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Our understanding of the clinical aspects of IgA nephropathy (IgAN) has advanced since the 10th International Symposium on IgA Nephropathy in 2004. In this review we discuss new developments in areas of familial IgAN, the emerging field of biomarkers, and prognostic features. Familial disease continues to account for a significant number of newly diagnosed patients with IgAN. These patients have clinical manifestations and long-term outcomes similar to those of patients with sporadic disease. Characterization of the IgAN1 gene linked to IgAN in some Italian and American multiplex families has remained elusive. Other multiplex IgAN pedigrees have shown no linkage to any locus. With advances in technology to better measure and characterize polypeptides in small concentrations, the area of biomarkers has generated substantial interest since 2004. New potential disease-specific biomarkers of IgAN include the IgA1 neoepitope at the threonine228 and/or serine230IgA1 hinge-region residues, serum levels of galactose-deficient IgA, and urinary IgA-IgG immune complexes. Other investigators have used proteomic approaches to find panels of urinary polypeptides (many of which have not been sequenced) that discriminate patients with IgAN from normal healthy controls as well as patients with various other proteinuric renal diseases. These or other related findings may provide the necessary tools to better classify phenotypes in multiplex pedigrees and to improve monitoring of disease progression or response to therapy.
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http://dx.doi.org/10.1159/000102284 | DOI Listing |
Front Immunol
December 2024
Department of Nephrology, First Teaching Hospital of Tianjin University of Traditional Chinese Medicine, National Clinical Research Center for Chinese Medicine Acupuncture and Moxibustion, Tianjin, China.
Nephrotic syndrome (NS) represents a prevalent syndrome among various chronic kidney disease pathologies and is known for its higher severity and worse prognosis compared with chronic glomerulonephritis. Understanding its pathogenesis and identifying more effective treatment modalities have long been a concern of kidney specialists. With the introduction of the gut-kidney axis concept and the progress in omics technologies, alterations in the gut microbiota have been observed in primary and secondary NS.
View Article and Find Full Text PDFThe combination of nephrotic syndrome with mild histopathological lesions of IgA nephropathy is considered by some as a special form of IgA nephropathy with superimposed minimal change disease (MCD) while by others as coincidental deposition of IgA in patients with MCD (MCD-IgAN). We present the first case of complete remission of nephrotic syndrome in a 55-year-old man with MCD-IgAN after the administration of targeted-release formulation of budesonide (TRF-budesonide). The patient's treatment with TRF-budesonide, even though methylprednisolone, mycophenolate mofetil, and cyclophosphamide had been previously tried, is of particular importance because it not only suggests that TRF-budesonide appears to be a promising treatment for MCD-IgAN but may also provide a new therapeutic option for patients with podocytopathies.
View Article and Find Full Text PDFClin J Am Soc Nephrol
December 2024
Department of Biomedical engineering, Emory University, Atlanta, GA, USA.
Background: Interstitial fibrosis and tubular atrophy (IFTA), and density and shape of peritubular capillaries (PTCs), are independently prognostic of disease progression. This study aimed to identify novel digital biomarkers of disease progression and assess the clinical relevance of the interplay between a variety of PTC characteristics and their microenvironment in glomerular diseases.
Methods: A total of 344 NEPTUNE/CureGN participants were included: 112 minimal change disease, 134 focal segmental glomerulosclerosis, 61 membranous nephropathy, and 37 IgA nephropathy.
Rheumatology (Oxford)
December 2024
AP-HP, Université Paris Saclay, department of internal medicine and clinical immunology, Bicêtre Hospital, Le Kremlin Bicêtre, France.
Objective: To describe presentation, treatment and outcome of immune checkpoint inhibitor (ICI) associated-vasculitis in cancer patients in a multicentre study.
Methods: Thanks to the ImmunoCancer International Registry (ICIR), a multidisciplinary network focused on the research of the immune related adverse events related to cancer immunotherapies, patients presenting with a clinical and/or radiological suspicion of vasculitis, and histological evidence of vasculitis after being exposed to ICIs were retrospectively identified.
Results: Twenty eight cases were identified in the ICIR registry.
Tissue Cell
December 2024
School of Pharmacy & Technology Management, SVKM's Narsee Monjee Institute of Management Studies (NMIMS), Polepally SEZ, TSIIC, Jadcherla, Mahbubnagar, Hyderabad 509301, India.
In this study, we investigated the efficacy of oxymatrine, a phytochemical alkaloid, in reducing inflammation and fibrosis in a rat model of IgA nephropathy (IgAN) through modulation of the TGF-β/SMAD signaling pathway. Thirty Sprague Dawley rats were randomized into control, IgAN, and treatment groups, the latter receiving oxymatrine postinduction of IgAN. Induced by bovine serum albumin, carbon tetrachloride, and lipopolysaccharides, the disease model was validated by immunofluorescence and histopathological analyses, confirming significant renal deposition of IgA and increased fibrosis markers (IL-6, TGF-β, SMAD 3, and α-SMA).
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