B-cell Pathophysiology

The immune origin of idiopathic nephrotic syndrome was first proposed by Shaloub in 1974, with T-cells as the dominant hypothesis for decades. The therapeutic effectiveness of rituximab shifted understanding of nephrotic syndrome pathophys…

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The immune origin of idiopathic nephrotic syndrome was first proposed by Shaloub in 1974, with T-cells as the dominant hypothesis for decades. The therapeutic effectiveness of rituximab shifted understanding of nephrotic syndrome pathophysiology toward a key role for B-cells. The identification of anti-nephrin autoantibodies, immunoglobulin immunoadsorption efficacy, and B-cell dysregulation collectively support a humoral autoimmune aetiology for nephrotic syndrome. Memory B-cells (CD19+/CD27+) have been identified as having a specific contributory role in idiopathic nephrotic syndrome.