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Pedia > Vasculitides, Misc + CDB > Flashcards

Vasculitides, Misc + CDB Flashcards

1
Q

MC vasculitis in children

A

HSP

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2
Q

Immune-mediated vasculitis that affects the GI tract, joints, and kidneys and causes a characteristic rash

A

HSP

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3
Q

Size of vessels involved in HSP

A

Small

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4
Q

HSP commonly follows a

A

URI caused by group A strep or a virus

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5
Q

Deposited in small vessels of skin and glomeruli of patients with HSP

A

IgA and C3

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6
Q

Hallmark of HSP

A

Palpable purpura

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7
Q

Seen in biopsy of HSP

A

Leukoclastic angiitis

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8
Q

Henoch-Schonlein Purpura is associated with what GI complication?

A

Intussusception

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9
Q

Old rotavirus vaccine causes this GI problem

A

Intussusception

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10
Q

Most common cause of nonthrombocytopenic purpura in children

A

HSP

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11
Q

T/F HSP is usually benign and self-limited

A

T

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12
Q

2nd MC vasculitis in children

A

Kawasaki disease

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13
Q

What are the other names for kawasaki disease

A

Mucocutaneous lymph node syndrome

Infantile polyartritis nodosa

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14
Q

Criteria for Kawasaki Disease

A

Fever of greater than or equal to 5 days plus at least 4 of the following (first 4 are the PRINCIPAL criteria):

1) Non-suppurative bilateral conjunctivitis
2) Mucocutaneous lesions (strawberry tongue, dry red cracked lips, diffuse erythema of oral cavity)
3) Induration of palms and soles
4) Unilateral non-suppurative cervical LAD > 1.5 cm
5) Polymorphic truncal rash

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15
Q

Management for Kawasaki disease

A

IVIg
High-dose aspirin (↓ high platelets)
To be given within 10 days of disease onset

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16
Q

ASA therapy can cause what syndrome

A

Reye syndrome

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17
Q

MC cardiac complication of Kawasaki disease

A

Coronary artery aneurysm

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18
Q

Kawasaki: Characteristic desquamation

A

Glove and stocking (periungual) desquamation of fingers and toes

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19
Q

Goals in the treatment of Kawasaki

A

1) Acute phase: Decrease inflammation of coronary artery and myocardium
2) After acute phase: Prevent thrombosis and platelet aggregation

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20
Q

Etiology of Kawasaki disease

A

Unknown

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21
Q

T/F Kawasaki: Affects children (> 80% under age 4 years)

A

T

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22
Q

T/F Kawasaki: More common in Asians than other racial groups

A

T

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23
Q

T/F Kawasaki: More common in females than males (ratio 1.5:1)

A

F, males

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24
Q

Vasculitis: 􏱄 Sterile pyuria
􏱄 Aseptic meningitis
􏱄 Thrombocytosis
􏱄 Desquamation of fingers and toes

A

Kawasaki

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25
Q

Most significant sequelae of Kawasaki

A

􏱄 Coronary aneurysms (usually resolve within 12 months of adequate therapy)
􏱄 Pericardial effusion
􏱄 CHF

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26
Q

Kawasaki, initial study at diagnosis to establish baseline and to evaluate for early coronary aneurysms

A

2D echo

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27
Q

KD most commonly occurs in children younger than ___ of age, with a peak between ___, and is rare in children older than ___

A

5 years, 2 to 3 years, 7 years

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28
Q

KD predominantly affect the ___ arteries

A

Medium sized arteries particularly coronary arteries

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29
Q

5 day fever +

A

Coronary artery abnormalities

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30
Q

If there is more than 4 principal criteria of Kawasaki, you can diagnose KD in what day of illness

A

4th day of fever

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31
Q

Most important manifestation of Kawasaki

A

Aneurysmal involvement of coronary arteries, NOT myocarditis

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32
Q

Phases of Kawasaki

A
  • acute febrile phase - heralded by high fever w/o apparent source, lasts for 1-2 weeks
  • subacute phase - resolution of fever, desquamation, thrombocytosis, highest incidence of sudden death, last 2 weeks
  • convalescence phase - disappearance of sx, until ESR is normal ( 6-8 wks after onset of illness)
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33
Q

Kawasaki: Giant coronary artery aneurysms, which are rare but occur most commonly in very young children, can appear during this phase

A

Acute phase

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34
Q

Kawasaki: This phase heralds the onset of coronary artery aneurysms

A

Subacute phase

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35
Q

Poses the highest risk of morbidity and mortality in Kawasaki

A

Coronary artery aneurysm

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36
Q

Risk factors for development of coronary artery aneurysms in Kawasaki

A

Prolonged fever, prolonged elevation of inflammatory parameters (ESR), age younger than 1 year or older than 6 years, and male gender

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37
Q

Beau lines of the fingernails may appear during this phase

A

Convalescent

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38
Q

May be given to prevent Reye syndrome with ASA

A

Flu vaccine

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39
Q

The only illness for which it is okay to give aspirin to a child

A

Kawasaki

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40
Q

A necrotizing inflammation of the small and MEDIUM-sized muscular arteries with FIBRINOID necrosis, thrombosis, and infarction; gangrene of distal extremities is found in severe disease

A

Polyarteritis nodosa

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41
Q

Infection associated with polyarteritis nodosa

A

HBV infection

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42
Q

Vasculitis associated with p-ANCA

A

Polyarteritis nodosa

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43
Q

Polyarteritis nodosa, treatment

A

Corticosteroids

44
Q

Pulseless disease

A

Takayasu arteritis

45
Q

Vasculitis presenting with hypertension and abdominal pain

A

Polyarteritis nodosa

46
Q

Giant cell arteritis of the aorta (and large branches)

A

Takayasu

47
Q

Also known as aortoarteritis

A

Takayasu

48
Q

Takayasu: ___ is the predominantly affected site in the pediatric population

A

Thoracoabdominal aorta

49
Q

Takayasu, male vs female

A

Female

50
Q

Vasculitis with LV dysfunction and CHF

A

Takayasu

51
Q

age 3-10
hx of episodic pain occurring at night after physical activity in the daytime
relieved by rubbing
no limp

A

growing pains

52
Q

3- 10 yrs old
increase activity
hyperextensible joint

A

benign hypermobility syndrome

53
Q

gottron rash
heliotrope rash
erythematous rash on elbows and knees is pathognomonic of __

A

JDM

54
Q

what infection induces + ANA

A

parvovirus b19
EBV
endocarditis

55
Q

2 components of JIA pathogenesis

A

immunogenic susceptability

external trigger

56
Q

arthritis is defined as

A

intraarticular swelling + 2 of the ff

  • limitation of motion
  • tenderness on motion
  • heat or erythema
57
Q

is a rare but potentially fatal complication of SJIA with profound anemia, thrombocyteopenia, leukopenia
spiking fever with fall of ESR ( due to hypofibrinogeneia and hepatic dysfucntion

A

MAS ( macrophage activating syndrome) of HLH

– treated with methylpred high dose

58
Q

3-10 yrs
severe hip pain lasting for a week
referred pain to the thigh and knee
normal ESR and wbc

A

transient synovitis

59
Q

4 out of 11 of these criteria establishes diagnosis of SLE

A
  • malar rash
  • discoid rash
  • photosensitivity
  • oral ulcers
  • non erosive arthritis
  • serositis
  • renal manifestation
  • seizure/psychosis
  • hemolytic anemia
  • immunologic abnormalities
60
Q

what test correlates with disease activity in SLE

A

ds DNA
crp
hemolytic complement

61
Q

treatment of SLE

A

hydroxychloroquine ( 5-7mgkgday)

corticosteroid

62
Q

criteria for juvenile dermatomyositis diagnosis

A

classic rash + 3 of the ff

  • weakness
  • muscle enzyme elevation
  • mypathy/denervation
  • necrosis/inflammation in muscle biopsy
63
Q

triad of reynauds phenomenon induced by cold and emotional stress

A

blanching
cyanosis
erythema of the digits

64
Q

painful shallow ulcer, arthritis involving the large joints, eryhtema nodosum
treated with colchicine

A

behcets

65
Q

what is gelling phenomenon

A

stiffness after physical activity

66
Q

fatigue is a common presenting complaint of rheumatic disease

A

JUVENILE DERMATOMYOSITIS (JDM)

67
Q

difference of facial rash of jdm and SLE

A

in SLE the rash is malar that does not cross the nasolabial folds

68
Q

hyperkeratotic rash on the face and around the ears in african descent girl

A

discoid rash

69
Q

saddle deformity is seen in

A

wegeners granulomatosis

70
Q

oral ulcers are seen in what rheumatic disease

A

sle and behcet disease

71
Q

___ is more specific in SLE than leukopneia

A

lymphopenia

72
Q

what antibody is associated with neonatal lupus

A

Rho (SSA)

73
Q

pseudporphyria is characterized by hypopigmented depressed scars after trauma caused by what what NSAID

A

naproxen

74
Q

the cornerstone of therapy in pediatric rheumatology due to its sustained effectiveness and low toxicity

A

methotrexate

75
Q

it is the most common rheumatic disease in children

A

JIA

76
Q

what is the most common subtype juvenile idiopathic arthritis (JIA)

A

pauciarticular

77
Q

JIA is common at what ages

A

2-4 and 10-14 ( bimodal)

78
Q

what cell plays a major role JIA

A

t cell lymphocytes

79
Q

involves less that 4 joints within the first 6 months affecting large joints

A

oligoarthritis

80
Q

inflammation of more than 5 joint on both lower and upper extremities

A

polyarthritis

81
Q

for systemic JIA these lesions are classic and distributed on trunk and proximal extremities

A

salmon colored lesions

82
Q

cutaneous hypersensitivity to superficial trauma present in SJIA (systemic JIA)

A

kobner phenomenon

83
Q

ANA is elevated and useful in what conditon

A

chronic uveitis in JIA

84
Q

marked inflammation where tendons and ligaments attach to bone, a key feature of spondyloarthritidis

A

enthesis

85
Q

oligo arthritis, enthesis involving the hips

A

juvenile ankylosing spondylitis

86
Q

reiter syndrome triad

A

arthritis, uveitis, conjuctivitis

87
Q

for post infectious arthritis due to strep disease treatment ___

A

penicillin prophylaxis for at least 1 year

88
Q

what is the strongest risk factor of SLE

A

gender ( female)

89
Q

hyperkeratosis follicular plugging and infiltration of mononuclear cell into the dermal-epidermal junction

A

discoid rash

90
Q

deposition of immune complexes in the epidermal junction characteristic of sle

A

lupus band test

91
Q

what is the hallmark of SLE

A

multiorgan disease

92
Q

kind of SLE that is equal in both males and females

A

drug induced SLE

93
Q

acute common cause of death in SLE

A

infection and complication of GN

94
Q

long term, most common cause of death in SLE

A

complications of atherosclerosis and malignancy

95
Q

most feared complication of neonatal lupus

A

complete heart block

96
Q

most common inflammatory myositis in children

A

Juvenile dermatomyositis (JDM)

97
Q

child with rash on neck and chest photosensitivity, weakness, diffused facial rash, erythema on knees and elbow with bluish discoloration of the eyes

A

JDM

98
Q

erythema of the neck and trunk seen in JDM is called

A

shawl sign

99
Q

blue violet discoloration of the eyelids seen in JDM

A

heliotrope rash

100
Q

pink atrophic plaques in interphalangeal joint seen in JDM

A

gottron papules

101
Q

the use of hands to stand from a sitting position commonly seen in JDM

A

gower sign

102
Q

a variant of JDM with rash but no weakness or inflammation

A

amyopathic JDM

103
Q

methylpred is the treatment of choice for JDM, ___ is given to reduce toxicity of its inhibition

A

folic acid

104
Q

__ is the most common visceral manifestation of of Systemic sclerosis

A

pulmonary disease

105
Q

___ most frequent initial symptom in pediatric systemic sclerosis

A

raynauds phenomenon

106
Q

___ a condition of episodic color changes and the development of nodules in severe cold exposure seen in SLE

A

childblains

107
Q

the most common cause of death in scleroderma

A

heart failure due to myocardial and pulmonary fibrosis

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