Vasculitides, Misc + CDB Flashcards
MC vasculitis in children
HSP
Immune-mediated vasculitis that affects the GI tract, joints, and kidneys and causes a characteristic rash
HSP
Size of vessels involved in HSP
Small
HSP commonly follows a
URI caused by group A strep or a virus
Deposited in small vessels of skin and glomeruli of patients with HSP
IgA and C3
Hallmark of HSP
Palpable purpura
Seen in biopsy of HSP
Leukoclastic angiitis
Henoch-Schonlein Purpura is associated with what GI complication?
Intussusception
Old rotavirus vaccine causes this GI problem
Intussusception
Most common cause of nonthrombocytopenic purpura in children
HSP
T/F HSP is usually benign and self-limited
T
2nd MC vasculitis in children
Kawasaki disease
What are the other names for kawasaki disease
Mucocutaneous lymph node syndrome
Infantile polyartritis nodosa
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
Management for Kawasaki disease
IVIg
High-dose aspirin (↓ high platelets)
To be given within 10 days of disease onset
ASA therapy can cause what syndrome
Reye syndrome
MC cardiac complication of Kawasaki disease
Coronary artery aneurysm
Kawasaki: Characteristic desquamation
Glove and stocking (periungual) desquamation of fingers and toes
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
Etiology of Kawasaki disease
Unknown
T/F Kawasaki: Affects children (> 80% under age 4 years)
T
T/F Kawasaki: More common in Asians than other racial groups
T
T/F Kawasaki: More common in females than males (ratio 1.5:1)
F, males
Vasculitis: Sterile pyuria
Aseptic meningitis
Thrombocytosis
Desquamation of fingers and toes
Kawasaki
Most significant sequelae of Kawasaki
Coronary aneurysms (usually resolve within 12 months of adequate therapy)
Pericardial effusion
CHF
Kawasaki, initial study at diagnosis to establish baseline and to evaluate for early coronary aneurysms
2D echo
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
KD predominantly affect the ___ arteries
Medium sized arteries particularly coronary arteries
5 day fever +
Coronary artery abnormalities
If there is more than 4 principal criteria of Kawasaki, you can diagnose KD in what day of illness
4th day of fever
Most important manifestation of Kawasaki
Aneurysmal involvement of coronary arteries, NOT myocarditis
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)
Kawasaki: Giant coronary artery aneurysms, which are rare but occur most commonly in very young children, can appear during this phase
Acute phase
Kawasaki: This phase heralds the onset of coronary artery aneurysms
Subacute phase
Poses the highest risk of morbidity and mortality in Kawasaki
Coronary artery aneurysm
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
Beau lines of the fingernails may appear during this phase
Convalescent
May be given to prevent Reye syndrome with ASA
Flu vaccine
The only illness for which it is okay to give aspirin to a child
Kawasaki
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
Infection associated with polyarteritis nodosa
HBV infection
Vasculitis associated with p-ANCA
Polyarteritis nodosa
Polyarteritis nodosa, treatment
Corticosteroids
Pulseless disease
Takayasu arteritis
Vasculitis presenting with hypertension and abdominal pain
Polyarteritis nodosa
Giant cell arteritis of the aorta (and large branches)
Takayasu
Also known as aortoarteritis
Takayasu
Takayasu: ___ is the predominantly affected site in the pediatric population
Thoracoabdominal aorta
Takayasu, male vs female
Female
Vasculitis with LV dysfunction and CHF
Takayasu
age 3-10
hx of episodic pain occurring at night after physical activity in the daytime
relieved by rubbing
no limp
growing pains
3- 10 yrs old
increase activity
hyperextensible joint
benign hypermobility syndrome
gottron rash
heliotrope rash
erythematous rash on elbows and knees is pathognomonic of __
JDM
what infection induces + ANA
parvovirus b19
EBV
endocarditis
2 components of JIA pathogenesis
immunogenic susceptability
external trigger
arthritis is defined as
intraarticular swelling + 2 of the ff
- limitation of motion
- tenderness on motion
- heat or erythema
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
3-10 yrs
severe hip pain lasting for a week
referred pain to the thigh and knee
normal ESR and wbc
transient synovitis
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
what test correlates with disease activity in SLE
ds DNA
crp
hemolytic complement
treatment of SLE
hydroxychloroquine ( 5-7mgkgday)
corticosteroid
criteria for juvenile dermatomyositis diagnosis
classic rash + 3 of the ff
- weakness
- muscle enzyme elevation
- mypathy/denervation
- necrosis/inflammation in muscle biopsy
triad of reynauds phenomenon induced by cold and emotional stress
blanching
cyanosis
erythema of the digits
painful shallow ulcer, arthritis involving the large joints, eryhtema nodosum
treated with colchicine
behcets
what is gelling phenomenon
stiffness after physical activity
fatigue is a common presenting complaint of rheumatic disease
JUVENILE DERMATOMYOSITIS (JDM)
difference of facial rash of jdm and SLE
in SLE the rash is malar that does not cross the nasolabial folds
hyperkeratotic rash on the face and around the ears in african descent girl
discoid rash
saddle deformity is seen in
wegeners granulomatosis
oral ulcers are seen in what rheumatic disease
sle and behcet disease
___ is more specific in SLE than leukopneia
lymphopenia
what antibody is associated with neonatal lupus
Rho (SSA)
pseudporphyria is characterized by hypopigmented depressed scars after trauma caused by what what NSAID
naproxen
the cornerstone of therapy in pediatric rheumatology due to its sustained effectiveness and low toxicity
methotrexate
it is the most common rheumatic disease in children
JIA
what is the most common subtype juvenile idiopathic arthritis (JIA)
pauciarticular
JIA is common at what ages
2-4 and 10-14 ( bimodal)
what cell plays a major role JIA
t cell lymphocytes
involves less that 4 joints within the first 6 months affecting large joints
oligoarthritis
inflammation of more than 5 joint on both lower and upper extremities
polyarthritis
for systemic JIA these lesions are classic and distributed on trunk and proximal extremities
salmon colored lesions
cutaneous hypersensitivity to superficial trauma present in SJIA (systemic JIA)
kobner phenomenon
ANA is elevated and useful in what conditon
chronic uveitis in JIA
marked inflammation where tendons and ligaments attach to bone, a key feature of spondyloarthritidis
enthesis
oligo arthritis, enthesis involving the hips
juvenile ankylosing spondylitis
reiter syndrome triad
arthritis, uveitis, conjuctivitis
for post infectious arthritis due to strep disease treatment ___
penicillin prophylaxis for at least 1 year
what is the strongest risk factor of SLE
gender ( female)
hyperkeratosis follicular plugging and infiltration of mononuclear cell into the dermal-epidermal junction
discoid rash
deposition of immune complexes in the epidermal junction characteristic of sle
lupus band test
what is the hallmark of SLE
multiorgan disease
kind of SLE that is equal in both males and females
drug induced SLE
acute common cause of death in SLE
infection and complication of GN
long term, most common cause of death in SLE
complications of atherosclerosis and malignancy
most feared complication of neonatal lupus
complete heart block
most common inflammatory myositis in children
Juvenile dermatomyositis (JDM)
child with rash on neck and chest photosensitivity, weakness, diffused facial rash, erythema on knees and elbow with bluish discoloration of the eyes
JDM
erythema of the neck and trunk seen in JDM is called
shawl sign
blue violet discoloration of the eyelids seen in JDM
heliotrope rash
pink atrophic plaques in interphalangeal joint seen in JDM
gottron papules
the use of hands to stand from a sitting position commonly seen in JDM
gower sign
a variant of JDM with rash but no weakness or inflammation
amyopathic JDM
methylpred is the treatment of choice for JDM, ___ is given to reduce toxicity of its inhibition
folic acid
__ is the most common visceral manifestation of of Systemic sclerosis
pulmonary disease
___ most frequent initial symptom in pediatric systemic sclerosis
raynauds phenomenon
___ a condition of episodic color changes and the development of nodules in severe cold exposure seen in SLE
childblains
the most common cause of death in scleroderma
heart failure due to myocardial and pulmonary fibrosis
