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Review
. 2024 Oct 4;12(10):2259.
doi: 10.3390/biomedicines12102259.

An Updated Comprehensive Review on Diseases Associated with Nephrotic Syndromes

Ralph Wendt  1 , Alina Sobhani  2 , Paul Diefenhardt  2   3 , Moritz Trappe  2 , Linus Alexander Völker  2   3
Affiliations
Review

An Updated Comprehensive Review on Diseases Associated with Nephrotic Syndromes

Ralph Wendt et al. Biomedicines. .

Abstract

There have been exciting advances in our knowledge of primary glomerular diseases and nephrotic syndromes in recent years. Beyond the histological pattern from renal biopsy, more precise phenotyping of the diseases and the use of modern nephrogenetics helps to improve treatment decisions and sometimes also avoid unnecessary exposure to potentially toxic immunosuppression. New biomarkers have led to easier and more accurate diagnoses and more targeted therapeutic decisions. The treatment landscape is becoming wider with a pipeline of promising new therapeutic agents with more sophisticated approaches. This review focuses on all aspects of entities that are associated with nephrotic syndromes with updated information on recent advances in each field. This includes podocytopathies (focal segmental glomerulosclerosis and minimal-change disease), membranous nephropathy, membranoproliferative glomerulonephritis, IgA nephropathy, fibrillary glomerulonephritis, amyloidosis, and monoclonal gammopathy of renal significance in the context of the nephrotic syndrome, but also renal involvement in systemic diseases, diabetic nephropathy, and drugs that are associated with nephrotic syndromes.

Keywords: DKD; FSGS; IgAN; MGRS; MPGN; PLA2R; edema; membranous nephropathy; minimal change disease; nephrin; nephrotic syndrome; proteinuria.

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Conflict of interest statement

All authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Pathophysiology of IgAN. (Adapted from [78]).
Figure 2
Figure 2
Pathophysiology of IgAN targeted by current and new drugs (adapted from [78]).
Figure 3
Figure 3
MPGN classification, adapted from [106,107] A-CP, alternative complement pathway; C-MPGN, complement-mediated MPGN; C3GN, C3 glomerulonephritis; DDD, dense deposit disease; HUS, hemolytic uremic syndrome; I-MPGN, immunoglobulin (Ig)-/immune complex-mediated MPGN; MPGN, membranoproliferative glomerulonephritis; TMA, thrombotic microangiopathy.
Figure 4
Figure 4
Evaluation of FSGS lesions on kidney biopsy. GT: Genetic testing.
See this image and copyright information in PMC

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