About IgA Vasculitis with Nephritis
IgA vasculitis (formerly Henoch-Schönlein purpura) is a small-vessel immune complex vasculitis characterized by predominant IgA deposition in involved organs (kidneys, skin, GI tract). The condition presents with characteristic tetrad of palpable purpura (small red spots on lower extremities and buttocks), arthritis/arthralgias, abdominal pain, and glomerulonephritis, though not all features present in every patient.
IgA-dominant glomerulonephritis develops in approximately 40-50% of patients with systemic disease; when kidney biopsy performed in all patients, IgA deposition found in ~100%. The glomerulonephritis ranges from microscopic hematuria and mild proteinuria to rapidly progressive glomerulonephritis with crescents requiring urgent intervention. About 1-5% of children progress to end-stage renal disease requiring dialysis or transplantation, while progression rates are significantly higher in adults (5-10% at 5 years). GI manifestations range from mild abdominal pain to life-threatening gastrointestinal bleeding requiring transfusion and endoscopic intervention.
Secondary complications including HUS (hemolytic uremic syndrome) and pulmonary hemorrhage occur occasionally. Elevated serum IgA level is characteristic but not diagnostic, as elevated IgA occurs in other conditions. Most children achieve complete remission of systemic disease, though renal involvement duration and severity predict persistent or progressive renal disease.
Common Symptoms of IgA Vasculitis with Nephritis
Recognizing the signs of IgA Vasculitis with Nephritis early can lead to faster diagnosis and better outcomes. Symptoms may vary in severity from person to person. If you or a loved one are experiencing any of the following, consider speaking with a specialist.
- Palpable purpura on lower extremities and buttocks
- Arthritis and arthralgias
- Glomerulonephritis with hematuria and proteinuria
- Abdominal pain and GI bleeding
- Renal dysfunction and hypertension
- Elevated serum IgA levels
Who IgA Vasculitis with Nephritis Affects
Most common in children ages 3-10 years with peak incidence 4-6 years, though can occur at any age from infancy to elderly. Slight male predominance at approximately 1.5:1 to 2:1 male-to-female ratio. Significantly higher prevalence in East Asian populations (particularly Japan, Korea, China) and Mediterranean populations compared to Northern European and African populations.
Estimated prevalence varies 5-10 fold between populations. Seasonal variation with increased incidence in autumn and winter correlating with upper respiratory infections. Often preceded by upper respiratory infection or other infectious trigger 1-2 weeks prior to symptom onset. Recurrent or persistent hematuria occurs in approximately 40% of patients even after systemic disease resolution.
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