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MSK II Exam 2 > Juvenile Arthritis > Flashcards

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

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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.

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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

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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.

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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.

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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

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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
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8
Q

kids with limps are what until proven otherwise?

A

leukemia

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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
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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

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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
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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

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13
Q

Systemic Disease Investigations

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

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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
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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

MSK II Exam 2 (16 decks)

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