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.
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Target exams
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]
Branch 1 — the corticosteroid decision and the renal-prevention evidence
The diagnosis is confirmed. What is your acute management, and what is the role of the corticosteroid? State the indications, the dose, and the evidence from the Ronkainen 2006 and the Jauhola 2012 trials on whether the prednisone prevents the nephropathy. What is the renal monitoring schedule, and why is it mandatory regardless of the corticosteroid use? [2][3]
Branch 2 — the intussusception and the surgical interface
The same boy re-presents two days later with the severe worsening abdominal pain, the bile-stained vomiting, and a palpable right-sided mass. What is the complication, what is its typical location in this disease, why does it matter for the reduction method, and how is it managed? [4][6]
Branch 3 — the nephrotic presentation and the immunosuppression
The boy instead develops the oedema, the hypertension, and a nephrotic-range proteinuria six weeks into the illness. What is the significance, what is the indication for the renal biopsy, how does the International Study of Kidney Disease in Children classification guide the treatment, and what is the role of the angiotensin-converting-enzyme inhibitor and the immunosuppression? [5][6]
Closing — coordination and the long-term outcome
In one sentence, what determines the long-term outcome of IgA vasculitis, and why does the general paediatrician sit at the centre of the classification, the corticosteroid decision, the renal monitoring, and the multidisciplinary referral? [4][5]
References
- [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]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]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]McCarthy HJ, Tizard EJ Clinical practice: Diagnosis and management of Henoch-Schönlein purpura Eur J Pediatr, 2010.PMID 20012647
- [5]Davin JC, Coppo R Henoch-Schönlein purpura nephritis in children Nat Rev Nephrol, 2014.PMID 25072122
- [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