Clinical features of Iga Vasculitis

Clinical features of Iga Vasculitis

The classic tetrad of palpable purpura, arthritis, abdominal pain, and renal disease occurs in up to 80% of cases. The rash may begin as macular erythema and urticarial lesions, but may progress rapidly to purpura.

The lower extremities and buttocks are the most common sites for the rash. Scrotal and scalp edema can be seen, particularly in children. The joints are involved in 60% to 84% of patients.

The involvement is usually transient, oligoarticular, and most commonly involves the ankles and knees. It is typically nondeforming. GI lesions may cause severe cramping, abdominal pain, intussusception, hemorrhage, and rarely, ileal perforation (severe presentations such as intussusception are rare in adults). In cases of severe abdominal pain and concern for intussusception, ultrasound may be considered over barium enema as involvement in HSP is typically more proximal than idiopathic cases. Renal involvement is seen in 50% of patients and usually manifests as asymptomatic proteinuria and hematuria. However, more marked findings may occur, including nephrotic syndrome and acute renal failure. Most patients have complete resolution of renal manifestations with only a small subset showing persistent and/or progressive disease (5%–10% children and 10%–15% adults).

IgAV is often acute in onset, and resolution is rapid and complete in 97% of cases, except in a minority of patients (3%–5%) with chronic renal disease. Persons of any age can be affected, but IgAV occurs primarily in children aged 2 to 10 years. Adults have more severe disease with a higher frequency of progressive renal disease. Patients of any age (especially adults) suspected of having IgAV for whom a skin biopsy is negative for IgA should be evaluated for ANCA-associated vasculitis, anti-C1q disease, or IgA paraproteinemia.

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