Juvenile Arthritis Flashcards

1
Q

Juvenile Arthritis Synonyms:

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

CHILDHOOD ARTHRITIS IS NOT

A HOMOGENIOUS ENTITY

A
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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

HOT JOINTS BY THE NUMBERS

A

Pauci <5 joints

  • Mono-arthritis 1 joint
  • Oligo 2-4 joints

Extended Pauci 5-6 joints

Poly 6+ joints

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

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

A

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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Demographics

A

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.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

ACR Classification Criteria for the Diagnosis of JIA

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

JIA - Differential Diagnosis

A
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

kids with limps are what until proven otherwise?

A

leukemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

JIA: Subtype Classification

A

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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Characteristics of Growing Pains

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Systemic JIA: Major Features

A

can be with just one joint involved

  1. Systemic Signs & Symptoms
    Malaise, fever, rash, adenopathy, hepatosplenomegaly, serositis, hepatitis, DIC, anemia
  2. Musculoskeletal Symptoms
    Arthritis, myalgia, arthralgias
  3. Age of Onset
    Usually under 5 yoa
    If under 5 yoa F=M
    If > 5yoa F>M
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

JIA Rash

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

Distribution:

  • Chest
  • Axilla
  • thighs & upper arms

Pruritis unusual

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Systemic Disease Investigations

A
ESR - high
CBC 
- Anemia
- Leukocytosis (inc. polys
- Thrombocytosis
IgM RF - neg
ANA - neg
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Systemic Disease Course and Prognosis

A
  • 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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Systemic Disease Management:

A
Splinting to prevent deformity
Physical and Occupational Therapy
NSAID - pain, fever &amp; inflammation
Corticosteroids - in severe cases
DMARDs - ? benefit
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Pauciarticular Disease: Subtype I

A

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

17
Q

Pauciarticular Disease: Subtype I- Investigations:

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

RF - neg

ANA - freq + (40-75%)

HLA A2, DR5, DRw6, & DRw8

18
Q

Pauciarticular Disease: Subtype I- course and prognosis

A

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
19
Q

Pauciarticular Disease: Subtype I - management

A
Splinting
PT &amp; OT
NSAIDs
Local corticosteroid injections
- controversial
Frequent ophthalmologic assessment and tx
20
Q

Pauciarticular Disease: Subtype II

A

(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)

21
Q

Enthesopathy

A

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

22
Q

Pauciarticular Disease: Subtype II Investigations

A

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

23
Q

Pauciarticular Disease: Subtype II - Course & Prognosis:

A

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

1/3 may develop spondylitis, hip and cervical problems

24
Q

Pauciarticular Disease: Subtype II - Management:

A
PT &amp; OT
Posture training
NSAIDs
? Anti-TNF agents
Local corticosteroid injections
Hip arthroplasty
* Ophthalmologic examination and follow-up
(always)
25
Q

Pauciarticular JIA: Subtype III

A

(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
26
Q

Pauciarticular JIA: Subtype III (Psoriatic)- Investigations

A

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

27
Q

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

A
Young onset (+/-) associated with iritis
Remitting &amp; relapsing, even into adulthood
Occasionally severely destructive
Occasionally spondylitis develops
28
Q

Pauciarticular JIA: Subtype III (Psoriatic) - management

A
PT and OT
Splinting
NSAIDs
May require immunosuppression
- Methotrexate (MTX)
Biologic Agents
- anti-TNF, IL-1ra
29
Q

Polyarticular JRA

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

Polyarticular JIA: RF negative

A

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

31
Q

Polyarticular JIA: RF negative Investigations

A
ESR - increased
CBC:
- Anemia
- Mild leukocytosis
- Thrombocytosis

RF - negative
ANA - occas. positive

32
Q

Polyarticular JIA: RF negative Course and prognosis

A

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

33
Q

Polyarticular JIA: RF negative management

A
Splinting to prevent deformity
PT/OT to maintain &amp; improve joint &amp; muscle function
NSAIDs
DMARDs
Anti-TNF Agents
34
Q

Polyarticular JIA: RF positive

A

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

35
Q

Polyarticular JIA: RF positive - Investigations:

A
ESR – increased 
CBC: moderate anemia
RF – positive, high titre
ANA – may be positive
HLA-DR4 – frequently positive
X-rays – early erosive changes
36
Q

Polyarticular JIA: RF positive Course and prognosis

A

Persistent - serious joint destruction & poor function

Additional long-term hazards:

  • C1-C2 subluxation
  • aortic insufficiency & amyloidosis
37
Q

Polyarticular JIA: RF positive Management

A
Splinting
PT and OT
NSAIDs
DMARDs (MTX)
Biologic Agents (anti-TNF)
Surgical intervention
38
Q

Treatment of JIA

A
  • NSAIDs*
  • Gold*
    Antimalarials
  • Sulfasalazine*
    Methotrexate
    Azathioprine
  • Anti-TNF Agents (Etanercept)*
    Steroids
    Adjunctive treatments
                          *approv
39
Q

Management of Juvenile Arthritis

A

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