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Good Morning All!!

Good Morning All!!. Morning Report: Thursday, November 10 th , 2011. Henoch-Sch Ö nlein Purpura. Epidemiology. Most common systemic vasculitis of childhood Incidence 10/100,000 children/yr Average age of occurrence is 6 yo Majority of cases <10 yo Affects boys more commonly (2:1)

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Good Morning All!!

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  1. Good Morning All!! Morning Report: Thursday, November 10th, 2011

  2. Henoch-SchÖnleinPurpura

  3. Epidemiology • Most common systemic vasculitis of childhood • Incidence 10/100,000 children/yr • Average age of occurrence is 6 yo • Majority of cases <10 yo • Affects boys more commonly (2:1) • More cases in the winter and spring

  4. Etiology • Take a guess…UNKNOWN! • Many suspected “triggers”: • Allergies • Bacteria • Streptococcus pyogenes • Viruses • 50% of cases preceeded by a URI • Drugs • PCN • Cephalosporins • Thiazide diuretics • Vaccines • Insect bites

  5. Pathophysiology • Leukocytoclasticvasculitis • Aka: small vessel vasculitis • IgA accumulation in vessel walls and the renal mesangium • Leads to inflammation and necrosis of predominantly postcapillaryvenules, capillaries and arterioles

  6. Clinical Presentation

  7. NonthrombocytopenicPurpura

  8. The Rash… • Palpable purpuric lesions seen in 100% of patients • Presenting symptom in 50% • Usually found on the lower extremities in the dependent, pressure-bearing areas • Buttocks especially prone • Lesions can also occur on UE, face and trunk • Angioedema of the scalp/face,extremities, and GU region occurs in 20-46% of patients

  9. The Rash… Deep red macules palpable purpura or hemorrhagic bullae

  10. Arthritis and/or Arthralgia

  11. Joint Complaints… • Occur in 50-80% of patients • Presenting symptom in 25% • Periarthritis • Edema AROUND the joints and inflammation involving the tendon sheaths • Affected joints may be swollen, tender and painful on motion • Most commonly affects knees and ankles

  12. GI Disease

  13. GI Unpleasantness… • GI disease noted in 67% of affected children • Due to submucosal and subserosal hemorrhage • Most common manifestations: • Colicky abdominal pain • +/- vomiting • Stools with gross or occult blood • Can also see: • Intussusception • 4-5% of patients • Ileoileal in location rather than ileocolic

  14. GI Unpleasentness… • Can also see (con’t): • Bowel infarction/ perforation • Pancreatitis • Hydrops of the gallbladder

  15. *Renal Disease

  16. Renal Manifestations… • Occur in up to 40-50% of patients with HSP • Most present in the first month following rash • BUT can occur anytime within 3-4 months • Histopathology very similar to Berger disease • IgA deposition • *Wide range of clinical expression: • Microscopic hematuria (most common) • Mild proteinuria • Nephritic and/or nephroticsyndrome

  17. Renal Manifestations • Prognosis • Hematuria alone • No long-term sequelae • Combination nephritic/nephrotic syndromes • 50% of these patients develop ESRD within 10 years • *Persistence of nephrotic-range proteinuria is the most accurate predictor of eventual renal failure • *Overall, progression to renal failure is seen in only 1-5% of patients with HSP

  18. *Less Common Features of HSP • Orchitis • CNS symptoms • HA • Seizures • Coma • Guillain-Barre syndrome • Parotitis • Carditis • Pulmonary hemorrhage

  19. *Lab Findings • Non-specific • CBC • Nml or elevated WBC ct • Eosinophilia • Anemia (secondary to bleeding) • Elevated plt ct • BMP • Electrolyte disturbances due to GI disease • Elevated BUN/Cr with renal disease • UA • Microscopic hematuria (*most common lab finding*) • Proteinuria • Elevated inflammatory markers • Decreased Factor VIII levels • Normal PT/PTT

  20. Treatment • Supportive Care • NSAIDs for pain management • Avoid with significant GI or renal disease • Maintain hydration, nutrition, electrolyte balance • Glucocorticoids • Controversial!! • Quickly improve abdominal pain and severe scrotal swelling/edema • No RCT have shown efficacy for HSP nephritis • ?May decrease the chance of persistent renal disease • ?May reduce risk of intussusception

  21. Prognosis • 67% of children with HSP run the course of the disease within 4 weeks of onset • Recurrence in 25% of patients • Usually manifests as rash and abdominal pain after a respiratory infection • Significant morbidity • Acute GI bleeds • Long-term renal involvement • Need to f/u with frequent UAs and BP measurements for the first 4 months after presentation • Most children who develop abnormal urine findings do so within 4 months of diagnosis

  22. Noon Conference: Dr. Mouallem, Fractures Thanks for your attention!

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