Henoch-Schönlein purpura (HSP)
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  • 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
  • 7. Arthralgia/ arthritis: 2nd 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 Lab. Serum IgA (50-70%) Abdominal U/S Biopsy .
  • 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 DD Purpura Hypersenstivity vacsulitis Other small vs vasculitis SLE infections Arthritis Autoimmune Septic arthritis Renal Abdominal Pain
  • 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.
  • 19. THANK YOU