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Morning Report

August 4, 2009
HSP
AKA Henoch-Schnlein Purpura
Most common form of systemic vasculitis in
children
90% of cases are pediatric
Majority are self limited
HSP
Epidemiology
3-15 years of age
Mean 6-7 years
Slight male predominance
Less frequently in black children
Rare in summer
Triggers
Preceding URI in 50%
Other infections, vaccinations, insect bites
Underlying cause is unknown
HSP
Pathogenesis
Immune-mediated
vasculitis
IgA deposition
small vessels of
skin and kidneys
HSP
Classic Tetrad
Palpable purpura
Without thrombocytopenia or coagulopathy

Present in almost all patients

Arthritis/Arthralgia
75%

Abdominal Pain
50%

Renal Disease
21-54%

Not all symptoms must be present for diagnosis


Takes days to weeks to develop
May present as abd pain or joint complaints*
HSP
Rash
Erythematous, macular
or urticarial wheals
Wheals coaslesce and
evolve to ecchymoses,
petechiae and
palpable purpura
Crops, symmetrical
distribution, located in
gravity/pressure
dependant areas
Lower extremities

Toddlers buttocks,

face, trunk and UE


HSP
Arthritis/Arthralgia
Transient or migratory
Oligoarticular
1 4 joints

Nondeforming w/o chronic


sequelae
Involved joints
Hip, knee, ankle

Less common elbow,

wrist or hand
Periarticular swelling and
tenderness
No effusion, erythema

or warmth
Cause considerable pain
and limited motion
HSP
GI
Mild symptoms
Nausea, vomiting, pain, transient paralytic ileus
Severe symptoms
Hemorrhage, bowel ischemia and necrosis,
intussusception, bowel perforation
Seen within 8 days of rash
Pain caused by submucosal hemorrhage and
edema
HSP
Intussusception
Most common severe
GI complication
Limited to small bowel
60% Ileal-Ileal
HSP
Renal
Mild
Isolated hematuria and/or proteinuria
Severe
Acute nephropathy w/ renal insufficiency
Within 4 weeks of presentation
Excellent prognosis*
Small risk of progressive disease
Nephrotic syndrome is poor prognostic sign*
HSP
Other manifestations
Orchitis
Seizures
Parotitis
Carditis
Pulmonary hemorrhage
HSP
Recurrence
1/3 of cases
Within 4 months
Milder and shorter
HSP
Diagnosis is Mainly Clinical
Unusual presentations may require biopsy

Lab*
No test is diagnostic
IgA elevated in 50-70%
Platelet count and coags normal
If inconclusive on H&P
U/A*
RBCs, casts, proteinuria
Screen beyond acute presentation
HSP
Treatment*
Outpatient
Mainly supportive
Hydration

IVFs or TPN
Rest
Symptomatic relief of pain
NSAIDs
Edema
Elevation of area
HSP
When to admit?
Not maintaining hydration
Severe abdominal pain
Significant GI bleeding
AMS
Severe joint involvement limiting ambulation
Renal Insufficiency
HTN and/or nephrotic syndrome
HSP
Steroids
Use is controversial
Reported benefits include
Decreased duration of abdominal pain

Decreased risk of intussusception, recurrence, renal

involvement
Have not been proven in studies

Recommended for use with severe abdominal pain


Prednisone 1-2mg/kg/day (max 60-80mg)

Methylprednisone 0.8-1.6mg/kg/day (max 64mg/day)

Weaned over 4-8 weeks


HSP
Prognosis
Excellent
Resolves within 1 month
Recurrence in 1/3
Milder
Morbidity
GI
Renal
HSP
Follow Up
UA and BP
1st 2 months
Weekly or biweekly
If results remain normal space to monthly then
every other month for the 1st year