Ch. 155 Juvenile Idiopathic Arthritis Flashcards

(50 cards)

1
Q

MC rheumatic disease in children

A

JIA (JRA accdg to ACR)

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2
Q

MC subtype of JIA in order

A

1) Oligoarthritis 40-50% 2) Poly- 3) Systemic

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3
Q

Gender predominance in sJIA

A

None

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4
Q

Gender predominance in oligo- and polyarticular JIA

A

F>M

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5
Q

Peak of onset of oligoarthritis

A

2-4 y/o

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6
Q

Peak of onset of polyarthritis

A

Bimodal, 2-4 y/o and 10-14 y/o

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7
Q

Peak of onset of sJIA

A

1-5 y/o

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8
Q

Non-genetic triggers for JIA

A

1) Viral and bacterial infections 2) Enhanced immune responses to bacterial or mycobacterial heat shock proteins 3) Abnormal reproductive hormone levels 4) Joint trauma

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9
Q

Components of the immune system affected in JIA

A

Both humoral and cell-mediated immunity

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10
Q

Cell type that plays a central role in the pathogenesis of JIA

A

T lymphocytes

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11
Q

Inflammatory synovitis in JIA is characterized pathologically by

A

Villous hypertrophy and hyperplasia with hyperemia and edema of synovial tissue

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12
Q

Advanced and uncontrolled JIA leads to

A

1) Pannus formation 2) Progressive erosion of articular cartilage and contiguous bone

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13
Q

Must be present to make a diagnosis of any JIA subtype

A

Arthritis

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14
Q

Criteria for classification of JRA

A

1) Age of onset <16 yrs 2) Arthritis in >1 joint 3) Duration ≥6 weeks Exclusion of other forms of juvenile arthritis

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15
Q

Arthritis of JRA is defined as

A

Swelling or effusion or presence of 2 or more of the ff: 1) Limitation of ROM, tenderness or pain on motion 3) Increased heat

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16
Q

Polyarthritis is defined as

A

≥5 inflamed joints

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17
Q

Oligoarthritis is defined as

A

≤4 inflamed joints

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18
Q

Arthritis + rash + characteristic quotidian fever

A

Systemic JIA

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19
Q

ILAE Classification of JIA: Systemic onset JIA

A

1) ≥1 joint with or preceded by fever of at least 2 weeks, daily, quotidian for at least 3 days 2) Evanescent erythematous rash 3) Generalized LN enlargement 4) Hepatomegaly and/or splenomegaly 5) Serositis

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20
Q

ILAE Classification of JIA: Oligoarticular JIA

A

1) 1-4 joints during first 6 months 2) PERSISTENT: 1-4 throughout entire disease course 3) EXTENDED: >4 after 1st 6 months

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21
Q

ILAE Classification of JIA: Polyarthritis RF negative

A

≥5 joints during 1st 6 months, RF negative

22
Q

ILAE Classification of JIA: Polyarthritis RF positive

A

≥5 joints during 1st 6 months, RF positive in ≥2 tests at least 3 months apart during 1st 6 months of disease

23
Q

Spondyloarthropathies: Psoriatic arthritis, criteria

A

Arthritis + psoriasis OR arthritis + at least 2 of the ff: 1) Dactylitis 2) Nail pitting and onycholysis 3) Psoriasis in a 1st degree relative

24
Q

Spondyloarthropathies: Enthesitis-related arthritis, criteria

A

Arthritis + enthesitis or arthritis or enthesitis with at least 2 of the ff: 1) Presence or history of SI joint tenderness and/or inflammatory lumbosacral pain 2) HLA-B27 positive 3) Onset >6 y/o in a male 4) Acute symptomatic anterior uveitis 5) History of ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis, with IBD, Reiter syndrome, or acute anterior uveitis in a 1st degree relative

25
HLA-B27 positive
Enthesitis-related arthritis
26
T/F In JIA, involvement of the hip is almost never a presenting sign
T, suggests a spondyloarthropathy or a nonrheumatologic cause
27
Confers increased risk for asymptomatic anterior uveitis
(+) ANA
28
PE finding that reflects chronic temporomandibbular joint disease
Micrognathia
29
Rash of systemic JIA
Evanescent, salmon-colored, linear or circular, most common over trunk and proximal extremities
30
Rare but potentially fatal complication of SoJIA
MAS
31
Clinical manifestations of SoJIA
Acute profound anemia, thrombocytopenia, leukopenia, high spiking fevers, lymphadenopathy, hepatosplenomegaly
32
Preliminary diagnostic guidelines for MAS complicating JIA: Lab criteria
1) Decreased platelet count 2) Elevated AST 3) Decreased WBC 4) Hypofibrinogenemia
33
Preliminary diagnostic guidelines for MAS complicating JIA: Clinical criteria
1) CNS dysfunction 2) Hemorrhages 3) Hepatosplenomegaly
34
Preliminary diagnostic guidelines for MAS complicating JIA: Histopath criterion
Macrophage hemophagocytosis in the BM aspirate
35
Preliminary diagnostic guidelines for MAS complicating JIA: Diagnostic rule
2 or more lab criteria OR ≥2 clinical and/or lab criteria
36
T/F JIA is a clinical diagnosis of exclusion
T
37
Associated with increased risk of chronic uveitis in JIA
(+) ANA
38
Treatment for JIA
NSAIDs, MTX, TNF-α antagonist
39
JIA Type, Types of Joints: Medium to large
Oligo
40
JIA Type, Types of Joints: Small to medium
Poly, systemic
41
JIA Type, Eye disease (uveitis): +++
Oligo
42
JIA Type, Eye disease (uveitis): ++
Poly
43
JIA Type, Eye disease (uveitis): +
Systemic
44
JIA Type: ANA ++
Oligo
45
JIA Type: ANA +
Poly
46
JIA Type: ANA -
Systemic
47
JIA Type: RF (+)
Poly
48
JIA Type, Outcomes: Excellent, >90% complete remission
Oligo
49
JIA Type, Outcomes: Good, >50% complete remission
Poly
50
JIA Type, Outcomes: Variable, depends on extent of arthritis
Systemic