Ch. 155 Juvenile Idiopathic Arthritis Flashcards Preview

Nelson - Rheumatic Diseases of Childhood > Ch. 155 Juvenile Idiopathic Arthritis > Flashcards

Flashcards in Ch. 155 Juvenile Idiopathic Arthritis Deck (50):
1

MC rheumatic disease in children

JIA (JRA accdg to ACR)

2

MC subtype of JIA in order

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

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Gender predominance in sJIA

None

4

Gender predominance in oligo- and polyarticular JIA

F>M

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Peak of onset of oligoarthritis

2-4 y/o

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Peak of onset of polyarthritis

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

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Peak of onset of sJIA

1-5 y/o

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Non-genetic triggers for JIA

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

9

Components of the immune system affected in JIA

Both humoral and cell-mediated immunity

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

T lymphocytes

11

Inflammatory synovitis in JIA is characterized pathologically by

Villous hypertrophy and hyperplasia with hyperemia and edema of synovial tissue

12

Advanced and uncontrolled JIA leads to

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

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Must be present to make a diagnosis of any JIA subtype

Arthritis

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Criteria for classification of JRA

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

15

Arthritis of JRA is defined as

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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Polyarthritis is defined as

≥5 inflamed joints

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Oligoarthritis is defined as

≤4 inflamed joints

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Arthritis + rash + characteristic quotidian fever

Systemic JIA

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ILAE Classification of JIA: Systemic onset JIA

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

20

ILAE Classification of JIA: Oligoarticular JIA

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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ILAE Classification of JIA: Polyarthritis RF negative

≥5 joints during 1st 6 months, RF negative

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ILAE Classification of JIA: Polyarthritis RF positive

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

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Spondyloarthropathies: Psoriatic arthritis, criteria

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

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Spondyloarthropathies: Enthesitis-related arthritis, criteria

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

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HLA-B27 positive

Enthesitis-related arthritis

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T/F In JIA, involvement of the hip is almost never a presenting sign

T, suggests a spondyloarthropathy or a nonrheumatologic cause

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Confers increased risk for asymptomatic anterior uveitis

(+) ANA

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PE finding that reflects chronic temporomandibbular joint disease

Micrognathia

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Rash of systemic JIA

Evanescent, salmon-colored, linear or circular, most common over trunk and proximal extremities

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Rare but potentially fatal complication of SoJIA

MAS

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Clinical manifestations of SoJIA

Acute profound anemia, thrombocytopenia, leukopenia, high spiking fevers, lymphadenopathy, hepatosplenomegaly

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Preliminary diagnostic guidelines for MAS complicating JIA: Lab criteria

1) Decreased platelet count 2) Elevated AST 3) Decreased WBC 4) Hypofibrinogenemia

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Preliminary diagnostic guidelines for MAS complicating JIA: Clinical criteria

1) CNS dysfunction 2) Hemorrhages 3) Hepatosplenomegaly

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Preliminary diagnostic guidelines for MAS complicating JIA: Histopath criterion

Macrophage hemophagocytosis in the BM aspirate

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Preliminary diagnostic guidelines for MAS complicating JIA: Diagnostic rule

2 or more lab criteria OR ≥2 clinical and/or lab criteria

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T/F JIA is a clinical diagnosis of exclusion

T

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Associated with increased risk of chronic uveitis in JIA

(+) ANA

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Treatment for JIA

NSAIDs, MTX, TNF-α antagonist

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JIA Type, Types of Joints: Medium to large

Oligo

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JIA Type, Types of Joints: Small to medium

Poly, systemic

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JIA Type, Eye disease (uveitis): +++

Oligo

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JIA Type, Eye disease (uveitis): ++

Poly

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JIA Type, Eye disease (uveitis): +

Systemic

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JIA Type: ANA ++

Oligo

45

JIA Type: ANA +

Poly

46

JIA Type: ANA -

Systemic

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JIA Type: RF (+)

Poly

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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