Henoch-Schönlein purpura (HSP)

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

2. BACKGROUND 1 st 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

6. Palpable Purpura : Symmetrical Dependent areas

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

8. 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.

9. 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.

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

11. DIAGNOSIS

12. 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

13. Differential diagnosis

14. 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

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

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

17. 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.

18. 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.

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