Is IgA nephropathy the same disease in different parts of the world?

Abstract

Since it was first described in 1968, immunoglobulin A nephropathy (IgAN) is understood to be the most common form of glomerulonephritis worldwide. The diagnosis of IgAN depends on the presence of dominant mesangial IgA1 deposition by renal biopsy. To date, a wide spectrum of clinical and pathologic features of IgAN have been observed, implying that IgAN might not be the same disease across the world. Here, we review the characteristics of IgAN from perspectives of epidemiology, clinical-pathological patterns, disease pathogenesis, and treatment response across different ethnic populations. Overall, IgAN is most prevalent in Asians, followed by Caucasians, and relatively rare in Africans. More severe clinical presentation and higher risk of disease progression have been reported in Asians than Europeans. Moreover, active lesions, such as endocapillary hypercellularity and crescents, are more commonly reported in Asians than Europeans. Response to corticosteroid/immunosuppression therapy is variably reported, with greater apparent efficacy reported in Asian than European studies. Although a multi-hit hypothesis has been suggested for IgAN, the relative importance of each "hit" may vary in different ethnic populations and this variation underlies the differences in presentation of IgAN. In the future, a better understanding of pathogenic pathways operating in different ethnic populations may help provide better biomarkers of disease and more precise targeting of treatment strategies for IgAN.