Clinical Presentation, Pathological Spectrum, and Outcomes of Alcoholic Cirrhosis-Related Immunoglobulin A Nephropathy

Abstract

BackgroundIgA nephropathy associated with cirrhosis is frequent but often overlooked as largely considered silent. Until now, little has been known about their presentation and outcomes. MethodsWe conducted a retrospective multicenter study on patients with kidney biopsy-proven cirrhosis-related IgA nephropathy (cirrhosis-IgAN), diagnosed between 2009 and 2022. We mixed them up with 83 primary IgA nephropathy (pIgAN) diagnosed during the same period, using a partitioning clustering approach, to determine common clinicopathological profiles. ResultsAll the 46 patients with cirrhosis-related IgA nephropathy had an excessive alcoholic consumption. Clinical presentation was severe with acute kidney injury (AKI) in 79%, alternative causes of AKI was found in 62% of cases. Three clinicopathological clusters were identified: one represented chronic involvement, the second could be assimilated to mild disease, and the third corresponded to a MPGN pattern and was associated with heavy proteinuria and intrinsic AKI (without alternative causes). While the two first were equally distributed between pIgAN and cirrhosis-IgAN, the third was more frequent in patients with cirrhosis. The cumulative mortality rate in cirrhosis-IgAN was 26% and 46% at 1-year and 3-years. Steroid exposure and moderate/severe AKI were associated with higher mortality and steroid exposure was associated with the occurrence of severe infection. ConclusionOur results suggest that high AKI incidence is related to extrinsic causes in most cases but can also be driven by IgA-dominant MPGN in a subset of patients. Steroid use was associated with infectious disease and mortality. Further studies are needed to clarify the place of immunosuppressive treatment in cirrhosis-IgAN patients.