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For further information, see CMDT Part 20-25: IgA Vasculitis

For further information, see CMDT Part 22-14: Nephritic Spectrum Glomerular Diseases

Key Features

  • Formerly called Henoch-Schönlein purpura

  • Systemic small-vessel leukocytoclastic vasculitis associated with IgA subclass 1 deposition in vessel walls

  • The most common systemic vasculitis in children; often associated with an inciting infection, such as group A streptococcus

  • Occurs in adults as well

  • Classic presentation is with palpable purpura

Clinical Findings

  • Purpuric skin lesions typically located on the lower extremities; may also be seen on the hands, arms, trunk, and buttocks

  • Joint symptoms are present in most patients; the knees and ankles are most commonly involved

  • Abdominal pain secondary to vasculitis of the intestinal tract is often associated with gastrointestinal bleeding

  • Hematuria signals the presence of a glomerular lesion that is usually reversible, although it occasionally may progress to chronic kidney disease

  • A decrease in GFR is common with a nephritic presentation

  • Renal lesions can be identical to those found in IgA nephropathy

  • Most patients with microscopic hematuria and minimal proteinuria recover fully over several weeks

  • Progressive CKD and possibly ESRD are more likely to develop in those with the nephrotic syndrome; the presence of both nephritic and nephrotic syndrome features poses the worst renal prognosis

Diagnosis

  • Skin biopsy can demonstrate leukocytoclastic vasculitis with IgA deposition

  • Kidney biopsy reveals segmental glomerulonephritis with crescents and mesangial deposition of IgA

  • Differential diagnosis

    • Immune thrombocytopenia

    • Meningococcemia

    • Rocky Mountain spotted fever

    • Rheumatoid arthritis (including juvenile form)

    • Polyarteritis nodosa

    • Endocarditis

    • Cryoglobulinemia

Treatment

  • Chronic courses with persistent or intermittent skin disease are more likely to occur in adults than children

  • The efficacy of treatment is not well established

  • In children, prednisone (1–2 mg/kg/day orally) does not decrease the frequency of proteinuria 1 year after onset of disease

  • Severe disease is often treated with aggressive immunosuppressive agents such as mycophenolate mofetil, but there is no consensus as to the efficacy of this approach or the optimal therapeutic regimen

  • Rituximab treatment and plasma exchange have been successful for severe nephritic disease according to case reports, but clinical trials are lacking

  • Rapidly progressive renal disease with crescent formation on biopsy may be treated as in ANCA-associated vasculitis

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