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Rheumatology Exam > Juvenile Idiopathic Arthritis > Flashcards

Juvenile Idiopathic Arthritis Flashcards

1
Q

ddx for joint pain in children

A
  1. trauma
  2. biomechanical
  3. infection
    -septic joint/osteomyelitis
    -lyme disease
    -gonococcus
    -viral arthritis
  4. reactive
    -enteric (shigella, salmonella, campylobacter)
    -acute rheumatic fever
  5. malignancy
    -leukemia
    -bone tumors (malignant & benign)
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2
Q

classic presentation of juvenile idiopathic arthritis

A

*3 year old female presents with history of left knee pain and swelling
*no history of recent illness or trauma
*exam: painful, warm left knee with an effusion and limited range of motion
*no fever or rash
*unremarkable CBC and ESR, Lyme negative
*ANA positive

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3
Q

joint pain in children - red flags (need immediate evaluation)

A

*fever, systemic upset (malaise, weight loss, night sweats)
*lymphadenopathy, hepatosplenomegaly
*bone pain
*persistent night waking
*incongruence between history and exam

summary - do NOT miss infection, malignancy, NAT

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4
Q

ddx for joint pain in kids: Lyme arthritis

A

*lyme arthritis is a late finding
*usually follows erythema migrans rash earlier
*more common in older kids with 1 knee affected

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5
Q

ddx for joint pain in kids: acute rheumatic fever

A

*Jones criteria
*supporting evidence of antecedent GAS infection (elevated or rising strep antibody titers, positive throat culture or rapid strep antigen)
*major criteria: carditis, polyarthritis (usually migratory), chorea/CNS disease, erythema marginatum, subcutaneous nodules

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6
Q

ddx for joint pain in kids: biomechanical causes

A

*benign nocturnal MSK pains of childhood (growing pains)
*joint hypermobility
*overuse syndromes
*apophysitis

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7
Q

Beighton Hypermobility Criteria (after age 6)

A
  1. touch thumb to forearm
  2. hyperextend MCPs (parallel forearm)
  3. > 10 degree hyperextension knees
  4. touch pals to floor (straight knees)
  5. excessive hip rotation
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8
Q

hypermobility syndrome

A

*behind the knee pain
*evening pain, especially bedtime
*worse after activities
*better with rubbing
*tx: NSAIDs prior to onset of pain, PT
*if concerned for connective tissue disorder (EDS, Marfan’s), refer to genetics

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9
Q

ddx for joint pain in kids: rheumatologic ddx

A

*SLE
*juvenile dermatomyositis
*mixed connective tissue disease
*vasculitis (IgA vasculitis, Kawasaki)
*periodic fever syndromes
*Juvenile idiopathic arthritis

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10
Q

arthritis vs arthralgia - defined

A

ARTHRITIS:
*effusion (swelling) or 2+ of the following:
-limited range of motion
-pain with motion
-warmth
-tenderness

ARTHRALGIA: joint pain without objective evidence of inflammation

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11
Q

juvenile idiopathic arthritis (JIA) - epidemiology

A

*most common chronic rheumatologic condition of childhood
*affects 1 to 4 per 1000 children

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12
Q

juvenile idiopathic arthritis (JIA) - overview

A

*heterogenous group of diseases characterized by arthritis that:
-begins before age 16
-involves 1+ joint
-persists 6+ weeks
*complications of poorly controlled JIA include growth disturbances, joint contractures and destruction, and chronic pain

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13
Q

juvenile idiopathic arthritis (JIA) - diagnosis

A

*rule out other causes (diagnosis of exclusion)
*no labs are diagnostic, but can be useful for supporting dx, prognosis, monitoring therapy toxicity
*6 months to determine subtype

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14
Q

ANA in JIA

A

*antibodies against proteins in the nucleus
*no diagnostic utility in JIA
*helpful in evaluation risk of uveitis in JIA (positive ANA = increased risk of developing uveitis)

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15
Q

RF & CCP in JIA

A

*in pts with polyarticular JIA, RF+ and CCP+ can have more aggressive disease
*not diagnostic of JIA

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16
Q

HLA B27 in JIA

A

*associated with spondyloarthritis, IBD, psoriatic arthritis, and reactive arthritis
*up to 10% of general population is positive
*not diagnostic of JIA

17
Q

7 categories of JIA

A

*younger kids:
-oligoarticular
-polyarticular, RF negative
-polyarticular, RF positive

*spondyloarthropathies:
-psoriatic
-enthesitis related
-undifferentiated

*systemic JIA

18
Q

oligoarticular JIA

A

*1-3 year old girl, ANA+, arthritic joint (knee > ankle > fingers)
*4 or fewer arthritic joints within first 6 months of diagnosis
*next step = REFER TO OPHTHAMOLOGY (due to chance of iritis)

19
Q

polyarticular, RF negative JIA

A

*more than 5 arthritic joints within first 6 months of diagnosis
*RF negative
*biphasic trend: 1-3 yo and adolescence
*TMJ disease
*number of affected joints tend to be less than in RF+

20
Q

polyarticular, RF positive JIA

A

*more than 5 arthritic joints within first 6 months of diagnosis
*RF positive
*teenage females
*upper and lower extremity, large and small joints involved
*similar to adult RA
*CCP positive

21
Q

enthesitis-related arthritis

A

*arthritis and enthesitis
OR
*arthritis plus 2 of the following:
-presence or history of inflammatory SI tenderness and/or inflammatory lumbosacral pain
-presence of HLA B27 antigen
-onset of arthritis in a male over age 6
-history of ankylosing spondylitis, ERA, sacroiliitis, with IBD, Reiter’s syndrome, or acute anterior uveitis in first-degree relative

22
Q

systemic juvenile idiopathic arthritis (JIA) - epidemiology

A

*frequency: 10% of JIA
*occurs equally in males and females
*peak age: 1-5 years old
*arthritis (usually polyarticular)
*fever with daily high spikes for a minimum of 2 weeks

23
Q

systemic juvenile idiopathic arthritis (JIA) - diagnostic criteria

A
  1. quotidian fever (occurs daily at intervals) of at least 2 weeks duration
    PLUS
  2. arthritis in any number of joints
    AND at least one of the following:
    a. evanescent rash (faint, salmon-colored, nonpruritic, macular, transient; Koebner phenomenon)
    b. generalized lymphadenopathy
    c. enlarged liver or spleen
    d. serositis
24
Q

systemic juvenile idiopathic arthritis (JIA) - labs

A

*elevated WBC
*anemia
*thrombocytosis
*elevated ESR and CRP
*mildly elevated LFTs
*elevated ferritin

25
Q

macrophage activation syndrome - overview

A

*complication of systemic JIA (also seen in lupus, Kawasaki, MIS-C)
*uncontrollable activation and expansion of T cells and macrophages leading to overwhelming systemic inflammatory response
*febrile pt with known or suspected systemic JIA with high ferritin and low platelets

26
Q

macrophage activation syndrome - bone marrow findings

A

*systemic “cytokine storm:”
-cytopenias
-liver dysfunction
-coagulopathy
-extreme hyperferritinemia

27
Q

macrophage activation syndrome - treatment

A

*methylprednisone
*anakinra
*canakinumab
*tocilizumab
*cyclosporine

28
Q

uveitis in JIA

A

*chronic anterior non-granulomatous inflammation affecting the iris and ciliary body

29
Q

complications of JIA

A

*uveitis
*synechiae (asymptomatic iritis)
*TMJ disease
*joint contracture
*limb length discrepancy

Rheumatology Exam (20 decks)

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