hsp 2.pptx

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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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(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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(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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(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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hsp 2.pptx

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