Morning Report

August 4, 2009

HSP

AKA Henoch-Schönlein 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