This document discusses Henoch-Schönlein purpura (HSP), a systemic vasculitic syndrome seen primarily in children. It was first described in 1801 in a 5-year old boy presenting with hematuria, abdominal pain, joint pains and skin rash. HSP is characterized by immunoglobulin A deposition which can cause palpable purpura rashes, arthralgia, abdominal pain, and potentially serious renal involvement in 21-54% of cases. Diagnosis involves laboratory tests, abdominal ultrasound, and skin or renal biopsy. Treatment is usually supportive with NSAIDs, glucocorticoids in severe cases, and disease-modifying agents for gastrointestinal and renal complications.

1. Henoch-Schönlein
purpura (HSP)
Ahmed Abdul Ghany

2. BACKGROUND
1st described in 1801 by William Heberden, a
physician in london, who wrote about a case of
a 5 year old boy with hematuria, abdominal
pain, joint pains and skin rash.

3. EPIDEMIOLOGY
HSP (IgAV) is a systemic vasculitic syndrome
seen primarily in children.
Male –to- female ratio: 1.8: 1

4. PATHOGENESIS
Immunoglobulin A
deposition

5. CLINICAL
MANIFESTATIONS
Joints
Abdominal
pain
Renal
Palpable
Purpura

6. Palpable Purpura:
Symmetrical
Dependent areas

8. Arthralgia/ arthritis:
2nd most common presentation 84%
Usually transient or migratory
Oligoarticular
Nondeforming
Lower extremity large joints

9. Abdominal Pain:
50% of patients complain of colicky pain
typically develop within 8 days of the
appearance of rash.
GI bleeding in 20 – 30 %
Inussusception is a common complication in
children.

10. Renal disease:
Ranges from 21-54 %
Hematuria with or without red cell cast.
Proteinuria ranges from mild to nephrotic range.
Elevated creatinine and/ or HTN.

11. Other organs:
CNS including intracerebral hemorrhage.
Pulmonary hemorrhage
Keratits and uveitis

12. DIAGNOSIS
Lab.
Serum IgA
(50-70%)
Abdominal U/S
Biopsy .

13. Renal biopsy is reserved
for patients in whom the
diagnosis is uncertain or
evidence of sever renal
impairment
Skin biopsy including
small blood vessels of
superficial dermis

14. Differential diagnosis
DD
Purpura
Hypersenstivity
vacsulitis
Other small vs
vasculitis
SLE
infections
Arthritis
Autoimmune
Septic arthritis
Renal
Abdominal
Pain

15. Management
Admission is warranted for the following:
• Sever abdominal pain
• GI bleeding
• Elevated creatinine, HTN, and/ or nephrotic
• Sever joint involvement
• Changes in mental status

16. Supportive care:
• Includes adequate hydration, rest and pain
relief.

17. Symptomatic therapy:
NSAIDs:
• Naproxen 10 – 20 mg/kg
• Ibuprofin and other NSAIDs are equally
effective

18. Glucocorticoids
• Their use in patients with HSP is controversial
• Prednisone 1- 2 mg /kg daily (max 80 mg)
• To be used only in patients with symptoms
sever enough to affect oral intake or daily
activities.

19. Disease modifying agents:
• Targeted toward preventing or ameliorating GI
and renal complications.
• Limited data suggest that cyclophosphamide
and cyclosporine may be beneficial.
• Plasmapharesis has been used in patients with
crescentic disease and rapidly progressive
renal failure.

20. THANK YOU