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Henoch-Schönlein purpura (HSP)

Henoch-Schönlein purpura (HSP)






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    Henoch-Schönlein purpura (HSP) Henoch-Schönlein purpura (HSP) Presentation Transcript

    • Henoch-Schönlein purpura (HSP) Ahmed Abdul Ghany
    • 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.
    • EPIDEMIOLOGY HSP (IgAV) is a systemic vasculitic syndrome seen primarily in children. Male –to- female ratio: 1.8: 1
    • PATHOGENESIS Immunoglobulin A deposition
    • CLINICAL MANIFESTATIONS Joints Abdominal pain Renal Palpable Purpura
    • Palpable Purpura: Symmetrical Dependent areas
    • Arthralgia/ arthritis: 2nd most common presentation 84% Usually transient or migratory Oligoarticular Nondeforming Lower extremity large joints
    • 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.
    • 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.
    • Other organs: CNS including intracerebral hemorrhage. Pulmonary hemorrhage Keratits and uveitis
    • DIAGNOSIS Lab. Serum IgA (50-70%) Abdominal U/S Biopsy .
    • 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
    • Differential diagnosis DD Purpura Hypersenstivity vacsulitis Other small vs vasculitis SLE infections Arthritis Autoimmune Septic arthritis Renal Abdominal Pain
    • 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
    • Supportive care: • Includes adequate hydration, rest and pain relief.
    • Symptomatic therapy: NSAIDs: • Naproxen 10 – 20 mg/kg • Ibuprofin and other NSAIDs are equally effective
    • 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.
    • 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.