ArticleKidney international reports2026
Systematic Review of IgA Nephropathy Coexisting With Alport Syndrome.
Article in Kidney international reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Introduction: In recent years, IgA nephropathy (IgAN) coexisting with Alport syndrome (AS) has been increasingly reported. However, the clinicopathological and molecular features, treatment, and prognosis of this entity have not been fully elucidated. Methods: We first retrospectively analyzed pediatric patients with IgAN coexisting with AS (IgAN-AS) admitted to our center, and performed galactose-deficient IgA1 (Gd-IgA1) immunostaining on renal biopsy specimens. We then systematically searched PubMed, Scopus, Web of Science, Wanfang Data, and the China National Knowledge Infrastructure for publications reporting on patients with IgAN-AS. Results: A total of 81 patients from 72 families were included. Microscopic hematuria was a universal finding. Nephrotic-range proteinuria was significantly more frequent in children than in adults (73.7% vs. 8.8%). Pathological findings confirmed the dual diagnosis, with mesangial IgA-dominant deposits and significant glomerular basement membrane abnormalities, including thinning (69.5%, 41/59) and typical AS-related changes (28.8%, 17/59). Genetic analysis identified 66 distinct pathogenic variants across Conclusion: IgAN-AS appears to be a heterogeneous condition with potentially unfavorable kidney outcomes. Some patients exhibited both immune and structural injury, suggesting that biomarkers with high sensitivity and specificity for IgAN may aid in disease stratification.
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