritis in≥ 1joints
. Exclusion of other causes of
childhood arthritis
for ≥ 6 weeks
5
Oligoarthritis
Arthritis in < 4 joints
Persistent type
extended type
at any time during the onset or course of the disease
In first 6 months of disease
but > 5 joints after 6 months
It is the most common type of JRA (60%)
6
Manifestations
The typical child with oligoarticular JRA is a girl who is:
. Often the family notices that the child "walks funny" in
the morning, but after a little while seems fine.
. Usually begins at 1-4 years of age
. limping without complaint.
and unusual after
7 years.
7
.They are often ANA positive( 65-85%)
.They have the greatest risk of uveitis than other types of JRA
(30-50%) .
.Arthritis usually occurs in large joints
Knee
Ankle
Wrist
Elbow
fingers,toes,hip
Early hip or small joints involvement are unusual
8
. Uveitis is the most common complication.
. Leg length discrepancy is another complication
(due to increased blood flow to the inflamed joint) .
. Persistent form has the best articular outcome of all
JRA forms; many cases are benign and resolving
within six months .
Course & Prognosis
10
. Up to 50% of oligo JRA progress to extended form
(30% in first 2 years).
Persistent form Extended form
wrist, hand or ankle Involvement
Symmetric Arthritis Arthritis >1 joint ESRPositive ANA
Risk Factors
11
Polyarthritis JRA
. It is more frequent in girls than boys (3:1).
. It Is an arthritis in > 5 joints during the first 6 months;
and is second most common type of JRA (30%).
. It has a bimodal distribution of the age at onset
although may occur at any age < 16.1-3 years 8-10 years
12
Poly JRA
RF+ RF- (majority)
Usually Girl
Later onset HLA-DR4+symmetric small joint involvement
erosionSubcutaneous nodules prognosis
13
Manifestations
. Large joints generally ( knee, wrist,…) involve, but small
joints (hand, foot) too .
. Often the child does not complain pain ,but has pain on
motion.
. Systemic symptoms or sign is not dominant features, but
fever, organomegaly & adenopathy may be present.
14
. Apophyseal joint involvement (neck pain),
C1-C2 subluxation & TM joint involvement (micrognathia)
may also occur.
. Uveitis also occurs as in oligoJRA but less often (5%) .
. Many children in the younger age group are ANA positive .
15
Course & Prognosis
Onset at later ageEarly involvement of small joints (hand, foot)Hip involvementResistance to treatmentSystemic manifestationsErosionsRF+Nodules
Prognosis
. Prognosis is better for RF- poly JRA than RF+ form, except uveitis that is more common in former.
Arthritis Rheum. 2007 Feb
Systemic-onset Disease
10% of JRA
A child typically 1-6 years (girls=boys), with:
. Fever for ≥ 2 weeks, that is quotidian for ≥ 3 days
(usually afternoon or evening) .
. Characteristic rash
Macule or maculopapule
Transient
Non pruritic
May occur at any site (trunk, proximal of limbs)
and Koebner phenomenon
17
. Arthritis
wrists, knees, and ankles is most typical
hand, hip, cervical spine,..
and may occur with other symptoms or weeks to months later .
. Visceral involvement
Hepatomegaly, hepatitis
Splenomegaly
Generalized adenopathy
Pericarditis
Rarely: pulmonary fibrosis, CNS disease
Uveitis is rare in systemic disease
18
Course & Prognosis
The typical child with systemic onset
relative quiescence of
4-6 months period of fever, rash & varying degree of arthritis
systemic manifestations
But up to 50% evolve chronic polyarthritisThe long term prognosis is determined by the severity of arthritis
20
Poor prognostic sign
Those with > 6 months of fever, thrombocytosis & need for corticostreoid
Life-threatening complication :Macrophage activating syndrome
21
Chronic disease activity may take several different forms
Continue with fever and rash for years, but little progression of their arthritis
Persistent systemic manifestations with progressive arthritis
Resolution of the fever & rash, but have a destructive arthritis
22
Ocular involvement
. Uveitis is a serious complication of JRA.
The frequency of it differs by subtype
Oligo
23
> poly > systemic
Risk factors
Age at onset of arthritis
Duration of disease
ANA+
Subtype of JRA
Uveitis is often initially silent Screening is necessary
Girls
24
Screening
Subgroup Frequency
Any JRA category except SJRA≤ 6 years at onset, ANA +
Any JRA category except SJRA≤ 6 years at onset, ANA -
Any JRA category except SJRA ≥ 7 years at onset, ANA +/-
SJRA
Every 3-4 months for 4 years, then every 6 months for 3 years,then annually
Every 6 months for 4 years,then annually
Every 6 months for 4 years,then annually
Annually
25
JRA
Immunologic Factors
Genetic Factors
EnvironmentalFactors
Familial Predisposition(especially in Oligoarticular form)
HLA II genes (DR1,..)
Non HLA-genes
Ethnicity (European descent > Asian or black) Differ in Incidence and subtype Arthritis Rheum. 2007 Jun
(Especially oilgo & poly forms) B cells RF
(poly forms)
ANA(oligo forms)
Immune complex
complement activation
Differ between oligo form & other forms
NK cell activity
Immunologic Factors
Changes in TH1& TH2 cytokines
TNF-α, IL-1, IL-6 & IL-18
IL-10
Regulatory T-cells (in persistent form of oligo and not extended form)
Activity of systemic JRA
Rubella
EBV
Influenza
Chlamydia
May be explain seasonal variations
Environmental
Factors
A trigger (such as an infectious agent)
Physical Trauma
Psychological Factors
26
Management
. Management of JRA has many components, that
pharmacotherapy has is only one of which.
. Treatment in most children is prolonged over many years.
. Because of the impact of disease on family and child,
they mast be regard in management.
27
. Keeping the child with JRA out of the hospital .
The best management is carried out by multi disciplinary team
28
Aim of treatment
Control of pain
Prevention of loss or restoration of range of motion
promotion of normal growth
Considering psychological aspects
29
So a comprehensive management needs
physical & occupational therapy
Psychologcal care
Nutritional aspectspharmacologic management
30
Growth Retardation& Osteopenia
Disease Effect
Nutrition
Drug side Effects
Mechanical Problems
Control of disease
31
Pharmacologic management
Oligoarthritis
Poor response to NSAIDS
Good respoonseto intra-articular corticostroids
. Not responder to
local injections
. Extended Type
. Small Joint
Involvement
Treat like Polyarthritis
32
Systemic-onset Disease
. Systemic corticosteroids use for arthritis, fever &
serositis .
. Other DMARDS recommend like in polyarthritis; IVIG
occasionally prescribe for systemic manifestations.
. Anakinra recently prescribe as a first DMARD.34