Ch. 155 Juvenile Idiopathic Arthritis

Question 1

MC rheumatic disease in children

Answer 1

JIA (JRA accdg to ACR)

Question 2

MC subtype of JIA in order

Answer 2

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

Question 3

Gender predominance in sJIA

Answer 3

None

Question 4

Gender predominance in oligo- and polyarticular JIA

Answer 4

F>M

Question 5

Peak of onset of oligoarthritis

Answer 5

2-4 y/o

Question 6

Peak of onset of polyarthritis

Answer 6

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

Question 7

Peak of onset of sJIA

Answer 7

1-5 y/o

Question 8

Non-genetic triggers for JIA

Answer 8

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

Question 9

Components of the immune system affected in JIA

Answer 9

Both humoral and cell-mediated immunity

Question 10

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

Answer 10

T lymphocytes

Question 11

Inflammatory synovitis in JIA is characterized pathologically by

Answer 11

Villous hypertrophy and hyperplasia with hyperemia and edema of synovial tissue

Question 12

Advanced and uncontrolled JIA leads to

Answer 12

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

Question 13

Must be present to make a diagnosis of any JIA subtype

Answer 13

Arthritis

Question 14

Criteria for classification of JRA

Answer 14

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

Question 15

Arthritis of JRA is defined as

Answer 15

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

Question 16

Polyarthritis is defined as

Answer 16

≥5 inflamed joints

Question 17

Oligoarthritis is defined as

Answer 17

≤4 inflamed joints

Question 18

Arthritis + rash + characteristic quotidian fever

Answer 18

Systemic JIA

Question 19

ILAE Classification of JIA: Systemic onset JIA

Answer 19

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

Question 20

ILAE Classification of JIA: Oligoarticular JIA

Answer 20

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

Question 21

ILAE Classification of JIA: Polyarthritis RF negative

Answer 21

≥5 joints during 1st 6 months, RF negative

Question 22

ILAE Classification of JIA: Polyarthritis RF positive

Answer 22

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

Question 23

Spondyloarthropathies: Psoriatic arthritis, criteria

Answer 23

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

Question 24

Spondyloarthropathies: Enthesitis-related arthritis, criteria

Answer 24

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

Question 25

HLA-B27 positive

Answer 25

Enthesitis-related arthritis

Question 26

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

Answer 26

T, suggests a spondyloarthropathy or a nonrheumatologic cause

Question 27

Confers increased risk for asymptomatic anterior uveitis

Answer 27

(+) ANA

Question 28

PE finding that reflects chronic temporomandibbular joint disease

Answer 28

Micrognathia

Question 29

Rash of systemic JIA

Answer 29

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

Question 30

Rare but potentially fatal complication of SoJIA

Answer 30

MAS

Question 31

Clinical manifestations of SoJIA

Answer 31

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

Question 32

Preliminary diagnostic guidelines for MAS complicating JIA: Lab criteria

Answer 32

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

Question 33

Preliminary diagnostic guidelines for MAS complicating JIA: Clinical criteria

Answer 33

1) CNS dysfunction 2) Hemorrhages 3) Hepatosplenomegaly

Question 34

Preliminary diagnostic guidelines for MAS complicating JIA: Histopath criterion

Answer 34

Macrophage hemophagocytosis in the BM aspirate

Question 35

Preliminary diagnostic guidelines for MAS complicating JIA: Diagnostic rule

Answer 35

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

Question 36

T/F JIA is a clinical diagnosis of exclusion

Answer 36

T

Question 37

Associated with increased risk of chronic uveitis in JIA

Answer 37

(+) ANA

Question 38

Treatment for JIA

Answer 38

NSAIDs, MTX, TNF-α antagonist

Question 39

JIA Type, Types of Joints: Medium to large

Answer 39

Oligo

Question 40

JIA Type, Types of Joints: Small to medium

Answer 40

Poly, systemic

Question 41

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

Answer 41

Oligo

Question 42

JIA Type, Eye disease (uveitis): ++

Answer 42

Poly

Question 43

JIA Type, Eye disease (uveitis): +

Answer 43

Systemic

Question 44

JIA Type: ANA ++

Answer 44

Oligo

Question 45

JIA Type: ANA +

Answer 45

Poly

Question 46

JIA Type: ANA -

Answer 46

Systemic

Question 47

JIA Type: RF (+)

Answer 47

Poly

Question 48

JIA Type, Outcomes: Excellent, >90% complete remission

Answer 48

Oligo

Question 49

JIA Type, Outcomes: Good, >50% complete remission

Answer 49

Poly

Question 50

JIA Type, Outcomes: Variable, depends on extent of arthritis

Answer 50

Systemic