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Paeds Vivasrheumatology-musculoskeletal-and-sports

Paeds Vivas · rheumatology-musculoskeletal-and-sports

IgA vasculitis — branching viva

Branching viva on IgA vasculitis (Henoch-Schonlein purpura): recognising the classic tetrad and applying the EULAR/PRINTO/PRES criteria, then branching to the corticosteroid evidence and the renal monitoring, to the intussusception and the surgical interface, and to the nephrotic presentation and the immunosuppression.

branching clinical structured oral1 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
Emergency department: a five-year-old boy, one week after a sore throat, with a raised purplish rash on his lower legs and ankles, swollen painful knees, colicky abdominal pain, and a urinalysis showing microscopic haematuria with trace protein. The examiner asks: name the diagnosis and give the classification criteria, then branches to the corticosteroid decision and the evidence that prednisone does not prevent the nephropathy, to the same boy re-presenting with severe worsening abdominal pain and bile-stained vomiting that opens the intussusception and its surgical interface, and finally to a nephrotic-range proteinuria with hypertension that opens the renal biopsy and the immunosuppression pathway.

Opening question

A five-year-old boy, one week after a sore throat, presents with a raised purplish rash on his lower legs and ankles, swollen painful knees, colicky abdominal pain, and a urinalysis showing microscopic haematuria with trace protein. His platelet count and coagulation are normal. Name the diagnosis, give the EULAR/PRINTO/PRES classification criteria, and state what excludes the life-threatening mimics at the bedside. [1][4]

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References6ShowHide
  1. [1]Ozen S, Pistorio A, Iusan SM, et al EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria Ann Rheum Dis, 2010.PMID 20413568
  2. [2]Ronkainen J, Koskimies O, Ala-Houhala M, et al Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial J Pediatr, 2006.PMID 16887443
  3. [3]Jauhola O, Ronkainen J, Koskimies O, et al Outcome of Henoch-Schönlein purpura 8 years after treatment with a placebo or prednisone at disease onset Pediatr Nephrol, 2012.PMID 22311342
  4. [4]McCarthy HJ, Tizard EJ Clinical practice: Diagnosis and management of Henoch-Schönlein purpura Eur J Pediatr, 2010.PMID 20012647
  5. [5]Davin JC, Coppo R Henoch-Schönlein purpura nephritis in children Nat Rev Nephrol, 2014.PMID 25072122
  6. [6]Ozen S, Marks SD, Brogan P, et al European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative Rheumatology (Oxford), 2019.PMID 30879080
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