IgA vasculitis

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Target exams

Red flags

• A febrile ill child with rapidly progressive purpura or petechiae is meningococcaemia or sepsis until proven otherwise, not IgA vasculitis; treat with empiric antibiotics within the first hour while the diagnosis is clarified
• Severe colicky abdominal pain in IgA vasculitis, especially with vomiting, a palpable mass, or gastrointestinal bleeding, raises intussusception, which is often ileo-ileal and may require surgical rather than enema reduction
• Nephrotic or nephritic syndrome at presentation, with hypertension, oedema, oliguria, a rising creatinine, or haematuria with proteinuria, is significant renal involvement that demands nephrology referral, biopsy consideration, and blood pressure and urinalysis monitoring for at least six months
• Scrotal swelling and pain in IgA vasculitis may mimic testicular torsion; both can coexist, so urgent surgical assessment and Doppler ultrasound are required when the torsion cannot be excluded clinically
• Prednisone does not reliably prevent the nephropathy; the routine use of corticosteroids for renal prevention alone is not recommended, and every child needs blood pressure and urinalysis monitored for the full six months regardless of corticosteroid use
• A child who has apparently recovered can develop renal involvement up to six months after onset; the failure to maintain the monitoring schedule is the error that misses the disease that matters most for the long-term outcome

Life stages

Care settings

Clinical exam formats

Board mappings

• Recognises IgA vasculitis (Henoch-Schonlein purpura) as the commonest childhood small-vessel vasculitis, presenting with the tetrad of palpable purpura on dependent areas, arthritis or arthralgia, colicky abdominal pain, and renal involvement
• Applies the EULAR/PRINTO/PRES Ankara 2008 classification criteria of mandatory palpable purpura plus at least one of diffuse abdominal pain, IgA deposition on biopsy, arthritis or arthralgia, or renal involvement
• States that the long-term prognosis is determined by the renal involvement and that every child needs blood pressure and urinalysis monitoring for at least six months
• Explains the pathogenesis centred on galactose-deficient IgA1 immune complex deposition with complement activation causing leukocytoclastic vasculitis of small vessels in the skin, gut, joints and glomeruli
• Justifies the selective use of corticosteroids for severe gastrointestinal, joint, scrotal and renal disease, citing the evidence that prednisone does not reliably prevent nephropathy
• Coordinates the multidisciplinary care with nephrology for biopsy-proven nephritis, surgery for intussusception, and long-term renal surveillance using the European consensus SHARE recommendations
• The child with IgA vasculitis who develops severe abdominal pain, gastrointestinal bleeding, or intussusception, and the corticosteroid and surgical response
• The recognition of significant renal involvement (nephritic or nephrotic syndrome) and the threshold for biopsy, nephrology referral and immunosuppression
• The safety-netting of the family for the return features of abdominal pain, reduced urine, headache and swelling, and the blood pressure and urinalysis monitoring schedule
• Bedside assessment of the child with palpable purpura, distinguishing IgA vasculitis from meningococcaemia, sepsis, idiopathic thrombocytopenic purpura and other vasculitides
• Counselling the family on the typically excellent prognosis, the one-third relapse rate, and the critical importance of the renal monitoring schedule over the following six months
• Communication of the corticosteroid decision, balancing the relief of severe symptoms against the lack of a renal-preventive benefit, with clear safety-netting
• Level 1: Recognises the classic tetrad of palpable purpura, arthritis, abdominal pain and renal involvement, and identifies IgA vasculitis as the commonest childhood vasculitis
• Level 2: Applies the EULAR/PRINTO/PRES classification criteria, the corticosteroid indications, and the renal monitoring schedule with blood pressure and urinalysis
• Level 3: Leads the management of the severe complications including intussusception, nephrotic and nephritic syndrome, and the long-term renal surveillance
• The EULAR/PRINTO/PRES Ankara 2008 classification criteria for IgA vasculitis and the role of the skin biopsy showing IgA deposition
• The pathogenesis of galactose-deficient IgA1 immune complex deposition and leukocytoclastic vasculitis
• The indications for corticosteroids and the evidence that prednisone does not reliably prevent nephropathy
• Recognition of the palpable purpura on the lower legs and buttocks and the differentiation from meningococcaemia at the bedside
• Assessment and monitoring of renal involvement, including blood pressure, urinalysis and the indications for biopsy
• Communication with the family on the prognosis, the relapse risk and the importance of the follow-up monitoring
• IgA vasculitis as the commonest childhood vasculitis, presenting with the tetrad of palpable purpura, arthritis, abdominal pain and renal involvement
• The EULAR/PRINTO/PRES classification criteria and the role of the skin biopsy with IgA deposition in atypical cases
• The selective use of corticosteroids and the long-term renal monitoring for at least six months
• Recognition of the classic clinical tetrad and the differentiation from life-threatening mimics such as meningococcaemia and sepsis
• Application of the corticosteroid indications for severe gastrointestinal, joint, scrotal and renal disease
• Coordination of the renal monitoring schedule and the multidisciplinary referral for the severe complications
• Canadian approach to the diagnosis and classification of IgA vasculitis using the EULAR/PRINTO/PRES criteria
• The corticosteroid indications and the evidence-based approach to the renal monitoring
• The management of the severe complications including intussusception and nephritis, and the long-term follow-up