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Henoch-Schönlein Purpura
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Background
HSP is a small-vessel vasculitis characterized by
purpura, arthritis, abdominal pain, and
hematuria.
It is also known as anaphylactoid pupura.
It is the most common cause of non-
thrombocytopenic purpura in children
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Epidemiology
• Males are affected twice as frequently as
females.
• And the overall incidence is estimated to be
9/100000 population
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Pathophysiology
– This illness is considered by histopathology to be
an IgA mediated vasculitis of small vessels.
– And immunoflorescent techniques may showdeposition of IgA and C3 in the small vessels of
the skin and the renal glomeruli
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Mortality/Morbidity
In general, HSP is a benign self-limited disorder.
Fewer than 5% of cases cause chronic symptoms.
Fewer than 1% of cases progress to end-stage renal failure.
Sex: Male-to-female ratio is about 2:1.
Age: Seventy-five percent of patients affected are aged 2-8years.
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Physical
Palpable purpura, particularly on the buttocks and
legs, Edema of the hands, feet, scalp, and ears
Arthritis, most commonly involving the knees andankles
Abdominal tenderness
Gastrointestinal bleeding
Acute scrotal edema that may mimic testicular torsion
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Causes
The etiology of HSP is unknown.
About 50% of patients have a preceding upper
respiratory illness (URI).
Multiple infectious agents as well as drugs, foods,and insect bites may trigger HSP.
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Lab Studies
Diagnosis is clinical and not based on laboratoryevaluation. Routine lab test results are usually withinreference ranges.
Some lab studies help in excluding other diagnoses andin evaluating renal function, including urinalysis, CBCwith platelet count and differential, BUN, creatinine,prothrombin time (PT), activated partial thromboplastintime (aPTT),lipase.
Urinalysis: Hematuria and/or proteinuria are present in10-20% of patients.
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Lab Studies
Platelet count and coagulation studies:Platelet count is usually in the reference range but may be
elevated; the platelet count should not be low in HSP.
A normal platelet count rules out idiopathic
thrombocytopenic purpura (ITP). A normal platelet count and normal coagulation studies
(ie, PT, aPTT, fibrin split products) rule out thromboticthrombocytopenic purpura (TTP).
Lipase: A normal lipase makes acute pancreatitisvery unlikely.
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Lab Studies
CBC and differential: WBC count may be in the
reference range or elevated. Eosinophilia is
sometimes present.
Erythrocyte sedimentation rate: Sedimentation rate
may be in the reference range or elevated.
BUN and creatinine levels: These may be elevatedfrom renal involvement of HSP or from dehydration.
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Imaging Studies
Abdominal ultrasound may be better than barium enema
to diagnose intussusception, since HSP-related
intussusception is more often ileoileal instead of ileocolic
as is typical in idiopathic intussusception. And also due
to the risk of perforation.
Doppler or radionuclide testicular scan results show
normal or increased blood flow in HSP, in contrast to the
decreased blood flow seen in testicular torsion.
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• Definitive diagnosis of vasculitis, confirmed by biopsy of
an involved cutanuous site, shows leuckocytoclastic
angiits.
• Renal biopsy may show IgA mesengial deposition and
occasionally IgM,C3, and fibrin.
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• Emergency Department Care:
– Treatment of HSP is mostly supportive and includes
ensuring adequate hydration and monitoring for abdominal
and renal complications, and pain control with
acetominophen.
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Medications
• Nonsteroidal anti-inflammatory drugs (NSAIDs) may help
joint pain and do not worsen the purpura. However,
NSAIDs should be used cautiously in patients with renal
insufficiency.
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Medications
• A recent study of 12 patients with severe HSP
nephritis indicates that patients did well with a
treatment of methylprednisolone at 30 mg/kg/d for 3
days followed by
– oral corticosteroids at 2 mg/kg/d for 2 months,
– cyclophosphamide at 2 mg/kg/d for 2 months,
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Nonsteroidal anti-inflammatory drugs
(NSAIDs)
• These agents are most commonly used for therelief of mild to moderately severe pain
•
Although the effects of NSAIDs in the treatmentof pain tend to be patient specific, ibuprofenusually is the DOC for initial therapy.
• Other options include ketoprofen, and naproxen.
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Nonsteroidal anti-inflammatory drugs
(NSAIDs)
• Ibuprofen (Ibuprin, Advil, Motrin) –
– DOC for treatment of mild to moderately severe pain, if no
contraindications. – Inhibits inflammatory reactions and pain, probably by
decreasing activity of enzyme cyclooxygenase, which
results in inhibition of prostaglandin synthesis.
• Dose: 10 mg/kg PO q6h
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Nonsteroidal anti-inflammatory drugs
(NSAIDs)
• Ketoprofen (Oruvail, Orudis, Actron)
– Used for relief of mild to moderately severe pain
and inflammation.
• Dose:
– 3 months to 12 years: 0.1-1 mg/kg PO q6-8hrs
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Nonsteroidal anti-inflammatory drugs
(NSAIDs)
• Naproxen (Anaprox, Naprelan, Naprosyn)
– Used for relief of mild to moderately severe pain.
– Inhibits inflammatory reactions and pain by
decreasing activity of enzyme cyclooxygenase, whichresults in decrease of prostaglandin synthesis.
• Dose: 2.5 mg/kg/dose PO; not to exceed 10
mg/kg/d
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Corticosteroids
• These agents have anti-inflammatory properties and
cause profound and varied metabolic effects. In
addition, they modify the body's immune response
to diverse stimuli.
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Corticosteroids
• Prednisone
– Useful in treatment of inflammatory and
autoimmune reactions.
–By reversing increased capillary permeability andsuppressing PMN activity, may decrease
inflammation.
• Dose: 1-2 mg/kg/d PO divided bid
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Prognosis
HSP is generally a benign disease with an excellentprognosis.
More than 80% of patients have a single isolatedepisode lasting a few weeks.
Approximately 10-20% of patients have recurrences.
Fewer than 5% of patients develop chronic HSP.
Abdominal pain resolves spontaneously within 72 hoursin most patients.
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Thank You
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