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clin med > pediatric rheumatology > Flashcards

pediatric rheumatology Flashcards

1
Q

7 rheumatic diseases that present differently when in kids

A 
Juvenile Idiopathic Arthritis
Juvenile-onset Spondyloarthritis
Connective tissue diseases
Hypermobility syndrome
Growing pains
Kawasaki disease
Henoch-Schonlein Purpura (HSP)
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2
Q

who gets juvenile idiopathic arthritis most?

A

girls age 1-6yo (but can occur at any age)

after 16yo its considered adult arthritis

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3
Q

pathophys of JIA?

A

Probably similar immune phenomenon as adult RA but different HLA gene associations

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4
Q

Juvenile idiopathic arthritis can present what 3 different ways?

A

Systemic onset
Polyarticular onset
Pauciarticular onset

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5
Q

JIA: systemic onset (6 parts)

A
  • spike high fever
  • salmon-pink macular rash (koebner’s rash: develops where there is trauma)
  • pain in muscles/joints
  • enlarged spleen/liver
  • high WBCs
  • Polyserositis
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6
Q

what is polyserositis?

A

serous membranes inflamed pericarditis, pleuritis

occurs in systemic onset JIA

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7
Q

JIA: polyarticular onset (what is it + time period)

A

> 4 joints involved in first 6 months

  • Chronic pain and swelling in many symmetrical joints, large and small
  • Classically mandible involved, inflammation causes premature closure of growth plate and SHORTENED JAW
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8
Q

JIA: pauciarticular onset (what is it + time period)

A
  • Chronic arthritis of a few joints: <5 for 6 weeks
  • Large asymmetrical joint involvement
  • few if any systemic features ( except iridocyclitis in girls)
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9
Q

what is iridocyclitis?

A
  • inflammation in different parts of the eyes (iris + ciliary bodies)
  • asymptomatic but can cause blindness (if untreated)
  • iris isn’t round/symmetric
    (in pauciarticular JIA)
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10
Q

what % of kids with pauciarticular JIA get iridocyclitis? does it correlate with severity of arthritis?

A

30%

does NOT correlate with severity of arthritis

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11
Q

if someone has iridocyclitis, what do they need?

A

opthamology consult! slit lamp exam every 3-6 months

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12
Q

3 goals of txt for JIA

A

Restore function
Relieve pain
Maintain joint motion

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13
Q

JIA txt: nonpharm

A

PT/OT/ orthopedic

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14
Q

JIA txt: pharmacological

A

NSAIDS

DMARDs (less likely to use)

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15
Q

what are the liquid NSAIDs available ( for young kids) ?

A

Naprosyn, ibuprofen, diclofenac

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16
Q

DMARDs for JIA (3)

A

Reluctant to use unless have to because of unknowns about long term effects

  1. Hydroxychloroquine
  2. Methotrexate- also helps the eye (the only one)
  3. TNF inhibitors
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17
Q

prognosis of JIA? __ % get better with age

A

85%

18
Q

juvenile onset spondyloarthropathies: how are they like adult? how are they different:

A

Like adult: a group of HLA-B27 associated disorders

Unlike the adult form: in which inflammatory low back pain is the predominant clinical symptom, the juvenile form has more PERIPHERAL ENTHESITIS and ARTHRITIS (predominantly of the lower extremities) as its main clinical features

19
Q

connective tissue diseases in children: SLE, dermatomyositis, scleroderma

A

SLE – just like the adult counterpart
Dermatomyositis – similar to adults with an occasional case of calcinosis; polymyositis is very rare
Scleroderma – typically the “Localized” subtype; linear scleroderma (most commonly affects the legs in children)

20
Q

hypermobility syndrome: what is it? boys or girls more?

A

joint able to move beyond normal limits.
mostly in girls
(Consider when presented with joint pain, no inflammation, but increased range of motion)

21
Q

txt for hypermobility syndrome

A

Build up muscles around joints with physical therapy

NSAIDS

22
Q

what are Benign episodic bilateral limb pains?

A

growing pains (4-12 yo kids)

23
Q

classic presentation of growing pains

A

long bones (anterior thigh, knee, calf)
bilateral, symmetric
comes and goes (usually later evening/night)

24
Q

what is kawasaki dz?

A

Acute self-limited necrotizing vasculitis of small to medium vessels
Immune system fails to recognize self and develop inflammation of vessel walls
Leads to aneurysms that can rupture or inflammation can fill lumen and cause blockage with no blood flow to all distal tissues

25
Q

Acute self-limited necrotizing vasculitis of small to medium vessels

A

kawasaki dz

26
Q

who gets kawasaki Dz?

A

80% of cases are kids <4yo

27
Q

what triggers kawasaki Dz?

A

possibly virus (b/c it occurs in seasons)

28
Q

presentation of kawasaki Dz?

A

kinda non-specific: high fever, enlarged cervical lymph nodes, skin rash, arthralgia

29
Q

what is the skin rash like with kawasaki Dz?

A

Polymorphous exanthem, redness and swelling of hands and feet with desquamation (probably the most specific of the presenting symptoms)

30
Q

PE of kawasaki (4)

A

Conjunctivitis
Skin rash
Strawberry tongue, erythema of lips ( Inflammation of oral mucosa)
S3, S4, CHF, pericarditis

31
Q

while the Dx of kawasaki is clinical, what test should be done?

A

evaluate heart for cardiac complications with echo and angiography

32
Q

txt for kawasaki ? (why is early txt important?)

A

IVIG-intravenous immunoglobulin
Aspirin high doses
*early txt to prevent cardiac complications!

33
Q

what % of untreated kawasaki Dz get heart complications?

A

20-30%

can lead as far as myocarditis, pericarditis, MI, death

34
Q

what is Henoch-Schonlein Purpura?

A

small vessel Vasculitis affecting skin, GI, and kidney

Self-limiting

35
Q

what is the most common vasculitis to occur in children?

A

henoch-schonlein purpura

36
Q

who usually gets Henoch-schonlein purpura?

A

boys, usually after viral infection

37
Q

pathophys of henoch-schonlein purpura

A

IgA deposition in small blood vessels of skin (leukocytoclastic vasculitis), & sometimes GI tract & kidney

38
Q

4 clinical manifestations of henoch-schonlein purpura

A
  1. Palpable purpura- purple raised rash
  2. Arthritis/arthralgias (knee & ankle)
  3. Abdominal pain (GI bleed in 25%)
  4. hematuria, proteinuria (renal disease)
39
Q

Dx of henoch-schonlein purpura ?

A

Nonspecific tests of inflammation are present: Elevated ESR, WBC, mild anemia

  • usually made clinically
  • if VERY sick, you can bx the kidney for Dx confirmation
40
Q

what are the supportive and symptomatic txts for henoch-schonlein purpura?

A

Supportive – rest, hydrate, and pain control

Symptomatic – NSAIDs, acetaminophen for abdominal pain and joint pain

41
Q

prognosis of henoch-schonlein purpura

A

GREAT!

Get better in 1-6 weeks. Complications are uncommon

clin med (88 decks)

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