Vasculitides, Misc + CDB Flashcards
1
Q
MC vasculitis in children
A
HSP
2
Q
Immune-mediated vasculitis that affects the GI tract, joints, and kidneys and causes a characteristic rash
A
HSP
3
Q
Size of vessels involved in HSP
A
Small
4
Q
HSP commonly follows a
A
URI caused by group A strep or a virus
5
Q
Deposited in small vessels of skin and glomeruli of patients with HSP
A
IgA and C3
6
Q
Hallmark of HSP
A
Palpable purpura
7
Q
Seen in biopsy of HSP
A
Leukoclastic angiitis
8
Q
Henoch-Schonlein Purpura is associated with what GI complication?
A
Intussusception
9
Q
Old rotavirus vaccine causes this GI problem
A
Intussusception
10
Q
Most common cause of nonthrombocytopenic purpura in children
A
HSP
11
Q
T/F HSP is usually benign and self-limited
A
T
12
Q
2nd MC vasculitis in children
A
Kawasaki disease
13
Q
What are the other names for kawasaki disease
A
Mucocutaneous lymph node syndrome
Infantile polyartritis nodosa
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
15
Q
Management for Kawasaki disease
A
IVIg
High-dose aspirin (↓ high platelets)
To be given within 10 days of disease onset
16
Q
ASA therapy can cause what syndrome
A
Reye syndrome
17
Q
MC cardiac complication of Kawasaki disease
A
Coronary artery aneurysm
18
Q
Kawasaki: Characteristic desquamation
A
Glove and stocking (periungual) desquamation of fingers and toes
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
20
Q
Etiology of Kawasaki disease
A
Unknown
21
Q
T/F Kawasaki: Affects children (> 80% under age 4 years)
A
T
22
Q
T/F Kawasaki: More common in Asians than other racial groups
A
T
23
Q
T/F Kawasaki: More common in females than males (ratio 1.5:1)
A
F, males
24
Q
Vasculitis: Sterile pyuria
Aseptic meningitis
Thrombocytosis
Desquamation of fingers and toes
A
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
