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Flashcards in Vasculitides, Misc + CDB Deck (107):
1

MC vasculitis in children

HSP

2

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

HSP

3

Size of vessels involved in HSP

Small

4

HSP commonly follows a

URI caused by group A strep or a virus

5

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

IgA and C3

6

Hallmark of HSP

Palpable purpura

7

Seen in biopsy of HSP

Leukoclastic angiitis

8

Henoch-Schonlein Purpura is associated with what GI complication?

Intussusception

9

Old rotavirus vaccine causes this GI problem

Intussusception

10

Most common cause of nonthrombocytopenic purpura in children

HSP

11

T/F HSP is usually benign and self-limited

T

12

2nd MC vasculitis in children

Kawasaki disease

13

What are the other names for kawasaki disease

Mucocutaneous lymph node syndrome
Infantile polyartritis nodosa

14

Criteria for Kawasaki Disease

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

15

Management for Kawasaki disease

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

16

ASA therapy can cause what syndrome

Reye syndrome

17

MC cardiac complication of Kawasaki disease

Coronary artery aneurysm

18

Kawasaki: Characteristic desquamation

Glove and stocking (periungual) desquamation of fingers and toes

19

Goals in the treatment of Kawasaki

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

20

Etiology of Kawasaki disease

Unknown

21

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

T

22

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

T

23

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

F, males

24

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

Kawasaki

25

Most significant sequelae of Kawasaki

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

26

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

2D echo

27

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

5 years, 2 to 3 years, 7 years

28

KD predominantly affect the ___ arteries

Medium sized arteries particularly coronary arteries

29

5 day fever +

Coronary artery abnormalities

30

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

4th day of fever

31

Most important manifestation of Kawasaki

Aneurysmal involvement of coronary arteries, NOT myocarditis

32

Phases of Kawasaki

*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)

33

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

Acute phase

34

Kawasaki: This phase heralds the onset of coronary artery aneurysms

Subacute phase

35

Poses the highest risk of morbidity and mortality in Kawasaki

Coronary artery aneurysm

36

Risk factors for development of coronary artery aneurysms in Kawasaki

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

37

Beau lines of the fingernails may appear during this phase

Convalescent

38

May be given to prevent Reye syndrome with ASA

Flu vaccine

39

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

Kawasaki

40

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

Polyarteritis nodosa

41

Infection associated with polyarteritis nodosa

HBV infection

42

Vasculitis associated with p-ANCA

Polyarteritis nodosa

43

Polyarteritis nodosa, treatment

Corticosteroids

44

Pulseless disease

Takayasu arteritis

45

Vasculitis presenting with hypertension and abdominal pain

Polyarteritis nodosa

46

Giant cell arteritis of the aorta (and large branches)

Takayasu

47

Also known as aortoarteritis

Takayasu

48

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

Thoracoabdominal aorta

49

Takayasu, male vs female

Female

50

Vasculitis with LV dysfunction and CHF

Takayasu

51

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

growing pains

52

3- 10 yrs old
increase activity
hyperextensible joint

benign hypermobility syndrome

53

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

JDM

54

what infection induces + ANA

parvovirus b19
EBV
endocarditis

55

2 components of JIA pathogenesis

immunogenic susceptability
external trigger

56

arthritis is defined as

intraarticular swelling + 2 of the ff
- limitation of motion
- tenderness on motion
- heat or erythema

57

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

MAS ( macrophage activating syndrome) of HLH
-- treated with methylpred high dose

58

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

transient synovitis

59

4 out of 11 of these criteria establishes diagnosis of SLE

- malar rash
- discoid rash
- photosensitivity
- oral ulcers
- non erosive arthritis
- serositis
- renal manifestation
- seizure/psychosis
- hemolytic anemia
- immunologic abnormalities

60

what test correlates with disease activity in SLE

ds DNA
crp
hemolytic complement

61

treatment of SLE

hydroxychloroquine ( 5-7mgkgday)
corticosteroid

62

criteria for juvenile dermatomyositis diagnosis

classic rash + 3 of the ff
- weakness
- muscle enzyme elevation
- mypathy/denervation
- necrosis/inflammation in muscle biopsy

63

triad of reynauds phenomenon induced by cold and emotional stress

blanching
cyanosis
erythema of the digits

64

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

behcets

65

what is gelling phenomenon

stiffness after physical activity

66

fatigue is a common presenting complaint of rheumatic disease

JUVENILE DERMATOMYOSITIS (JDM)

67

difference of facial rash of jdm and SLE

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

68

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

discoid rash

69

saddle deformity is seen in

wegeners granulomatosis

70

oral ulcers are seen in what rheumatic disease

sle and behcet disease

71

___ is more specific in SLE than leukopneia

lymphopenia

72

what antibody is associated with neonatal lupus

Rho (SSA)

73

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

naproxen

74

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

methotrexate

75

it is the most common rheumatic disease in children

JIA

76

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

pauciarticular

77

JIA is common at what ages

2-4 and 10-14 ( bimodal)

78

what cell plays a major role JIA

t cell lymphocytes

79

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

oligoarthritis

80

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

polyarthritis

81

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

salmon colored lesions

82

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

kobner phenomenon

83

ANA is elevated and useful in what conditon

chronic uveitis in JIA

84

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

enthesis

85

oligo arthritis, enthesis involving the hips

juvenile ankylosing spondylitis

86

reiter syndrome triad

arthritis, uveitis, conjuctivitis

87

for post infectious arthritis due to strep disease treatment ___

penicillin prophylaxis for at least 1 year

88

what is the strongest risk factor of SLE

gender ( female)

89

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

discoid rash

90

deposition of immune complexes in the epidermal junction characteristic of sle

lupus band test

91

what is the hallmark of SLE

multiorgan disease

92

kind of SLE that is equal in both males and females

drug induced SLE

93

acute common cause of death in SLE

infection and complication of GN

94

long term, most common cause of death in SLE

complications of atherosclerosis and malignancy

95

most feared complication of neonatal lupus

complete heart block

96

most common inflammatory myositis in children

Juvenile dermatomyositis (JDM)

97

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

JDM

98

erythema of the neck and trunk seen in JDM is called

shawl sign

99

blue violet discoloration of the eyelids seen in JDM

heliotrope rash

100

pink atrophic plaques in interphalangeal joint seen in JDM

gottron papules

101

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

gower sign

102

a variant of JDM with rash but no weakness or inflammation

amyopathic JDM

103

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

folic acid

104

__ is the most common visceral manifestation of of Systemic sclerosis

pulmonary disease

105

___ most frequent initial symptom in pediatric systemic sclerosis

raynauds phenomenon

106

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

childblains

107

the most common cause of death in scleroderma

heart failure due to myocardial and pulmonary fibrosis