Journal ArticleDOI
The immune system and kidney disease: basic concepts and clinical implications
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TLDR
The kidneys are frequently targeted by pathogenic immune responses against renal autoantigens or by local manifestations of systemic autoimmunity, causing intestinal barrier dysfunction, systemic inflammation and immunodeficiency that contribute to the morbidity and mortality of patients with kidney disease.Abstract:
The kidneys are frequently targeted by pathogenic immune responses against renal autoantigens or by local manifestations of systemic autoimmunity. Recent studies in rodent models and humans have uncovered several underlying mechanisms that can be used to explain the previously enigmatic immunopathology of many kidney diseases. These mechanisms include kidney-specific damage-associated molecular patterns that cause sterile inflammation, the crosstalk between renal dendritic cells and T cells, the development of kidney-targeting autoantibodies and molecular mimicry with microbial pathogens. Conversely, kidney failure affects general immunity, causing intestinal barrier dysfunction, systemic inflammation and immunodeficiency that contribute to the morbidity and mortality of patients with kidney disease. In this Review, we summarize the recent findings regarding the interactions between the kidneys and the immune system.read more
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Case report: Unilateral panuveitis as a manifestation of Alport syndrome in a Chinese pediatric patient
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TL;DR: In this paper , BTLA signaling effectively restrained nephritogenic Th1 cells and promoted regulatory T cells in a model of crescentic glomerulonephritis (GN) in Btla-deficient mice and wild-type littermate controls.
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The five types of glomerulonephritis classified by pathogenesis, activity, and chronicity (GN-AC).
TL;DR: In this article , the authors apply a conceptual framework of immune-mediated disorders to Glomerulonephritis (GN) guided by immunopathogenesis and hence immunophenotyping: 1) Infection-related GN require pathogen identification and control, 2) Autoimmunity-relatedGN, defined by presence of autoantibodies, and 3) AlloimmunITY-related MG in transplant recipients both require the suppression of adaptive immunity in lymphoid organs and bone marrow, 4) Autoinflammation-related GM, e.g., inborn errors of immunity diagnosed by genetic testing, requires suppression of single cytokine or complement pathways, and 5) Monoclonal gammopathy-related GG requires B or plasma cell clone-directed therapy.
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Coming of Age—CC Chemokine Ligand 18 in ANCA-Associated Vasculitis
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References
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Journal ArticleDOI
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TL;DR: The main advantages of the current revised classification is that it provides a clear and unequivocal description of the various lesions and classes of lupus nephritis, allowing a better standardization and lending a basis for further clinicopathologic studies.
Journal ArticleDOI
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TL;DR: A majority of patients with idiopathic membranous nephropathy have antibodies against a conformation-dependent epitope in PLA(2)R, indicating that PLA( 2)R is a major antigen in this disease.
Journal ArticleDOI
Cellular pathophysiology of ischemic acute kidney injury
Joseph V. Bonventre,Li Yang +1 more
TL;DR: The major components of this dynamic process, which involves hemodynamic alterations, inflammation, and endothelial and epithelial cell injury, followed by repair that can be adaptive and restore epithelial integrity or maladaptive, leading to chronic kidney disease are reviewed.
Journal ArticleDOI
von Willebrand Factor–Cleaving Protease in Thrombotic Thrombocytopenic Purpura and the Hemolytic–Uremic Syndrome
Miha Furlan,Rodolfo Robles,Miriam Galbusera,Giuseppe Remuzzi,Paul A. Kyrle,Brigitte Brenner,Manuela Krause,Inge Scharrer,Volker Aumann,Uwe Mittler,Max Solenthaler,Bernhard Lämmle +11 more
TL;DR: In this article, the authors investigated the prevalence of von Willebrand factor-cleaving protease deficiency in patients with familial and non-familial forms of thrombocytopenic purpura and hemolytic-uremic syndrome.
Journal ArticleDOI
Netting neutrophils in autoimmune small-vessel vasculitis.
Kai Kessenbrock,Markus Krumbholz,Ulf Schönermarck,Walter Back,Wolfgang Gross,Zena Werb,Hermann-Josef Gröne,Volker Brinkmann,Dieter E. Jenne +8 more
TL;DR: It is shown that chromatin fibers, so-called neutrophil extracellular traps (NETs), are released by ANCA-stimulated neutrophils and contain the targeted autoantigens proteinase-3 and myeloperoxidase (MPO).