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Childhood Arthritis

RCSI students
2013

Arthralgia Or Arthritis
Arthralgia : pain in the joint
Arthritis : Swelling or any 2 of
Warmnethness
Tenderness
Limited movements

Scenario 1
A 2 years old child ,c/o left knee swelling for 6 days
preceded by trauma while jumping in kindergarten.
Hx of fever and unable to bear on weight. X ray of
left knee show no fracture.

What is your impression ??


Infective arthritis

Scenario 2
A 18 month old girl ,c/o of right hip pain with limping
for 3 days that gradually improved. Hx of cold, fever,
coryza preceded the pain episode.

What is your impression ??


Reactive arthritis

Scenario 3
4 years old boy, Hx of knees pain, intermittent,
mainly at night after playing all the day, relieved by
massage and pain killer. O/E healthy boy with normal
knees and normal lab results.

What is your impression ??


Growing pain

Scenario 4

7 years old girl presented with right ankle swelling, sustained


for 1 day followed by left knee swelling for few hours and then
right wrist pain and swelling. Hx of fever for 7 days and sore
throat 2 weeks ago.

What is your impression ??


Acute rheumatic fever

Scenario 5
4 years old girl with Hx of right knee swelling and
low grade fever for 3 months, no Hx of trauma, skin
rash. O/E right knee swelling, little tender and other
system exam. Uneventful.

Impression??
Chronic arthritis, ?? JIA

Arthritis in children
Either

Inflammatory or NON Inflammatory


How long ?

Inflammatory :
Infection: Septic arthritis, Osteomyelitis
Rheumatic disorders: SLE,JIA
Neoplastic disorders:
Could be;
1.
2.

Acute
Chronic

Arthritis in children
NON inflammatory:
1. Growing pain; peak age:4-5 years. poplitial fossa.
2. Hypermobility syndrome.
3. Reflex neurovascular dystrophy;
Hyperesthesia, mottled skin coloring and vascular instability.

Juvenile Idiopathic arthritis

Definition
Juvenile Idiopathic arthritis (JRA) is a chronic arthritis that persists for a
minimum of six consecutive weeks in one or more joints, commencing
before the age of 16 years and after active exclusion of other causes.

Criteria for the diagnosis of JRA


ACR criteria

Age at onset < 16 years


Arthritis
Duration of disease 6 weeks or longer

Exclusion of other form of arthritis

Classification(ILAR 2005)
By mode of onset during the first six months
A.

Systemic: arthritis, rash and fever for 2 weeks

B.

Pauci-articular; < 4 joints

C.

Extended pauci-articular; exceeding 4 joints after 6 months of


onset of arthritis

D.
E.

Poly-articular RF + ; > 5 joints


Poly-articular RF - : > 5 joints

Classification (contd)
F. Enthesitis-related arthritis
G. Psoriatic arthritis
H. Others: arthritis of unknown cause that begins during
childhood and persists for at least 6 weeks.

Systemic onset JRA

Age: < 5 years


M:F: equal < 5 years
More in female after 5 years
C/F: - High remittent fever
- Evanescent rash
- Malaise
- Weight loss
- Lymphadenopathy
- Hepatosplenomegally
- Serositis
Most patients develop arthritis within 3 months.

Prognosis
Episodes of systemic
disease
Progressive arthritis
Poor growth
Vasculitis

Pauciarticular JRA

Age: 1-3 years


F:M: 4:1
C/F - < 4 joints, large joints
- growth anomalies
Other system involved
Eyes: Iridocyclitis (uveitis) in the first five years
Slit lamb exam.
Complications:Band keratopathy,cataract
Lab findings: ANA + (40 75%)
Prognosis: 60% Remission

Pauci-JIA
Pauci-JIA

synovial cyst

Polyarticular JRA RF

Any age
More in Female

C/F

- Large and small joints (sparing mcps)


- Micrognathia
- Tenosynovitis
- Joints deformity
- Erosions

Polyarticular JRA RF -

Polyarticular JRA-RF +
Over 8 years of age
More in Female
Resemble RA in adult
C/F

All joints including MCPs


Rheumatoid nodules (elbow)
Progressive arthritis

Prognosis: Serious joints destruction


Vasculitis

Enthesitis related arthritis

Either of the followings


1. Arthritis and enthesitis or
2. Arthritis or enthesitis with at least two of the following signs
and symptoms:
a. Tenderness in the sacroiliac joint
b. Positive for HLA B27
c. Any relative with second degree of relationship has HLA-B27
related disease
d. Anterior uveitis
e. A boy age 8 years or older has developed arthritis

Enthesitis related arthritis


Age: 8 years and more
Male predominant
C/F

- Peripheral arthritis
- Enthesopathies Planter Fascia,
Achilles tendon
- Acute Iritis
- Sacroiliac pain
- Axial disease
HLA associations: 50% - 75% HLAB27

Pathogenesis of JIA
Synovitis of JRA characterised by villus hypertrophy and
hyperplasia and hyperaemia and oedema of sub synovial tissue
Pannus formation.

Progressive erosion of articular cartilage and contiguous bone


Excessive immune reactivity of several type of cells in
predisposed children (? viruses)
Recruitment of T cells is made possible by certain HLA type found
with increased frequency in affected children
T cells and B cells involved

Diagnosis
No one path gnomonic criteria of these diseases in
children
Diagnosis based on a history compatible with
inflammatory joints disease and a physical
examination that confirm presence of objective
arthritis.
Lab abnormality ( ESR, CRP, Low Hb) support the
diagnosis in absence of results suggest different Dx.

Lab Findings
CBC: low Hb, High WBC, High Plat,
ESR, CRP, Globulin, immunoglobulin.
High ferritin.
ANA in 40-85% of pauci JIA
ANA, homogenous or speckled, associated with increase risk of
developing uveitis
RF ,older children with poly JIA, portends a poor prognosis.
consecutive positive tests over a defined period.

Radiology Findings

Radiographic changes: soft tissue swelling,


osteoporosis, periostitis sub- chondral erosions,
narrowing of cartilage space
Varying degree of bony destruction and fusion
MRI

Radiology Findings

X-ray sacroiliac joint in ERA

Differential diagnosis
Systemic onset JIA:
Infection (EBV,CMV,TORCH)
Collagen vascular disease; SLE, others
Malignancies; ALL, others.
Other types of JIA;
CVD
Malignancies

Management of JRA
Team approach

Rheumatologist
Ophthalmologist
Physiotherapist
Psychologist

Treatment of JRA
Drugs
Physiotherapy
Occupational therapy

Treatment of JIA
5. Biological agents (anti
TNF,Anti-IL6,Anti-IL1)

4. Cortico-steroids,
Azathioprine, cyclosporine

3. Methotrexate

2. IA steroid, hydroxychloroquine

1. Non-steroidal antiinflammatory drugs.

First line treatment of JIA

NSAID
Empirical
Aspirin, Naproxen, Ibuprofen
Try 3 NSAID for 2 month each
Monitor CBC, LFT, Urea, Creatinine
Intra-articular steroid.

Management of JRA
When we use corticosteroid for JIA
Overwhelming systemic illness
Bridge therapy,lower doses
Intra Articular injection for acute, or synovial cyst.
Ophthalmic use; uveitis
Slit lamp ophthalmic examination
Calcium intake

Second line therapy in JIA

First choice: Methotrexate


Effective
Safe
Tolerable

The response after 8 weeks


Clinical response
Reduction of inflammatory
markers

Dose: 10-20mg /m2/wkly


Oral, sc, lm
Folic acid 5 mg (1day after)

Monitoring: CBC, LFT, U/Cr every 46 wks

DMARDs (contd)

Sulphasalazine
Polyarticular JRA HLA B27 related diseases
Effective
Safe
Mechanism: adenosine release
Cyclosporine A
Refractory JRA
Mechansim:
Dose: 3.5 5mg/kg/day
Blocks IL2 gene
Inhibits T-cell activation
Monitor: blood pressure, urea, creatinine
Combination with MTX

Uveitis

Monitoring of uveitis; Slit lamp examination

Treatment: Mydriatics and steroid eye drops


No response after 6 months
Sub tenon injection of steroid
Oral prednisolone
NSAID
Refractory Uveitis
Cyclosporine A, azathioprine
Surgery

Physiotherapy

Relief of pain
Ice/heat packs
Tens

Maintenance of joint function


Stretch
Active exercises
Splints
Night
Day
Corrective
Serial Casting
Appropriate foot wear

Occupational Therapy

Hand Function

Joint range of movements


Grip strength
Muscle power
Deformity
Functional ability
Sensation

Hydrotherapy

Warm water
Support body/limbs
Assists joints movement
Resist movements

Counseling of the Family

Education

Sharing management

Psychologic and social development


Regular attendance at school
Emotional support for family and children with JRA
Team effort should include social workers