Anti-glomerular Basement Membrane Research Articles

Renal transplantation in Alport syndrome.

Kidney transplantation is recognized as an effective treatment for end-stage renal disease in Alport syndrome, demonstrating outcomes comparable to or even superior to those in other causes of renal failure. When considering living related donor kidney transplantation for Alport syndrome patients, it is crucial to consider genetic factors during the donor selection process. In addition to a comprehensive health check, genetic testing is strongly recommended for potential donors at risk of carrying mutations in COL4A3-COL4A5 before undergoing kidney transplantation. Individuals carrying these mutations face an inherent risk of kidney disease and due to the possibility of further deterioration in renal function after nephrectomy for transplantation, they are not suitable as priority donors. Posttransplant anti-glomerular basement membrane nephritis is rare but can lead to graft loss, especially in males with X-linked Alport syndrome.

Vascularites immunologiques des petits et moyens vaisseaux : physiopathologie et diagnostic biologique

AbstractVasculitis is defined by inflammation of the vascular endothelium, it is rare and difficult to diagnose. This article will only discuss primary non-infectious vasculitis, as is done in the international nomenclature of Chapel Hill, the second version of which aimed to replace the eponymous names of diseases with expressions related to their pathophysiological mechanism or their histological characteristics. This nomenclature of vasculitis takes into account the size of the vessels and the pathophysiological mechanisms which, in the involvement of small and medium-sized vessels, are immunological mechanisms. These are first, vasculitis with immune complex deposition, including cryoglobulinemic vasculitis, vasculitis with IgA deposits, hypocomplementemic urticarial vasculitis (McDuffie’s disease) and vasculitis with anti-glomerular basement membrane Abs (Goodpasture’s disease), and second, vasculitis with anti-neutrophil cytoplasmic Abs (ANCA), including granulomatosis with polyangiitis (formerly Wegener’s syndrome), eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) and microscopic polyangiitis. In this article, the pathophysiological mechanisms of these immunological vasculitides and the biological diagnostic tools will be developed, allowing biologists to participate in the diagnosis of these rare and frequently severe diseases.

ANCA and Anti-glomerular Basement Membrane (GBM) Dual Antibody Positivity: A Case Series and Proposal for a Clinical Classification

ANCA and Anti-glomerular Basement Membrane (GBM) Dual Antibody Positivity: A Case Series and Proposal for a Clinical Classification

Rapidly Progressive Glomerulonephritis (RPGN) Due to Myeloperoxidase Anti-neutrophil Cytoplasmic Antibodies (MPO-ANCA)-Associated Vasculitis and Anti-glomerular Basement Membrane (GBM) Disease

Rapidly Progressive Glomerulonephritis (RPGN) Due to Myeloperoxidase Anti-neutrophil Cytoplasmic Antibodies (MPO-ANCA)-Associated Vasculitis and Anti-glomerular Basement Membrane (GBM) Disease

Efficacy and Safety of Rituximab in Anti-glomerular Basement Membrane (GBM) Disease

Efficacy and Safety of Rituximab in Anti-glomerular Basement Membrane (GBM) Disease

A Rare Case of Concurrent IgA Nephropathy and Anti-glomerular Basement Membrane (GBM) Disease

A Rare Case of Concurrent IgA Nephropathy and Anti-glomerular Basement Membrane (GBM) Disease

Diffuse Alveolar Hemorrhage (DAH) Secondary to Influenza B in a Kidney Transplant Recipient with Anti-glomerular Basement Membrane (Anti-GBM) Disease

Diffuse Alveolar Hemorrhage (DAH) Secondary to Influenza B in a Kidney Transplant Recipient with Anti-glomerular Basement Membrane (Anti-GBM) Disease

First Reported Case of Rapidly Progressive Glomerulonephritis (RPGN) Due to Combined Renal-Limited ANCA-Associated Vasculitis and Anti-glomerular Basement Membrane (GBM) Disease in a Young Man with Koolen-de Vries Syndrome

First Reported Case of Rapidly Progressive Glomerulonephritis (RPGN) Due to Combined Renal-Limited ANCA-Associated Vasculitis and Anti-glomerular Basement Membrane (GBM) Disease in a Young Man with Koolen-de Vries Syndrome

Crescentic Glomerulonephritis (GN) with Anti-glomerular Basement Membrane (GBM) Antibodies and ANCA: Double Trouble

Crescentic Glomerulonephritis (GN) with Anti-glomerular Basement Membrane (GBM) Antibodies and ANCA: Double Trouble

Case Series of Avacopan in Dual-Positive Anti-glomerular Basement Membrane (GBM) and ANCA Disease

Case Series of Avacopan in Dual-Positive Anti-glomerular Basement Membrane (GBM) and ANCA Disease

Rituximab in Addition to Plasma Exchange, Cyclophosphamide, and Steroids for the Treatment of Anti-glomerular Basement Membrane (GBM) Disease

Rituximab in Addition to Plasma Exchange, Cyclophosphamide, and Steroids for the Treatment of Anti-glomerular Basement Membrane (GBM) Disease

Case of Crescentic Fibrillary Glomerulonephritis with Linear IgG Mimicking Anti-glomerular Basement Membrane Nephritis with Good Response to Treatment

Case of Crescentic Fibrillary Glomerulonephritis with Linear IgG Mimicking Anti-glomerular Basement Membrane Nephritis with Good Response to Treatment

Atypical Anti-glomerular Basement Membrane Disease: An Emerging Clinical Dilemma

Atypical Anti-glomerular Basement Membrane Disease: An Emerging Clinical Dilemma

Double Trouble in the Basement! A Case of Phospholipase A2 Receptor (PLA2R)-Associated Membranous Nephropathy with Atypical Anti-glomerular Basement Membrane Disease

Double Trouble in the Basement! A Case of Phospholipase A2 Receptor (PLA2R)-Associated Membranous Nephropathy with Atypical Anti-glomerular Basement Membrane Disease

Perlecan Is a Novel Target of Autoantibodies in Anti-glomerular Basement Membrane Disease

Perlecan Is a Novel Target of Autoantibodies in Anti-glomerular Basement Membrane Disease

Outcomes after Kidney Transplantation in Anti-glomerular Basement Membrane Disease

Outcomes after Kidney Transplantation in Anti-glomerular Basement Membrane Disease

Single-cell RNA sequencing data locate ALDH1A2-mediated retinoic acid synthetic pathway to glomerular parietal epithelial cells.

Aldehyde dehydrogenase 1, family member A2, is a retinoic acid-synthesizing enzyme encoded by Aldh1a2 in mice and ALDH1A2 in humans. This enzyme is indispensable for kidney development, but its role in kidney physiology and pathophysiology remains to be fully defined. In this review, we mined single-cell and single-nucleus RNA sequencing databases of mouse and human kidneys and found that glomerular parietal epithelial cells (PECs) express a full set of genes encoding proteins needed for cellular vitamin A uptake, intracellular transport, and metabolism into retinoic acid. In particular, Aldh1a2/ALDH1A2 mRNAs are selectively enriched in mouse and human PECs. Aldh1a2 expression in PECs is greatly increased in a mouse model of anti-glomerular basement membrane glomerulonephritis and moderately induced in a mouse model of ischemia-reperfusion acute kidney injury. Aldh1a2 expression in PECs is substantially repressed in a chronic kidney disease mouse model combining diabetes, hypertension, and partial nephrectomy and is moderately repressed in mouse models of focal segmental glomerulosclerosis and diabetic nephropathy. Single-nucleus RNA sequencing data show that ALDH1A2 mRNA expression in PECs is diminished in patients with chronic kidney disease associated with diabetes, hypertension and polycystic kidney disease. In addition to data mining, we also performed Spearman's rank correlation coefficient analyses and identified gene transcripts correlated with Aldh1a2/ALDH1A2 transcripts in mouse PECs and PEC subtypes, and in human PECs of healthy subjects and patients with AKI or CKD. Furthermore, we conducted Gene Ontology pathway analyses and identified the biological pathways enriched among these Aldh1a2/ALDH1A2-correlated genes. Our data mining and analyses led us to hypothesize that ALDH1A2-mediated retinoic acid synthesis in PECs plays a yet-undefined role in the kidney and that its dysregulation mediates injury. Conditional, PEC-selective Aldh1a2 knockout, RNA silencing and transgenic mouse models will be useful tools to test this hypothesis. Clinical studies on genetics, epigenetics, expression and functions of ALDH1A2 and other genes needed for retinoic acid biosynthesis and signaling are also warranted.

Atypical Anti-GBM with ANCA Vasculitis- A Rarest of the Rare Entity: Index Case from Eastern India

Anti-glomerular basement membrane (GBM) antibody glomerulonephritis is an extremely rare glomerular disease. Around 90% of the patients are positive for serum anti-GBM antibodies while up to 10% can be negative. In such patients, only a kidney biopsy can reveal the anti-GBM disease it is then labeled as an atypical anti-GBM disease. Though anti-GBM disease can be associated with Anti Neutrophil Cytoplasmic Antibodies (ANCA) positivity, it is extremely rare to find atypical anti-GBM with ANCA positivity so much so that till now there are very few such cases reported from across the world. The case presented here is one such case where the patient presented with adult-onset nephrotic syndrome features with active urinary sediments and mildly deranged renal function. Myeloperoxidase (MPO) ANCA was positive and it was considered ANCA-associated crescentic glomerulonephritis (GN) but after the renal biopsy the picture was of anti-GBM disease. She was treated with pulse methylprednisolone but her creatinine increased in the meantime and considering anti-GBM she was put on Plasma Exchange (PLEX). She received 5 sessions of PLEX after which her renal function improved. She was also planned for Rituximab which could not be given due to local infection. As there are no protocols for treating such cases because of the extremely rare nature of the presentation, this case will increase the understanding of such presentations for the clinicians. This will help to plan for building the approach for such cases.

Effective management of necrotizing crescentic glomerulonephritis using an aggressive combination therapy including avacopan in a patient double-seropositive for anti-GBM antibodies and ANCA: a case report.

The prognosis of anti-glomerular basement membrane (anti-GBM) nephritis, often accompanied by the presence of antineutrophil cytoplasmic antibodies (ANCA), is poor, and even with aggressive therapeutic approaches, kidney replacement therapy (KRT) is typically required. Here, we present a case of necrotizing crescentic glomerulonephritis in a patient double-seropositive for anti-GBM antibodies and ANCA who successfully achieved dialysis independence following aggressive treatment, including avacopan. The patient was a 77-year-old woman with rapidly progressive glomerulonephritis and double seropositivity for myeloperoxidase-ANCA and anti-GBM antibodies. A kidney biopsy revealed diffuse cellular crescents with necrosis and immunoglobin (Ig)G1 and IgG3 positivity on immunofluorescence staining, leading to a histological diagnosis of anti-glomerular basement membrane nephritis. Our treatment approach involved a novel combination of glucocorticoids, rituximab, low-dose cyclophosphamide, and plasma exchange complemented by avacopan. Temporary hemodialysis was required, and the patient successfully discontinued dialysis after 12 sessions despite a poor histological prognosis. This case underscores the significance of considering aggressive therapeutic strategies, including avacopan, for severe anti-GBM nephritis, even in the absence of lung involvement, to avert the need for KRT.

Glomerulonephritis during Mycobacterium tuberculosis infection: scoping review

IntroductionPeople with Tuberculosis (TB) infection may present with glomerulonephritis (GN). The range of presentations, renal pathologies, and clinical outcomes are uncertain. Whether clinical features that establish if GN etiology is medication or TB related, and possible benefits of immunosuppression remain uncertain.MethodsA scoping review was completed, searching MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, Web of Science and Conference s from Inception to December, 2023. The study population included patients with TB infection who developed GN and underwent renal biopsy. All data regarding presentation, patient characteristics, renal pathology, management of TB and GN, and outcomes were summarized.ResultsThere were 62 studies identified, with 130 patients. These cases included a spectrum of presentations including acute kidney injury, nephrotic syndrome and hypertension, and a range of 10 different renal pathology diagnoses. Cases that included immunosuppression and outcomes ranged from complete remission to long-term dialysis dependence. The presence of granulomas (4/4, 100%), anti-glomerular basement membrane disease (3/3, 100%), amyloidosis (75/76, 98.7%), and focal segmental glomerulosclerosis (2/2, 100%) were specific for GN being TB-infection related. On the other hand, minimal change disease was specific for anti-TB therapy related (7/7, 100%). While patients with more aggressive forms of GN commonly were prescribed immunosuppression, this study was unable to confirm efficacy. Only rifampin or isoniazid were implicated in drug-associated GN.DiscussionThis study provides a clear rationale for renal biopsy in patients with TB and GN, and outlines predictors for the GN etiology. Thus, this study establishes key criteria to optimize diagnosis and management of patients with TB and GN.