Effectiveness of a Novel PLA2R1 Knock-in Middle Age Rat Model in Repairing Renal Function Damage.

Phospholipase A2 receptor 1 (PLA2R1) exists important role in membranous nephropathy. In this study, we evaluate a PLA2R1 in a middle-aged rat model of renal function repair to further investigate the molecular mechanisms of membranous nephropathy. We analyzed the PLA2R1 knockout (KO) model and PLA2R1 knock in (KI) model in rats, extending the time to 85 weeks of age.

[IgA vasculitis with necrosis of the small intestine secondary to monoclonal gammopathy of renal significance: A case report].

Monoclonal gammopathy of undetermined significance combined with renal damage is named monoclonal gammopathy of renal significance. There are few reports about IgA vasculitis in patients with monoclonal gammopathy of undetermined significance. Here, we report a case of monoclonal gammopathy of renal significance, who had manifestations of IgA vasculitis, including purpura, gastrointestinal bleeding and joint pain.

Technology Innovation for Discovering Renal Autoantibodies in Autoimmune Conditions.

Autoimmune glomerulonephritis is a homogeneous area of renal pathology with clinical relevance in terms of its numerical impact and difficulties in its treatment. Systemic lupus erythematosus/lupus nephritis and membranous nephropathy are the two most frequent autoimmune conditions with clinical relevance. They are characterized by glomerular deposition of circulating autoantibodies that recognize glomerular antigens.

Analysis of the Sensitivity and Specificity of Histopathological Findings for Diagnosing Lupus Nephritis.

Background: Since the introduction of the SLICC criteria in 2012, biopsy-proven lupus nephritis (LN) has been the only independent diagnostic criterion for systemic lupus erythematosus (SLE). This was reaffirmed by the EULAR/ACR in 2019, emphasizing the importance of renal biopsy in LN. However, the current classification lacks specific histopathological criteria for defining LN.

A single-center retrospective study of ectopic lymphoid tissues in idiopathic membranous nephropathy: clinical pathological characteristics and prognostic value.

Background: In recent years, ectopic lymphoid tissue (ELT) has been increasingly confirmed as a new biomarker for kidney injury or inflammation. However, there is insufficient research on the relationship between ELT grading and the progression of idiopathic membranous nephropathy (IMN).

Methods: A total of 147 patients with biopsy-proven IMN in our institution from March 2020 to June 2022 were classified into five grades based on the different distribution of lymphocyte subsets in renal tissue (G0: no B cells or T cells, G1: scattered B and T cells, G2: clustered B and T cells, G3: an aggregation region of B and T cells without a central network, G4: highly organized and formed zones of B and T cells with a central network of follicular dendritic cells and scattered macrophages), and were further divided into low-grade group (G0+G1), intermediate-grade group (G2) and high-grade group (G3+G4).

Cyclosporine and fedratinib combination therapy via modulating Th17/Treg balance in Rat model of membranous glomerulonephritis.

A progressive kidney disease associated with inflammation and the immune system is called membrane glomerulonephritis (MGN). The present study investigatedthe combination of cyclosporine and fedratinib on Th17/regulatory T cells (Tregs) in rat models of MGN. Rats were given several doses of anti-Fx1A to induce MGN, and the resultant five groups of rats were fedratinib-cyclosporin receiving PHN rats, fedratinib, cyclosporin, and healthy rats.

NELL-1 Associated Membranous Nephropathy - A Case Series from India.

Neural epidermal growth factor-like 1 (NELL-1) is responsible for a substantial proportion of cases of PLA2R-negative primary membranous nephropathy (MN). To investigate the occurrence, association, and treatment responses, we carried out this ambispective study. In the study, nine (10%) of all patients with MN at our center were found to be NELL-1 positive.

Characteristics of glomerulonephritis with dominant C1q precipitation compared to corresponding glomerulonephritis without C1q staining on immunofluorescent examination.

Background: The clinical significance and renal outcomes of C1q nephropathy (C1qN) are unclear; therefore, the implications of C1qN as a new pathological entity are uncertain. We compared the clinical characteristics of glomerulonephritis reclassified into cases that meet the definition of C1qN and glomerulonephritis not included in the definition of C1qN.

Methods: In total, 21,697 patients who underwent native kidney biopsy at 18 hospitals throughout Korea between 1979 and 2018 were retrospectively enrolled.

Prognostic value of serum complement cleavage factor Bb in idiopathic membranous nephropathy and establishment of nomogram model.

Complement activation is involved in idiopathic membranous nephropathy (IMN). We aimed to investigate the relationship of serum complement cleavage factor Bb with IMN progression, and to establish a model for early prediction of kidney outcomes. We measured serum factor Bb in a retrospective cohort of 449 IMN patients at the time of kidney biopsy.

Updated diagnostic and therapeutic management for membranous nephropathy.

Purpose Of Review: Pioneering contributions in membranous nephropathy over the last decade have greatly enhanced our comprehension of its pathogenesis, diagnosis, and treatments, igniting renewed interest in this entity. This review provides an updated perspective on the diagnosis and therapeutic management of membranous nephropathy.

Recent Findings: The identification of antiphospholipase A2 receptor (PLA2R) antibodies in 50-80% of membranous nephropathy patients was a key breakthrough.

Animal models of membranous nephropathy: more choices and higher similarity.

Membranous nephropathy (MN) is an antibody-mediated autoimmune glomerular disease in which PLA2R1 is the main autoantibody. It has become the most common cause of adult nephrotic syndrome, and about one-third of patients can progress to end-stage kidney disease, but its pathogenesis is still unclear. Animal models can be used as suitable tools to study the pathogenesis and treatment of MN.

Clinicopathological Features of Membranous Nephropathy Complicated by IgA Nephropathy: A Retrospective Analysis of Seven Cases.

Both membranous nephropathy (MN) and immunoglobulin A nephropathy (IgAN) are immune complex-mediated glomerular diseases, but the concurrent occurrence of these two conditions in the same patient is not common, a phenomenon that is currently not supported by clinical data in terms of treatment and prognosis. This study explores the clinical and pathological characteristics, as well as the treatment outcomes, of patients affected by MN and IgAN simultaneously. The clinical data, pathological features, and diagnostic and therapeutic information of seven cases of MN complicated by IgAN, treated between December 2015 and December 2022, were retrospectively analyzed.

Epitope Spreading in Immune-Mediated Glomerulonephritis: The Expanding Target.

Epitope spreading is a critical mechanism driving the progression of autoimmune glomerulonephritis. This phenomenon, where immune responses broaden from a single epitope to encompass additional targets, contributes to the complexity and severity of diseases such as membranous nephropathy (MN), lupus nephritis (LN), and ANCA-associated vasculitis (AAV). In MN, intramolecular spreading within the phospholipase A2 receptor correlates with a worse prognosis, while LN exemplifies both intra- and intermolecular spreading, exacerbating renal involvement.