What are the clinical manifestations and management of IgA (Immunoglobulin A) vasculitis, also known as Henoch-Schönlein purpura?

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From the Research

IgA vasculitis, also known as Henoch-Schönlein purpura, typically presents with palpable purpura, arthralgia/arthritis, abdominal pain, and renal involvement, and its management is primarily supportive, with corticosteroids and immunosuppressive agents reserved for severe cases 1. The clinical manifestations of IgA vasculitis include:

  • Palpable purpura, which appears as raised, non-blanching purpuric lesions predominantly on the lower extremities and buttocks
  • Arthralgia/arthritis, which commonly affects the knees and ankles with swelling and pain
  • Abdominal pain, which can include colicky abdominal pain, vomiting, and occasionally gastrointestinal bleeding
  • Renal involvement, which ranges from microscopic hematuria to nephrotic syndrome and occurs in 30-50% of patients The disease is caused by IgA immune complex deposition in small vessel walls, triggering inflammation and vasculitis, and its severity and outcome depend on the extent of renal involvement 2. Management of IgA vasculitis is primarily supportive, including:
  • Adequate hydration
  • Rest
  • Pain control with acetaminophen or NSAIDs (unless contraindicated by renal involvement) Corticosteroids may be used for severe abdominal pain, significant joint symptoms, or scrotal involvement, typically prednisone at 1-2 mg/kg/day for 1-2 weeks with tapering 3. For patients with significant renal involvement (nephrotic-range proteinuria or declining renal function), more aggressive immunosuppression may be necessary, including high-dose corticosteroids, cyclophosphamide, azathioprine, or mycophenolate mofetil 4. Regular monitoring of blood pressure, urinalysis, and renal function is essential during the acute phase and for 6-12 months afterward, as renal complications may develop later 5.

References

Research

IgA vasculitis.

Seminars in immunopathology, 2021

Research

A Review of IgA Vasculitis (Henoch-Schönlein Purpura) Past, Present, and Future.

Medical science monitor : international medical journal of experimental and clinical research, 2024

Research

IgA vasculitis (Henoch-Schönlein purpura).

Dermatology online journal, 2015

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This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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