PassFinals
Paediatrics

IgA Vasculitis

IgA immune-complex small-vessel vasculitis causes palpable purpura with variable joint, gastrointestinal and renal disease; the visible rash usually settles, but renal surveillance determines long-term safety.

In a nutshell

IgA vasculitis is an IgA small-vessel vasculitis causing palpable purpura plus variable joint, bowel and kidney involvement. Most children need supportive care, but severe abdominal pain needs active exclusion of intussusception and renal disease needs structured urine/blood-pressure surveillance for at least six months.

Classic presentation

A child after an infection with palpable purpura on the legs and buttocks, ankle or knee pain, possible colicky abdominal pain and normal platelets.

Key points

  • Purpura is palpable and usually lower-limb/buttock predominant; normal platelets support vasculitis rather than thrombocytopenia.
  • Always assess skin, joints, gut, scrotum and kidneys, and screen for sepsis when a child has a non-blanching rash.
  • Severe or unremitting abdominal pain, GI bleeding, vomiting, distension or a mass requires hospital and surgical/radiological assessment for intussusception.
  • Urinalysis is required at presentation and repeatedly for six months; normal initial urine does not exclude later nephritis.
  • Biopsy is a specialist decision for severe nephritis, including persistent heavy/moderate proteinuria, AKI or nephrotic syndrome.
  • Corticosteroids are not routine prophylaxis; specialist early treatment may be considered for severe GI disease after other causes, especially intussusception, are excluded.
  • Painful scrotal swelling needs urgent assessment because torsion can coexist with or mimic vasculitic involvement.

First-line investigation

Urinalysis, urine protein quantification when abnormal, blood pressure, renal function and FBC/platelets, with targeted imaging or specialist tests for complications.

Management

Triage the non-blanching rash and organ threat

  • Assess for sepsis, hydration, severe GI disease, nephritis, scrotal emergency, neurological disease and pulmonary haemorrhage before accepting uncomplicated IgA vasculitis.1,3,2

Confirm the clinical pattern and baseline renal status

  • Use palpable lower-limb-predominant purpura with normal platelets and compatible joint, bowel or renal features; record urine, proteinuria, blood pressure and renal function.1,2

Treat uncomplicated disease safely

  • Use rest, hydration and appropriate analgesia; avoid unsupervised ibuprofen when kidney involvement or dehydration is possible and check current BNF/BNFC advice.2,5

Escalate abdominal, scrotal and renal complications

  • Seek urgent specialist advice for severe GI symptoms, suspected intussusception, acute scrotal swelling, heavy/persistent proteinuria, AKI, nephrotic/nephritic syndrome or hypertension; use specialist corticosteroid and biopsy pathways.3,4,5

Continue renal surveillance after the rash resolves

  • Arrange frequent urine surveillance for six months, with blood pressure and renal-function follow-up according to findings, and extend nephrology follow-up for persistent abnormalities.1,2

Exam traps

  • Normal platelets are expected; IgA vasculitis is not ITP.
  • The rash disappearing does not end follow-up: nephritis can be delayed and silent.
  • Do not give corticosteroids for routine uncomplicated disease or before excluding intussusception in severe abdominal pain.
  • Do not label painful scrotal swelling as vasculitis until testicular torsion has been assessed.
  • Persistent proteinuria, AKI, nephrotic/nephritic syndrome or hypertension is a paediatric-nephrology pathway, not routine reassurance.

Illustrations

IgA immune complex depositionDiagram showing IgA immune complexes depositing in small vessel walls of skin, joints, gut and glomeruli, mapping each organ's involvement to the same underlying process.PassFinals · original
Palpable purpura distributionIllustration of a child showing the classic symmetrical purpuric rash over the extensor surfaces of the legs and buttocks.PassFinals · original

Key sources

  1. UK Kidney Association: Clinical Practice Guidelines for the Initial Management of IgA Vasculitis in children and young people (UK national guideline endorsed by RCPCH, final version December 2022 and published July 2023; review date December 2027.)Updated 11 Jul 2023
  2. NHS: Henoch-Schönlein purpura (HSP) (Current NHS information on symptoms, red flags, supportive care and 6-12 month kidney follow-up.)Updated 18 Oct 2023
  3. UK Kidney Association: Clinical Practice Guidelines for complications associated with IgA Vasculitis in children and young people (UK national guideline endorsed by RCPCH for renal, gastrointestinal, urological, skin and atypical complications.)Updated 11 Jul 2023
  4. Archives of Disease in Childhood: National recommendations for the management of children and young people with IgA vasculitis (UK national recommendations using best available evidence and group agreement, published online December 2024.)Updated 13 Dec 2024
  5. BNF online (Current UK prescribing information for analgesics, corticosteroids, ACE inhibitors, ARBs and immunosuppressive medicines.)

This page is exam revision material, not medical advice, and must not be used for patient care. Always check drug doses against the BNF and current guidance. Full disclaimer.