Which vasculitis involves the glomeruli causing nephrotic syndrome and also produces abdominal pain in a child with nephrotic syndrome?

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IgA Vasculitis (Henoch-Schönlein Purpura)

The vasculitis you're describing is IgA vasculitis (formerly Henoch-Schönlein purpura), which characteristically affects the glomeruli causing nephrotic syndrome and produces abdominal pain through gastrointestinal involvement in children. 1, 2

Clinical Presentation

IgA vasculitis is a systemic, immune complex-mediated, small-vessel leukocytoclastic vasculitis that presents with:

  • Glomerular involvement: Can range from microscopic hematuria to full nephrotic syndrome with proteinuria, hypoalbuminemia, and edema 1, 3
  • Gastrointestinal manifestations: Intense abdominal pain, bloody diarrhea, and protein-losing enteropathy 1
  • Classic purpura: Palpable, non-thrombocytopenic purpura (though notably, purpura may be absent in atypical presentations) 1
  • Joint involvement: Arthritis commonly occurs

This is the most common vasculitis in children 2, 4, and IgA vasculitis-associated nephritis (IgAVN) specifically describes the renal manifestation.

Diagnostic Approach

When nephrotic syndrome develops in a child with IgA vasculitis, kidney biopsy is indicated to assess severity and guide treatment decisions. 1, 3 The renal histology shows IgA-positive mesangial proliferative glomerulonephritis, which confirms the diagnosis 1, 5.

Key diagnostic features include:

  • IgA deposition on immunofluorescence
  • Mesangial proliferation with possible crescent formation
  • Galactose-deficient IgA1 staining is specifically positive 5

GI endoscopy may reveal duodenitis with hemorrhagic spots when abdominal symptoms are prominent 1.

Management Strategy

For Nephrotic State Without Kidney Injury

Approximately half of children with IgAVN presenting with nephrotic state (serum albumin <3.0 g/dL and proteinuria >2.0 g/gCr) achieve spontaneous resolution within 3 months without treatment. 3

Consider watchful waiting initially if:

  • Serum albumin remains >2.0 g/dL
  • Serum albumin shows quick upward trend
  • No evidence of kidney injury (preserved eGFR)

For Severe Disease or Lack of Spontaneous Improvement

High-dose corticosteroids are the primary treatment for IgAVN with nephrotic syndrome or severe GI complications. 1, 2, 5

The treatment approach includes:

  • Corticosteroids: Prednisone or prednisolone as first-line therapy 1, 5
  • Additional agents for severe nephritis: Cyclosporine and mycophenolate have demonstrated success in randomized trials for treating glomerulonephritis complications 2
  • Supportive care: Factor XIII supplementation may help with abdominal symptoms 5
  • Renin-angiotensin system blockade: For proteinuria reduction 5

Important Caveats

Steroids should NOT be used prophylactically - systematic reviews demonstrate they do not prevent complications when given prophylactically 2. Reserve corticosteroids for established disease with significant organ involvement.

Prognosis and Follow-up

IgA vasculitis spontaneously resolves in 94% of children 2, but a subset develops persistent renal involvement that can relapse years later. Six months of follow-up is essential to assess for disease relapse or remission 2, with particular attention to:

  • Urinalysis for proteinuria and hematuria
  • Serum albumin trends
  • Blood pressure monitoring
  • Renal function assessment

Long-term prognosis depends primarily on the extent of renal involvement 2. Children with severe hypoalbuminemia (albumin <2.0 g/dL) or prolonged nephrotic state warrant more aggressive monitoring and earlier consideration of immunosuppressive therapy 3.

References

1
Research

Henoch-Schönlein without Purpura: A Case Report and Review Literature.

Journal of the Medical Association of Thailand = Chotmaihet thangphaet, 2016

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