1. Morning Report
August 4, 2009

3. HSP AKA Henoch-Schönlein Purpura
Most common form of systemic vasculitis in children
90% of cases are pediatric
Majority are self limited

4. HSP Epidemiology 3-15 years of age Slight male predominance
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

5. HSP Pathogenesis Immune-mediated vasculitis
IgA deposition small vessels of skin and kidneys

6. HSP Classic Tetrad Not all symptoms must be present for diagnosis
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*

7. 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

8. 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

9. HSP GI Mild symptoms Severe symptoms Seen within 8 days of rash
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

10. HSP Intussusception Most common severe GI complication
Limited to small bowel
60% Ileal-Ileal

11. HSP Renal Mild Severe Within 4 weeks of presentation
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*

12. HSP Other manifestations Orchitis Seizures Parotitis Carditis
Pulmonary hemorrhage

13. HSP
Recurrence
1/3 of cases
Within 4 months
Milder and shorter

14. HSP Diagnosis is Mainly Clinical Lab*
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

15. HSP Treatment* Outpatient Mainly supportive Edema Hydration Rest
IVFs or TPN
Rest
Symptomatic relief of pain
NSAIDs
Edema
Elevation of area

16. 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

17. 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 mg/kg/day (max 64mg/day)
Weaned over 4-8 weeks

18. HSP Prognosis Morbidity Excellent Resolves within 1 month
Recurrence in 1/3
Milder
Morbidity GI
Renal

19. 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