Juvenile Arthritis Flashcards by Briana Tillman

Juvenile Arthritis Synonyms:

Juvenile Rheumatoid Arthritis (JRA) = USA
Juvenile Chronic Arthritis (JCA)= Europe
Juvenile Idiopathic Arthritis (JIA)= International League of Rheumatology

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CHILDHOOD ARTHRITIS IS NOT

A HOMOGENIOUS ENTITY

THERE IS NO SINGLE BEST
Characteristic Appearance
Diagnostic Criterion
Treatment
Uniform prognosis

CHILDREN WHO DO NOT FIT INTO ANY CATEGORY SHOULD BE REFERRED TO AS HAVING JUVINILE ARTHRITIS.

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HOT JOINTS BY THE NUMBERS

Pauci <5 joints

Mono-arthritis 1 joint
Oligo 2-4 joints

Extended Pauci 5-6 joints

Poly 6+ joints

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So what do you mean by “Hot Joints” in Kids?

Any swelling or effusion, increased warmth, and/or painful limited movement with or without tenderness in one or more joints lasting more than 6 weeks.

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Demographics

Onset usually under 9 Years of age
Incidence: From 1/1000 up to
12-20 /100,000
30 - 50,000 children in the U.S.

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ACR Classification Criteria for the Diagnosis of JIA

Age at onset < 17 years

Arthritis in 1 or more joints

Duration of disease > 6 weeks

Type of onset of disease during the first 6 mos

Polyarthritis
Pauciarthritis
Systemic Disease- (= Still’s)

Exclusion of other forms of juvenile arthritis

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JIA - Differential Diagnosis

SLE
Reactive Arthritis
Lyme Disease
Dermatomyositis
Kawasaki Disease
Rheumatic Fever
Inflammatory Bowel Dx
Hematologic Dx
Vasculitis
Septic Arthritis
Toxic Synovitis of Hip
Neoplasia
Infantile-onset multi-systemic inflammatory disease
Psychogenic Disease

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kids with limps are what until proven otherwise?

leukemia

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JIA: Subtype Classification

Systemic Onset (Still’s disease)

1 or > joints involved
extra-articular features > 6 weeks

Pauciarticular (Oligoarticular) (50%+)

Subtype I: Classic
Subtype II: Spondylitic
Subtype III: Psoriatic

Polyarticular (30 – 40%)

Rheumatoid Factor (RF) Positive
RF Negative

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Characteristics of Growing Pains

Age: 6-13 yrs of age

Distribution: Lower extremities
- Pain localized to thighs, calves & shins (not joints)
Pain most frequent late in day or night (not morning)

Correlation with strenuous exertion is variable

Normal growth & development

Treatment: heat, massage & analgesics

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Systemic JIA: Major Features

can be with just one joint involved

Systemic Signs & Symptoms
Malaise, fever, rash, adenopathy, hepatosplenomegaly, serositis, hepatitis, DIC, anemia
Musculoskeletal Symptoms
Arthritis, myalgia, arthralgias
Age of Onset
Usually under 5 yoa
If under 5 yoa F=M
If > 5yoa F>M

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JIA Rash

Evanescent (transient) and intermittent
Salmon pink
Circumscribed macular
Size: 2-6 mm or greater
- Often confluent

Distribution:

Chest
Axilla
thighs & upper arms

Pruritis unusual

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Systemic Disease Investigations

ESR - high
CBC 
- Anemia
- Leukocytosis (inc. polys
- Thrombocytosis
IgM RF - neg
ANA - neg

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Systemic Disease Course and Prognosis

Younger age of onset > risk of poor health
somatic and joints

1/2 will have recurrent episodes

1/3 have progressive arthritis

Amyloidosis occurs in some children with persistent disease activity

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Systemic Disease Management:

Splinting to prevent deformity
Physical and Occupational Therapy
NSAID - pain, fever &amp; inflammation
Corticosteroids - in severe cases
DMARDs - ? benefit

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Pauciarticular Disease: Subtype I

General Characteristics:
Age: under 6 years, F>M
Most common (>50%)

Clinical Features:
Joints: less than 5 involved (K, A, E, H)
Early growth abnormalities
** Chronic uveitis w/in 5 yrs (asymptomatic)
- Occurs in 1/3 of cases

Pauciarticular Disease: Subtype I- Investigations:

ESR - increased or wnl
CBC:
- Hb/Hct - wnl
- WBC - wnl
- Platelets - wnl

RF - neg

ANA - freq + (40-75%)

HLA A2, DR5, DRw6, & DRw8

Pauciarticular Disease: Subtype I- course and prognosis

Early detection and management important
Exacerbations and remissions
Long-term prognosis of joints good
- 1/5 develop polyarthritis

Complications:

Alteration in growth of affected limb
2/3 develop iridocyclitis in both eyes

Pauciarticular Disease: Subtype I - management

Splinting
PT &amp; OT
NSAIDs
Local corticosteroid injections
- controversial
Frequent ophthalmologic assessment and tx

Pauciarticular Disease: Subtype II

(Juvenile Spondyloarthropathy)

General Characteristics:
Age: > 9 years, M > F

Clinical Features:
Peripheral arthritis primarily in lower extremities
Enthesopathies
Acute iritis (looks all red/ bloodshot)
** Sacroiliac pain in some
** Axial disease in some
(either of which can be presenting feature)

Enthesopathy

disorder involving the attachment of a tendon or ligament to a bone. This site of attachment is known as the entheses.

Pauciarticular Disease: Subtype II Investigations

ESR - wnl to high
CBC - wnl
RF - neg
** HLA-B27 +

Pauciarticular Disease: Subtype II - Course & Prognosis:

Functional outcome good in 2/3 cases
- Some joint extension may occur

1/3 may develop spondylitis, hip and cervical problems

Pauciarticular Disease: Subtype II - Management:

PT &amp; OT
Posture training
NSAIDs
? Anti-TNF agents
Local corticosteroid injections
Hip arthroplasty
* Ophthalmologic examination and follow-up
(always)

Pauciarticular JIA: Subtype III

(Psoriatic) General Characteristics: - Age: ~8 years, F>M - Family History of psoriasis - Rarely systemic Clinical Features: - Occasional severe destructive arthritis - Dactylitis (swollen/inflamed digit) - Asymmetric peripheral joints - Psoriatic rash, nail pitting/onycholysis - Flexor tenosynovitis

Pauciarticular JIA: Subtype III (Psoriatic)- Investigations

ESR - varies with # of joints, may be high CBC: Hb/Hct - (+/-) low, WBC - (+/-) inc RF - neg ANA - ? +

Pauciarticular JIA: Subtype III (Psoriatic) - Course & Prognosis:

``` Young onset (+/-) associated with iritis Remitting & relapsing, even into adulthood Occasionally severely destructive Occasionally spondylitis develops ```

Pauciarticular JIA: Subtype III (Psoriatic) - management

``` PT and OT Splinting NSAIDs May require immunosuppression - Methotrexate (MTX) Biologic Agents - anti-TNF, IL-1ra ```

Polyarticular JRA

``` General Characteristics: > 5 joints 30% of JRA patients Subgroups: ** RF (+) --> adolescent, severe, similar to adults RF (–) --> milder disease ```

Polyarticular JIA: RF negative

General Characteristics: Any age, occasionally uner 1 year, F > M Clinical Features Can affect any joint (K, W, A, PIP & DIP) Reduced neck and TMJ ROM Flexor tenosynovitis +/- low grade fever Mild lymphadenopathy & hepatosplenomegaly

Polyarticular JIA: RF negative Investigations

``` ESR - increased CBC: - Anemia - Mild leukocytosis - Thrombocytosis ``` RF - negative ANA - occas. positive

Polyarticular JIA: RF negative Course and prognosis

Variable May be monocyclic but prolonged with good functional outcome Recurrent episodes tend to cause progressive deformities

Polyarticular JIA: RF negative management

``` Splinting to prevent deformity PT/OT to maintain & improve joint & muscle function NSAIDs DMARDs Anti-TNF Agents ```

Polyarticular JIA: RF positive

General Characteristics: > 8 years @ onset, F > M Clinical Features: Polyarthritis of any joint (small joints of W, H, A, F; K & H early) Rheumatoid nodules Vasculitis - uncommon & late No HVLA!!! Cervical subluxation particularly dangerous

Polyarticular JIA: RF positive - Investigations:

``` ESR – increased CBC: moderate anemia RF – positive, high titre ANA – may be positive HLA-DR4 – frequently positive X-rays – early erosive changes ```

Polyarticular JIA: RF positive Course and prognosis

Persistent - serious joint destruction & poor function Additional long-term hazards: - C1-C2 subluxation - aortic insufficiency & amyloidosis

Polyarticular JIA: RF positive Management

``` Splinting PT and OT NSAIDs DMARDs (MTX) Biologic Agents (anti-TNF) Surgical intervention ```

Treatment of JIA

* NSAIDs* * Gold* Antimalarials * Sulfasalazine* Methotrexate Azathioprine * Anti-TNF Agents (Etanercept)* Steroids Adjunctive treatments *approv

Management of Juvenile Arthritis

Accurate assessment of each case Consideration of whole child & family Treatment of Arthritis Treatment of Extra-Articular Manifestations