Henoch Schonlein Purpura

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Henoch Schonlein Purpura

  1. 2. HENOCH-SCHÖNLEIN PURPURA Morning Report July 6, 2007 Sima Patel, MD
  2. 3. DEFINITION <ul><li>Also called “anaphylactoid purpura” </li></ul><ul><li>HSP is a systemic vasculitic syndrome with: </li></ul><ul><ul><li>Palpable purpura </li></ul></ul><ul><ul><li>Arthralgias </li></ul></ul><ul><ul><li>GI involvement </li></ul></ul><ul><ul><li>Glomerulonephritis </li></ul></ul>
  3. 4. BACKGROUND <ul><li>First 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 a skin rash. </li></ul><ul><li>In 1837, Johann Schönlein and later in 1874, Edouard Henoch described multiple case reports of similar cases. They also showed an association of an upper respiratory infection preceding development of symptoms. </li></ul>
  4. 5. EPIDEMIOLOGY <ul><li>90% of cases reported in children </li></ul><ul><ul><li>Peak in children aged 4-7 </li></ul></ul><ul><li>Male:Female (1.5:1) </li></ul><ul><li>50% follow a URI </li></ul><ul><li>Renal disease is more severe in adults </li></ul>
  5. 6. PATHOGENESIS <ul><li>Likely mechanism thought to be an immune-complex mediated disease with deposits in the glomerular capillaries, dermal capillaries and GI tract. </li></ul><ul><li>Mesangial deposits of IgA are the same as those seen in IgA nephropathy </li></ul>
  6. 7. PRECIPITATING ANTIGENS <ul><li>INFECTIONS </li></ul><ul><ul><li>URI </li></ul></ul><ul><ul><li>Measles </li></ul></ul><ul><ul><li>Rubella </li></ul></ul><ul><ul><li>Parvovirus B19 </li></ul></ul><ul><ul><li>Mycoplasma </li></ul></ul><ul><ul><li>Coxsackie virus </li></ul></ul><ul><ul><li>Toxocara </li></ul></ul><ul><ul><li>Amebiasis </li></ul></ul><ul><ul><li>Salmonella </li></ul></ul><ul><ul><li>C.difficile </li></ul></ul><ul><ul><li>H.pylori </li></ul></ul><ul><ul><li>Adenovirus </li></ul></ul><ul><ul><li>Legionella </li></ul></ul><ul><ul><li>Tuberculosis </li></ul></ul><ul><ul><li>Mumps </li></ul></ul><ul><ul><li>Streptococcus </li></ul></ul><ul><ul><li>Morganella morganii </li></ul></ul>
  7. 8. PRECIPITATING ANTIGENS <ul><li>Drugs </li></ul><ul><ul><li>Vancomycin </li></ul></ul><ul><ul><li>Streptokinase </li></ul></ul><ul><ul><li>Ranitidine </li></ul></ul><ul><ul><li>Cefuroxime </li></ul></ul><ul><ul><li>Diclofenac </li></ul></ul><ul><ul><li>Enalapril </li></ul></ul><ul><ul><li>Captopril </li></ul></ul>
  8. 9. PRECIPITATING ANTIGENS <ul><li>Other: </li></ul><ul><ul><li>Food hypersensitivity </li></ul></ul><ul><ul><li>Cold exposure </li></ul></ul><ul><ul><li>Autosomal recessive Chronic granulomatous disease </li></ul></ul><ul><ul><li>Myelodysplastic syndrome </li></ul></ul><ul><ul><li>Small cell lung cancer </li></ul></ul><ul><ul><li>Breast cancer </li></ul></ul>
  9. 10. PATHOLOGIC FEATURES <ul><li>DERMATOLOGIC FINDINGS: Leukocytoclastic vasculitis with IgA deposition </li></ul>
  10. 11. Direct Immunofluorescence of skin biopsy. Granular IgA and C3 staining of cutaneous vasculature. http://www.medscape.com/viewarticle/459714
  11. 12. H & E stain of skin biopsy showing leukocytoclastic vasculitis with infiltration of neutrophils. http://www.medscape.com/viewarticle/459714
  12. 13. Skin biopsy: Leukocytoclastic vasculitis with mononuclear and polymorphonuclear cell infiltrates in the perivascular space <ul><ul><li>www.kjronline.org/abstract/view_articletext.asp?year=2004&page=178 </li></ul></ul>
  13. 14. PATHOLOGIC FEATURES <ul><li>RENAL FINDINGS: Granular deposits of IgA, mesangioproliferative glomerulonephritis and crescent formation </li></ul>
  14. 15. Renal biopsy: sclerosis and fibrous crescents in the glomerulus. http://www.ndt-educational.org/nagycase.asp
  15. 16. http://www.ndt-educational.org/nagycase.asp Immunofluorescence: Glomerular deposits of IgA
  16. 17. CLINICAL FEATURES <ul><li>Tetrad of symptoms </li></ul><ul><ul><li>Abdominal pain </li></ul></ul><ul><ul><li>Renal disease </li></ul></ul><ul><ul><li>Palpable purpura </li></ul></ul><ul><ul><li>Arthritis/arthralgias – more common in adults and most common in knees and ankles. Generally self-limiting </li></ul></ul>
  17. 18. CLINICAL FEATURES <ul><li>PALPABLE PURPURA: most commonly seen on lower extremities and buttocks, however can also been seen on the trunk and arms. </li></ul><ul><ul><li>Lesions begin as erythematous macules and progress to purpuric, non-blanching, nonpruritic lesions that may become confluent </li></ul></ul>
  18. 19. CLINICAL FINDINGS <ul><li>GI INVOLVEMENT: more common in children. Symptoms include abdominal pain, nausea, vomiting, diarrhea, constipation or bowel intussusception. May present with GI bleeding. </li></ul>
  19. 20. CLINICAL FEATURES <ul><li>RENAL INVOLVEMENT: </li></ul><ul><ul><li>in up to 50% of patients </li></ul></ul><ul><ul><li>Usually more rapidly progressive in adults. Rare in children </li></ul></ul><ul><ul><li>May present with hematuria </li></ul></ul><ul><ul><li>Can have mild glomerulonephritis leading to microscopic hematuria and can lead to a rapidly progressive glomerulonephritis with RBC casts </li></ul></ul><ul><ul><li>Usually resolve spontaneously. </li></ul></ul>
  20. 21. DIAGNOSTIC EVALUATION <ul><li>May have mild leukocytosis </li></ul><ul><li>Normal platelet count </li></ul><ul><li>Normal serum complement levels </li></ul><ul><li>Elevated IgA in 50% </li></ul>
  21. 22. DIAGNOSIS <ul><li>Generally a clinical diagnosis </li></ul><ul><li>Skin Biopsy: can be helpful and used to confirm IgA and C3 deposits and leukocytoclastic vasculitis. </li></ul><ul><li>Renal Biopsy: not usually needed for diagnosis. Will show mesangial IgA deposits and segmental glomerulonephritis </li></ul>
  22. 23. MANAGEMENT <ul><li>Usually self-limiting (1-6 weeks) </li></ul><ul><li>Steroids: </li></ul><ul><ul><li>may decrease tissue edema, may aid in arthralgias and some abdominal pain </li></ul></ul><ul><ul><li>Has not been shown to be beneficial in kidney disease or dermal manifestations </li></ul></ul><ul><ul><li>Does not lessen chance of recurrence </li></ul></ul><ul><ul><li>Does not shorten duration of disease </li></ul></ul>
  23. 24. MANAGEMENT <ul><li>if rapidly progressive glomerulonephritis </li></ul><ul><ul><li>Multidrug regimens with cytotoxic drugs however not many reports with treatment in adults. </li></ul></ul><ul><ul><li>Plasmaphoresis </li></ul></ul><ul><ul><li>IVIG </li></ul></ul><ul><li>Symptomatic management of GI symptoms and surgical intervention if warranted. </li></ul>
  24. 25. PROGNOSIS <ul><li>Prognostic factors: </li></ul><ul><ul><li>generally a milder course in children with shorter duration and fewer recurrences </li></ul></ul><ul><ul><li>Proteinuria >1gm/day with worse prognosis if develop nephrotic syndrome </li></ul></ul><ul><li>1-5% children progress to ESRD </li></ul><ul><li>Recurrence in up to 40% of patients </li></ul>
  25. 26. REFERENCES <ul><li>Kasper, Dennis, and Eugene Braunwald, 16th edition, eds. Harrison’s Principles of Internal Medicine. New York: McGraw-Hill, 2005. </li></ul><ul><li>Tierney Jr, Lawrence, and Stephen McPhee, 45th edition, eds. Current Medical Diagnosis and Treatment. New York: McGraw-Hill, 2006. </li></ul><ul><li>Uptodate: Clinical manifestations and diagnosis of Henoch-Schonlein Purpura </li></ul><ul><li>Anup Rai, et al., Henoch-Schonlein Purpura Nephritis. American Society of Nephrology. Volume 10, pages 2637-2644, 1999 </li></ul><ul><li>Espositio et al., Henoch-Schonlein Purpura in Chronic Hemodialys patients. Journal of Nephrology. Volume 12: 197-200, 1999 </li></ul>

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