The successful use of rituximab in crescentic <scp>IgA</scp> nephropathy with concurrent <scp>ANCA</scp> positivity

IgA nephropathy (IgAN) is the most common primary glomerulonephritis worldwide and it is characterized by mesangial IgA deposition. Asymptomatic hematuria with various degrees of proteinuria is the most common clinical presentation and up to 20-40% of patients develop end-stage kidney disease (ESKD) within 20 years after disease onset. The pathogenesis of IgAN involves four sequential processes known as the “four-hit hypothesis” that starts with the production of a galactose-deficient IgA1 (gd-IgA1), followed by the formation of anti-gd-IgA1 IgG or IgA1 autoantibodies and immune complexes that ultimately deposit in the glomerular mesangium, leading to inflammation and injury. Although several key questions about the production of gd-IgA1 and the formation of anti-gd-IgA1 antibodies remain unanswered, a growing body of evidence is shedding light on the innate and adaptive immune mechanisms involved in this complex pathogenic process. Herein, we will focus on these mechanisms that, along with genetic and environmental factors, are thought to play a key role in disease pathogenesis.

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Immune abnormalities in IgA nephropathy

Gentile

Sanchez-Russo

Riella

et al. 2023

Clinical Kidney Journal

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The New Progress of Treatment in IgA Nephropathy

张¹

2022

ACM

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The successful use of rituximab in crescentic IgA nephropathy with concurrent ANCA positivity

Cited by 2 publications

References 3 publications

Immune abnormalities in IgA nephropathy

Immune abnormalities in IgA nephropathy

The New Progress of Treatment in IgA Nephropathy

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