Paeds · rheumatology-musculoskeletal-and-sports

IgA vasculitis

Also known as Henoch-Schonlein purpura · HSP · IgA vasculitis · Henoch-Schonlein purpura nephritis · Anaphylactoid purpura

Fellowship guide to IgA vasculitis (Henoch-Schonlein purpura), the commonest childhood small-vessel vasculitis. Covers 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. Details the clinical tetrad, the pathogenesis centred on galactose-deficient IgA1 immune complex deposition with leukocytoclastic vasculitis, the differentiation from meningococcaemia and other mimics, and the selective corticosteroid indications for severe gastrointestinal, joint, scrotal and renal disease. Reproduces the evidence from the Ronkainen and Jauhola trials that prednisone does not reliably prevent nephropathy, the Cochrane review conclusions, and the renal monitoring schedule of blood pressure and urinalysis for at least six months. Addresses the intussusception, the scrotal involvement, the nephritic and nephrotic presentations, the relapse rate, and the long-term prognosis determined by the renal outcome.

high10 referencesUpdated 16 July 202631 min readVerification in progress

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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 clarifiedSevere 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 reductionNephrotic 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 monthsScrotal 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 clinicallyPrednisone 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 useA 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

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toddlerpreschoolschool-ageadolescentyoung-adult-transition

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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 involvementApplies 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 involvementStates 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 monthsExplains 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 glomeruliJustifies the selective use of corticosteroids for severe gastrointestinal, joint, scrotal and renal disease, citing the evidence that prednisone does not reliably prevent nephropathyCoordinates the multidisciplinary care with nephrology for biopsy-proven nephritis, surgery for intussusception, and long-term renal surveillance using the European consensus SHARE recommendationsThe child with IgA vasculitis who develops severe abdominal pain, gastrointestinal bleeding, or intussusception, and the corticosteroid and surgical responseThe recognition of significant renal involvement (nephritic or nephrotic syndrome) and the threshold for biopsy, nephrology referral and immunosuppressionThe safety-netting of the family for the return features of abdominal pain, reduced urine, headache and swelling, and the blood pressure and urinalysis monitoring scheduleBedside assessment of the child with palpable purpura, distinguishing IgA vasculitis from meningococcaemia, sepsis, idiopathic thrombocytopenic purpura and other vasculitidesCounselling 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 monthsCommunication of the corticosteroid decision, balancing the relief of severe symptoms against the lack of a renal-preventive benefit, with clear safety-nettingLevel 1: Recognises the classic tetrad of palpable purpura, arthritis, abdominal pain and renal involvement, and identifies IgA vasculitis as the commonest childhood vasculitisLevel 2: Applies the EULAR/PRINTO/PRES classification criteria, the corticosteroid indications, and the renal monitoring schedule with blood pressure and urinalysisLevel 3: Leads the management of the severe complications including intussusception, nephrotic and nephritic syndrome, and the long-term renal surveillanceThe EULAR/PRINTO/PRES Ankara 2008 classification criteria for IgA vasculitis and the role of the skin biopsy showing IgA depositionThe pathogenesis of galactose-deficient IgA1 immune complex deposition and leukocytoclastic vasculitisThe indications for corticosteroids and the evidence that prednisone does not reliably prevent nephropathyRecognition of the palpable purpura on the lower legs and buttocks and the differentiation from meningococcaemia at the bedsideAssessment and monitoring of renal involvement, including blood pressure, urinalysis and the indications for biopsyCommunication with the family on the prognosis, the relapse risk and the importance of the follow-up monitoringIgA vasculitis as the commonest childhood vasculitis, presenting with the tetrad of palpable purpura, arthritis, abdominal pain and renal involvementThe EULAR/PRINTO/PRES classification criteria and the role of the skin biopsy with IgA deposition in atypical casesThe selective use of corticosteroids and the long-term renal monitoring for at least six monthsRecognition of the classic clinical tetrad and the differentiation from life-threatening mimics such as meningococcaemia and sepsisApplication of the corticosteroid indications for severe gastrointestinal, joint, scrotal and renal diseaseCoordination of the renal monitoring schedule and the multidisciplinary referral for the severe complicationsCanadian approach to the diagnosis and classification of IgA vasculitis using the EULAR/PRINTO/PRES criteriaThe corticosteroid indications and the evidence-based approach to the renal monitoringThe management of the severe complications including intussusception and nephritis, and the long-term follow-up

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Target exams

RACP General PaediatricsRACP DWERACP DCEMRCPCH TheoryMRCPCH ClinicalABP General PediatricsRCPSC Pediatrics

Red flags

Life stages

toddlerpreschoolschool-ageadolescentyoung-adult-transition

Care settings

outpatientwarded-acuterural-remotetelehealth

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Overview & Definition

A five-year-old boy is brought in three days after a sore throat, with a raised purplish rash over his ankles and shins, swollen painful knees, and crampy abdominal pain. His mother is frightened by the rash. The rash is the clue. These are not bruises and not a clotting problem; they are palpable purpura, raised spots you can feel with your fingertip, sitting on the lower legs because gravity has dropped the leaking vessels lowest. Together with the joints and the belly, this is the classic face of IgA vasculitis, once called Henoch-Schonlein purpura, and it is the commonest vasculitis of childhood. [5][2]

IgA vasculitis is a small-vessel vasculitis driven by the deposition of IgA-dominated immune complexes in the walls of the capillaries, venules and arterioles of the skin, gut, joints and the kidneys. The four features that define it clinically are the palpable purpura on the dependent areas, the arthritis or arthralgia, the colicky abdominal pain, and the renal involvement. The rash is the constant feature and the one the diagnosis hangs on. The other three vary in their presence and their severity, and it is the renal involvement that determines the long-term outcome, which is why the management is built around detecting and watching it. [5][6]

The name changed in 2012, when the Chapel Hill Consensus Conference renamed Henoch-Schonlein purpura as IgA vasculitis, because the new name captures the pathogenesis. The disease is driven by immunoglobulin A, the antibody of the mucosal surfaces, and it is the same immunoglobulin A that drives IgA nephropathy, the commonest glomerulonephritis in the world. The two diseases share the mesangial IgA deposition and the galactose-deficient IgA1, and they sit at the two ends of a spectrum of IgA-driven disease, with IgA nephropathy confined to the kidney and IgA vasculitis spreading across the small vessels of the skin, the gut and the joints as well. [6][9]

[1] [10]

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You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References10Show ledger

[1]Ozen S, Pistorio A, Iusan SM, et al EULAR/PRINTO/PRES criteria for Henoch-Schonlein 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]Ruperto N, Ozen S, Pistorio A, et al EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation. Ann Rheum Dis, 2010.PMID 20388738
[3]Ronkainen J, Koskimies O, Ala-Houhala M, et al Early prednisone therapy in Henoch-Schonlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr, 2006.PMID 16887443
[4]Jauhola O, Ronkainen J, Koskimies O, et al Outcome of Henoch-Schonlein purpura 8 years after treatment with a placebo or prednisone at disease onset. Pediatr Nephrol, 2012.PMID 22311342
[5]McCarthy HJ, Tizard EJ Clinical practice: Diagnosis and management of Henoch-Schonlein purpura. Eur J Pediatr, 2010.PMID 20012647
[6]Davin JC, Coppo R Henoch-Schönlein purpura nephritis in children. Nat Rev Nephrol, 2014.PMID 25072122
[7]Chartapisak W, Opastirakul S, Hodson EM, et al Interventions for preventing and treating kidney disease in Henoch-Schonlein Purpura (HSP). Cochrane Database Syst Rev, 2009.PMID 19588365
[8]Chartapisak W, Opastiraku S, Willis NS, et al. Prevention and treatment of renal disease in Henoch-Schonlein purpura: a systematic review. Arch Dis Child, 2009.PMID 18701559
[9]Davin JC Henoch-Schonlein purpura nephritis: pathophysiology, treatment, and future strategy. Clin J Am Soc Nephrol, 2011.PMID 21393485
[10]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

IgA vasculitis

Also known as Henoch-Schonlein purpura · HSP · IgA vasculitis · Henoch-Schonlein purpura nephritis · Anaphylactoid purpura

high10 referencesUpdated 16 July 202631 min readVerification in progress

Practise this topic

Overview & Definition

[1]

[10]

Paediatrics Fellowship Pro

You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References10Show ledger

[1]Ozen S, Pistorio A, Iusan SM, et al EULAR/PRINTO/PRES criteria for Henoch-Schonlein 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]Ruperto N, Ozen S, Pistorio A, et al EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation. Ann Rheum Dis, 2010.PMID 20388738
[3]Ronkainen J, Koskimies O, Ala-Houhala M, et al Early prednisone therapy in Henoch-Schonlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr, 2006.PMID 16887443
[4]Jauhola O, Ronkainen J, Koskimies O, et al Outcome of Henoch-Schonlein purpura 8 years after treatment with a placebo or prednisone at disease onset. Pediatr Nephrol, 2012.PMID 22311342
[5]McCarthy HJ, Tizard EJ Clinical practice: Diagnosis and management of Henoch-Schonlein purpura. Eur J Pediatr, 2010.PMID 20012647
[6]Davin JC, Coppo R Henoch-Schönlein purpura nephritis in children. Nat Rev Nephrol, 2014.PMID 25072122
[7]Chartapisak W, Opastirakul S, Hodson EM, et al Interventions for preventing and treating kidney disease in Henoch-Schonlein Purpura (HSP). Cochrane Database Syst Rev, 2009.PMID 19588365
[8]Chartapisak W, Opastiraku S, Willis NS, et al. Prevention and treatment of renal disease in Henoch-Schonlein purpura: a systematic review. Arch Dis Child, 2009.PMID 18701559
[9]Davin JC Henoch-Schonlein purpura nephritis: pathophysiology, treatment, and future strategy. Clin J Am Soc Nephrol, 2011.PMID 21393485
[10]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