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Henoch Schonlein Purpura (HSP)

Henoch Schonlein Purpura (HSP). O verview. Summary Epidemiology Etiology and Pathogenesis Clinical Manifestations Diagnosis Treatment. Henoch Schonlein Purpura (HSP). Most common systemic vasculitis in childre n 90% of cases occur in the pediatric group Etiology : Unknown

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Henoch Schonlein Purpura (HSP)

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  1. HenochSchonlein Purpura(HSP)

  2. Overview • Summary • Epidemiology • Etiology and Pathogenesis • Clinical Manifestations • Diagnosis • Treatment

  3. HenochSchonlein Purpura (HSP) • Most common systemic vasculitis in children • 90% of cases occur in the pediatric group • Etiology: Unknown • Pathogenesis: End organ IgA immune complex (IC) deposition • Complications: Renal

  4. HenochSchonlein Purpura (HSP) • Clinical Diagnosis: • Palpable purpura without thrombocytopenia • Abdominal pain • Renal disease • Arthritis • Treatment: Disease usually self limited and treatment is usually symptomatic

  5. Epidemiology • Disease of early childhood ages 3-15 • 20 per 100,000 in UK children < 17 years-old • 70 per 100,000 in UK children 4-6 years-old • No comparable data in adults, less common • Male : Female ratio 1.2- 1.8: 1 Arthritis Rheum 1997 May;40(5):859-64

  6. Epidemiology • Less common in African American children • More severe course in adults • More frequent and severe renal disease  requirement for more aggressive treatment • Seasonal variance occurs in the fall, winter and spring and is rare in summer

  7. Etiology • Unknown etiology! • Precipitating antigen may be infectious • Many cases follow upper respiratory infection (URI) • Example: identical twins following adenovirus infection: • HSP in one, IgA nephropathy in other J Pediatr 1985 Jan;106(1):27-32.

  8. Pathogenesis • Immune-complex (IC) mediated disease associated with Immunoglobin A (IgA) deposition • Characteristic findings is leukocytoclastic vasculitis (LCV)of post capillary venules • Accompanied by IgA deposition within affected organs • IC of IgA1 is the ONLY subtype • Immune complexes activate complement (alternative)

  9. Pathogenesis • Hinge region O-linked glycans of IgA1 are deficient in galactose and/or sialic acid content • Renal mesangial cells bind galactose/sialic acid deficient hinge regions • Berger’s disease (IgA nephropathy) also involves IgA1 exclusively

  10. Clinical Features • Classic Tetrad: May develop over days to weeks • Rash (100%) • Arthralgias (82%) • Abdominal pain (63%) • GI bleeding (33%) • Renal disease (40%) Medicine (Baltimore) 1999 Nov;78(6):395-409.

  11. Clinical Features • Presenting feature by % • Rash 74% • Arthralgias 15% • Abdominal pain 12%

  12. Classification Criteria ACR 1990 EULAR / PRES 2005 Palpable purpura without coagulopathy or decreased platelets AND Diffuse abdominal pain Arthritis or arthralgia Bopsy with IgA deposition • Palpable Purpura • Age at onset < 20 years • Acute abdominal pain • Biopsy: • Granulocytes in walls of small arterioles / venules • > or = 2 criteria • 90% Sensitivity/Specificity

  13. Palpable Purpura • Erythematous, macular or urticarial wheals  petechia/palpable purpura • NORMAL clotting studies and platelets • Appears in crops • Symmetric distribution • Gravity/pressure dependent areas such as lower extremities

  14. Palpable Purpura • Localized subcutaneous edema in dependent and periorbital areas seen in children < 3 years old • Differential diagnosis of palpable purpura: • Mixed Cryoglobulinemia • Hypersensitivity vasculitis

  15. Arthritis/Arthralgias • Occur in up to 84% of patients • Uncommon sole presenting symptom at presentation • Transient, migratory oligoarthritis (1-4 joints) • Lower extremities > upper extremity joints • More common in hips, knees and ankles • Non-destructive arthritis (no chronic damage)

  16. Arthritis/Arthralgias • Prominent periarticular swelling with no synovitis • Significant pain + limited range of motion • Differential diagnosis (DDx): • Juvenile Idiopathic arthritis (JIA) • Rheumatoid arthritis (RA) • Systemic Lupus Erytematous (SLE)

  17. Gastrointestinal (GI) Symptoms Mild Symptoms Severe Symptoms GI bleed Bowel ischemia and necrosis Intussusception Bowel perforation • Nausea / vomiting • Abdominal pain • Transient paralytic leus

  18. Gastrointestinal (GI) Symptoms • Develops typically within 8 days of the rash • GI symptoms precede the rash in 15-35% of cases • Abdominal pain = submucosal hemorrhage + edema • Guaiac + stool is seen in 56% of patient • Massive GI hemorrhage is rare • Purpuric lesions may be seen on endoscopy

  19. Intusseception • Most common gastrointestinal complication of HSP • Incidence of 3.5% • Edema and hemorrhage contributes to development • Limited to small bowel in 60% of cases • Initial screening test: Ultrasound • Differential diagnosis: Appendicitis

  20. Renal Disease • Occurs in 20-54 % of children (more prevalent in adults) • 2 days to 4 weeks after onset of systemic symptoms • Hematuria is most common presentation • With or without red blood cell (RBC) casts • Nephrotic range proteinuria, ↑ creatinine and or hypertension  ↑ risk of progressive disease (adults)

  21. Renal Disease • % of glomeruli with crescents has prognostic significance • > 50% • 37% progressed to end stage renal disease (ESRD) • 18% with chronic renal insufficiency (CRI) • Differential diagnosis: • Berger’s Disease

  22. Renal Disease • Correlation between disease severity and biopsy findings • Asymptomatic hematuria: focal mesangial proliferation • Proteinuria: cellular proliferation • Nephrotic range proteinuria: crescents J Am Soc Nephrol 1999 Dec;10(12):2637-44

  23. Scotum Involvement • 2-38% involvement • Presentation mimics testicular torsion • Pain, tenderness and swelling • Evaluate with ultrasound or radionuclide scanning • Testicular torsion will show ↓ blood flow versus normal blood flow in HSP

  24. Clinical Findings in Adults with HSP • Similar manifestations as in children • Two main differences between adults and children: • Intussusception is rare in adults • Adults have ↑ risk of developing significant renal involvement including end-stage renal disease

  25. Diagnosis • Clinical manifestations (know the Tetrad) • Classification criteria • Gold Standard is biopsy • Demonstration of LCV with IgA deposition = HSP

  26. Biopsy Diagnosis • Biopsy obtained in children: • Unusual presentation or significant renal disease • Biopsy in adults: • Due to lower incidence, confirmation by biopsy is more important

  27. Skin Biopsy • Superficial dermis biopsy sufficient • Goal to obtain skin lesions less than 24 hour-old • Leukocytoclastic vasculitis in post capillary venules with IgA deposition = pathognomonic of HSP

  28. Renal Biopsy • Obtain in severe renal involvement or uncertain diagnosis • Characterized by IgA deposition in mesangium • Immunoflorence studies  IgG, fibrin, C3 • Mesangial proliferation to crescentic glomerulonephritis (GN) • Biopsy generally parallels clinical disease severity

  29. Labs • No lab test is diagnostic for HSP • Labs obtained tend to be non-specific • Normal coagulation studies and platelets • This differentiates HSP from other diseases • Need to obtain renal function and urinalysis at a diagnosis

  30. Imaging Studies • Obtain in patients with significant abdominal symptoms • Plain abdominal X-ray: • May demonstrate dilated bowel loops • Ultrasound: • ↑ bowel wall thickness, hematomas and intussusception

  31. Treatment • Complete recovery 94% children, 89% of adults • Supportive treatment in vast majority • Rest • Hydration • NSAIDS

  32. Treatment • Hospitalization indicated: • Inability to maintain adequate hydration with oral intake • Severe abdominal pain • Significant gastrointestinal bleeding • Changes in mental status • Severe joint involvement limiting ambulation • Renal insufficiency (↑ Creatinine), HTN, nephrotic syndrome

  33. Treatment with Corticosteroids (CS) • Use is controversial • Benefits include: • Shortened duration of abdominal pain • ↓ risk of intussusception • ↓ risk of recurrence • ↓ risk of renal involvement

  34. Corticosteroids • Results of benefit mixed in studies • Absence of definitive evidence of long term benefits • Only used in patients with severe symptoms requiring hospitalization • Prednisone 1-2 mg/kg/day, max dose of 60-80 mg/day • Inflammation will be improved but the pathophysiology of the disease does not appear to be affected

  35. Specific Therapy: GI disease • Gastrointestinal • Corticosteroids not proven to decrease risk of intussusception • Using corticosteroids after intussusception has occurred may obscure the signs of compromised bowel viability Medicine (Baltimore) 1999 Nov;78(6):395-409.

  36. Specific Therapy: Renal disease • Should only be considered in patients with: • Marked proteinuria and/or impaired renal function • Therapies for cresentic nephritis: • High dose methylprednisolone  oral prednisone for 3 months has shown benefit • Azathioprine and corticosteroids • Cyclophosphamide

  37. Recurrent Disease • Recurrence occurs in 1/3rd of children • Occurs within 4 months of initial episode • Usually milder and briefer and occur in patients: • Nephritis • Evidence of acute inflammation • Patients who received corticosteroids

  38. Summary • Most common systemic vasculitis in children • 90% of cases occur in the pediatric group • Etiology: Unknown • Pathogenesis: End organ IgA immune complex (IC) deposition • Complications: Renal, GI

  39. Summary • Clinical Diagnosis: • Palpable purpura without thrombocytopenia • Abdominal pain • Renal disease • Arthritis • Treatment: Typically symptomatic and self limited • Unclear role of corticosteroids in TX • No specific agent proven efficacious for persistent renal disease

  40. References • Niaudet P, et al. Renal manifestations of Henoch-Schönlein purpura. Uptodate 2012. • Dedeoglu F, et al. Clinical manifestations and diagnosis of Henoch-Schönlein purpura. Uptodate 2012. • Dedeoglu F, et al. Management of Henoch-Schönlein purpura. Uptodate 2012.

  41. Questions?

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