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systemic lupus , glomerulonephritis

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https://www.readbyqxmd.com/read/29987665/proliferative-glomerulonephritis-with-unusual-microlamellar-organized-deposits-related-to-monoclonal-immunoglobulin-g3-igg3-kappa
#1
Akiko Mii, Akira Shimizu, Daisuke Takada, Shuichi Tsuruoka
A 71-year-old woman presented with massive proteinuria and microhematuria. Renal biopsy showed diffuse global membranoproliferative and endocapillary proliferative lesions with leukocytic infiltration and an irregular duplication of the glomerular basement membrane on light microscopy. Immunofluorescence study showed granular deposits of monoclonal immunoglobulin G3 (IgG3) kappa, C3, and C1q in the glomeruli. Electron microscopy revealed unique structurally organized microlamellar electron-dense deposits. There was no evidence of systemic diseases such as paraproteinemia, cryoglobulinemia, or systemic lupus erythematosus...
July 9, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29925830/c-terminal-truncation-of-ifn-%C3%AE-inhibits-proinflammatory-macrophage-responses-and-is-deficient-in-autoimmune-disease
#2
Antoine Dufour, Caroline L Bellac, Ulrich Eckhard, Nestor Solis, Theo Klein, Reinhild Kappelhoff, Nikolaus Fortelny, Parker Jobin, Jacob Rozmus, Jennifer Mark, Paul Pavlidis, Vincent Dive, Sean J Barbour, Christopher M Overall
Controlled macrophage differentiation and activation in the initiation and resolution of inflammation is crucial for averting progression to chronic inflammatory and autoimmune diseases. Here we show a negative feedback mechanism for proinflammatory IFN-γ activation of macrophages driven by macrophage-associated matrix metalloproteinase 12 (MMP12). Through C-terminal truncation of IFN-γ at 135Glu↓Leu136 the IFN-γ receptor-binding site was efficiently removed thereby reducing JAK-STAT1 signaling and IFN-γ activation of proinflammatory macrophages...
June 20, 2018: Nature Communications
https://www.readbyqxmd.com/read/29925508/tlr4-cxcr4-plasma-cells-drive-nephritis-development-in-systemic-lupus-erythematosus
#3
Kongyang Ma, Jingyi Li, Xiaohui Wang, Xiang Lin, Wenhan Du, Xi Yang, Fangxiang Mou, Yongfei Fang, Yanbin Zhao, Xiaoping Hong, Kwok Wah Chan, Xiaoming Zhang, Dongzhou Liu, Lingyun Sun, Liwei Lu
OBJECTIVES: In patients with systemic lupus erythematosus (SLE), immune tolerance breakdown leads to autoantibody production and immune-complex glomerulonephritis. This study aimed to identify pathogenic plasma cells (PC) in the development of lupus nephritis. METHODS: PC subsets in peripheral blood and renal tissue of patients with SLE and lupus mice were examined by flow cytometry and confocal microscopy, respectively. Sorting-purified PCs from lupus mice were adoptively transferred into Rag2 -deficient recipients, in which immune-complex deposition and renal pathology were investigated...
June 20, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29922524/fibrillary-glomerulonephritis-in-a-patient-with-sjogren-s-syndrome
#4
Muhammad Uzair Lodhi, Tahira Sabeen Saleem, Muhammad Shariq Usman, Waliul Chowdhury, Aaron R Kuzel, Hafiz Imran Iqbal, Mustafa Rahim
Fibrillary glomerulonephritis (FGN) is an uncommon cause of primary glomerular disease. FGN is usually idiopathic; however, it has been associated with underlying malignancy or autoimmune diseases in some patients as well. The most commonly found autoimmune diseases in FGN patients include Graves' disease, systemic lupus nephritis, Chron's disease, and idiopathic thrombocytopenia purpura. FGN in a patient with underlying asymptomatic Sjogren's syndrome is very rare in the literature, with only two previously reported cases of this association...
April 15, 2018: Curēus
https://www.readbyqxmd.com/read/29907458/taci-deletion-protects-against-progressive-murine-lupus-nephritis-induced-by-baff-overexpression
#5
Tanvi Arkatkar, Holly M Jacobs, Samuel W Du, Quan-Zhen Li, Kelly L Hudkins, Charles E Alpers, David J Rawlings, Shaun W Jackson
B cells are known to promote the pathogenesis of systemic lupus erythematosus (SLE) via the production of pathogenic anti-nuclear antibodies. However, the signals required for autoreactive B cell activation and the immune mechanisms whereby B cells impact lupus nephritis pathology remain poorly understood. The B cell survival cytokine B cell activating factor of the TNF Family (BAFF) has been implicated in the pathogenesis of SLE and lupus nephritis in both animal models and human clinical studies. Although the BAFF receptor has been predicted to be the primary BAFF family receptor responsible for BAFF-driven humoral autoimmunity, in the current study we identify a critical role for signals downstream of Transmembrane Activator and CAML Interactor (TACI) in BAFF-dependent lupus nephritis...
June 12, 2018: Kidney International
https://www.readbyqxmd.com/read/29892304/essential-roles-for-mannose-binding-lectin-associated-serine-protease-1-3-in-the-development-of-lupus-like-glomerulonephritis-in-mrl-lpr-mice
#6
Takeshi Machida, Natsumi Sakamoto, Yumi Ishida, Minoru Takahashi, Teizo Fujita, Hideharu Sekine
The complement system, composed of the three activation pathways, has both protective and pathogenic roles in the development of systemic lupus erythematosus (or lupus), a prototypic autoimmune disease. The classical pathway contributes to the clearance of immune complexes (ICs) and apoptotic cells, whereas the alternative pathway (AP) exacerbates renal inflammation. The role of the lectin pathway (LP) in lupus has remained largely unknown. Mannose-binding lectin (MBL)-associated serine proteases (MASPs), which are associated with humoral pattern recognition molecules (MBL or ficolins), are the enzymatic constituents of the LP and AP...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29867982/renal-involvement-in-antiphospholipid-syndrome
#7
REVIEW
Alonso Turrent-Carriles, Juan Pablo Herrera-Félix, Mary-Carmen Amigo
Antiphospholipid syndrome is a complex autoimmune disease, characterized by the presence of vascular thrombosis, obstetric, hematologic, cutaneous, and cardiac manifestations. Renal disease in patients with antiphospholipid syndrome was not recognized in the first descriptions of the disease, but later on, the renal manifestations of the syndrome have been investigated widely. Renal manifestations of antiphospholipid syndrome conform a wide spectrum of diverse renal syndromes. Hypertension is one of the most frequent, but less commonly recognized renal alteration...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29775752/the-genetics-and-molecular-pathogenesis-of-systemic-lupus-erythematosus-sle-in-populations-of-different-ancestry
#8
REVIEW
George N Goulielmos, Maria I Zervou, Vassilis M Vazgiourakis, Yogita Ghodke-Puranik, Alexandros Garyfallos, Timothy B Niewold
Systemic lupus erythematosus (SLE; OMIM 152700) is a highly heterogeneous disorder, characterized by differences in autoantibody profile, serum cytokines, and a multi-system involvement commonly affecting the skin, renal, musculoskeletal, and hematopoetic systems clinical manifestations involving. Disease features range from mild manifestations, such as rash or arthritis, to life-threatening end-organ manifestations, such as glomerulonephritis or thrombosis, and it is difficult to predict which manifestations will affect a given patient...
August 20, 2018: Gene
https://www.readbyqxmd.com/read/29768670/patients-affected-by-a-new-variant-of-endemic-pemphigus-foliaceus-have-autoantibodies-colocalizing-with-myzap-p0071-desmoplakins-1-2-and-arvcf-causing-renal-damage
#9
A M Abreu-Velez, M S Howard, H Yi, A A Florez-Vargas
BACKGROUND: We have previously reported that about 30% of patients affected by a new variant of endemic pemphigus foliaceus (EPF) in El Bagre, Colombia (termed El Bagre-EPF or pemphigus Abreu-Manu) have systemic compromise. In the current study, we focused on studying autoreactivity to the kidney and its pathological correlations. AIM: To investigate patients with El Bagre-EPF for renal compromise. METHODS: We performed a case-control study, enrolling 57 patients with El Bagre-EPF and 57 controls from the endemic area, matched by age, sex, race, work activity, demographics and comorbidities...
May 16, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29730854/renal-involvement-in-antiphospholipid-syndrome
#10
REVIEW
Francisco Vileimar Andrade de Azevedo, Diego Germano Maia, Jozelio Freire de Carvalho, Carlos Ewerton Maia Rodrigues
This is a review of scientific publications on renal involvement in antiphospholipid syndrome (APS), with focus on clinical and histopathological findings and treatment. A search for English-language articles on renal involvement in APS covering the period 1980-2017 was conducted in Medline/PubMed and Scopus databases using the MeSH terms "antiphospholipid syndrome", "antiphospholipid antibodies", "glomerulonephritis" and "thrombotic microangiopathy" (TMA). APS nephropathy is primarily the result of thromboses in renal arteries or veins, intraparenchymatous arteries and glomerular capillaries...
May 5, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29724730/metabolic-pathways-and-immunometabolism-in-rare-kidney-diseases
#11
Peter C Grayson, Sean Eddy, Jaclyn N Taroni, Yaíma L Lightfoot, Laura Mariani, Hemang Parikh, Maja T Lindenmeyer, Wenjun Ju, Casey S Greene, Brad Godfrey, Clemens D Cohen, Jeffrey Krischer, Matthias Kretzler, Peter A Merkel
OBJECTIVES: To characterise renal tissue metabolic pathway gene expression in different forms of glomerulonephritis. METHODS: Patients with nephrotic syndrome (NS), antineutrophil cytoplasmic antibody-associated vasculitis (AAV), systemic lupus erythematosus (SLE) and healthy living donors (LD) were studied. Clinically indicated renal biopsies were obtained at time of diagnosis and microdissected into glomerular and tubulointerstitial compartments. Microarray-derived differential gene expression of 88 genes representing critical enzymes of metabolic pathways and 25 genes related to immune cell markers was compared between disease groups...
May 3, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29713015/atherogenic-dyslipidemia-promotes-autoimmune-follicular-helper-t-cell-responses-via-il-27
#12
Heeju Ryu, Hoyong Lim, Garam Choi, Young-Jun Park, Minkyoung Cho, Hyeongjin Na, Chul Won Ahn, Young Chul Kim, Wan-Uk Kim, Sang-Hak Lee, Yeonseok Chung
The incidence of atherosclerosis is higher among patients with systemic lupus erythematosus (SLE); however, the mechanism by which an atherogenic environment affects autoimmunity remains unclear. We found that reconstitution of atherosclerosis-prone Apoe-/- and Ldlr-/- mice with bone marrow from lupus-prone BXD2 mice resulted in increased autoantibody production and glomerulonephritis. This enhanced disease was associated with an increase in CXCR3+ follicular helper T cells (TFH cells). TFH cells isolated from Apoe-/- mice had higher expression of genes associated with inflammatory responses and SLE and were more potent in inducing production of the immunoglobulin IgG2c...
June 2018: Nature Immunology
https://www.readbyqxmd.com/read/29704464/administration-of-activated-lymphocyte-derived-dna-accelerates-and-aggravates-lupus-nephritis-in-b6-lpr-mice-a-new-approach-to-modify-a-lupus-murine-model
#13
Yuanyuan Zhu, Yan Yue, Sidong Xiong
B6/lpr mouse strain is a well-known systemic lupus erythematosus murine model characterized by uncontrolled lymphoproliferation and autoantibody production. However, it displays a delayed and mild development of lupus nephritis (LN), which is not conducive to the research of the pathogenesis and therapeutic strategies of this condition. Our previous study demonstrated that activated lymphocyte-derived DNA (ALD-DNA) could induce high urine protein levels and severe glomerulonephritis (GN) in BALB/c mice. In the present study, we tried to remedy delayed urine protein production and mild GN in B6/lpr mice via ALD-DNA immunization...
April 28, 2018: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/29698559/tuftsin-phosphorylcholine-tpc-equally-effective-to-methylprednisolone-in-ameliorating-lupus-nephritis-in-a-mice-model
#14
Asaf Shemer, Shay Kivity, Ora Shovman, Ori Perry, Abdulla Watad, Dana Ben-Ami Shor, Alex Volkov, Iris Barshack, Nicola L Bragazzi, Alexander Krule, Mati Fridkin, Howard Amital, Miri Blank, Yehuda Shoenfeld
BACKGROUND: The role of helminth treatment in autoimmune diseases is constantly growing. Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease with challenging treatment options. Tuftsin phosphorylcholine (TPC) is a novel helminth-based compound that modulates the host immune network. OBJECTIVES: This study was conducted to evaluate the potential value of TPC in ameliorating lupus nephritis in a murine model and specifically to compare the efficacy of TPC to the existing first-line therapy for SLE: corticosteroids (methylprednisolone)...
April 26, 2018: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/29687651/tlr9-deficiency-breaks-tolerance-to-rna-associated-antigens-and-upregulates-tlr7-protein-in-sle1-mice
#15
Teja Celhar, Hiroko Yasuga, Hui-Yin Lee, Olga Zharkova, Shubhita Tripathi, Susannah I Thornhill, Hao K Lu, Bijin Au, Lina H K Lim, Thomas P Thamboo, Shizuo Akira, Edward K Wakeland, John E Connolly, Anna-Marie Fairhurst
OBJECTIVE: Toll-like receptors (TLRs) 7 and 9 are important innate signaling molecules with opposing roles in the development and progression of Systemic Lupus Erythematosus (SLE). While multiple studies support a dependency on TLR7 for disease development, genetic ablation of TLR9 results in severe disease with glomerulonephritis (GN) by a largely unknown mechanism. The present study was designed to examine the suppressive role of TLR9 in the development of severe lupus. METHODS: We crossed Sle1 lupus-prone mice with TLR9-deficient mice to generate Sle1TLR9-/- ...
April 24, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29657218/rapidly-progressive-glomerulonephritis-a-wild-card-manifestation-of-lupus-nephritis
#16
Opeyemi O Komolafe
A significant proportion of individuals with systemic lupus erythematosus (SLE) will have clinical or laboratory evidence of renal involvement at some point in the course of their disease. Lupus nephritis (LN) is one of the more important systemic manifestations of SLE and although progression to end-stage renal disease (ESRD) is not usually the norm, a significant minority of patients will do so, some in a rather dramatic fashion. It is in such instances that LN constitutes a true nephrologic emergency. Gratifyingly, it is rarely a silent emergency and heightened awareness amongst clinicians about its unpredictable course should lead to earlier recognition and intervention...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29650017/toll-like-receptors-in-lupus-nephritis
#17
REVIEW
Satish Kumar Devarapu, Hans-Joachim Anders
The pathogenesis of systemic autoimmune diseases such as systemic lupus erythematosus (SLE) is based on the loss of self-tolerance against ubiquitous autoantigens involving all mechanisms of adaptive immunity. However, data accumulating over the last decade imply an important role also for numerous elements of innate immunity, namely the Toll-like receptors in the pathogenesis of SLE. Here we discuss their role in the most common organ complication of SLE, i.e. lupus nephritis. We summarize experimental and clinical data on the expression and functional contribution of the Toll-like receptors in immune complex glomerulonephritis, and intrarenal inflammation...
April 12, 2018: Journal of Biomedical Science
https://www.readbyqxmd.com/read/29594143/a-case-of-crescentic-glomerulonephritis-complicated-with-hypocomplementemic-urticarial-vasculitis-syndrome-and-anca-associated-vasculitis
#18
Marenao Tanaka, Norihito Moniwa, Tomohiro Mita, Toshiyuki Tobisawa, Tamaki Matsumoto, Atsushi Mochizuki, Tomohisa Yamashita, Toshiyuki Yano, Masato Furuhashi, Tetsuji Miura
Systemic urticaria in a 64-year-old woman was diagnosed as leukocytoclastic vasculitis by a punch biopsy of the skin. Her physical findings improved after prescription of prednisolone at a dose of 20 mg/day, but the skin rash relapsed with renal dysfunction, proteinuria, and hematuria when the dose of prednisolone was reduced over a period of 9 months to 1 mg/day. She was admitted to our institute for further examination, when urinary protein and plasma creatinine levels were 0.8 g/day and 1.7 mg/dL, respectively...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29550838/c1r-c1s-deficiency-is-insufficient-to-induce-murine-systemic-lupus-erythematosus
#19
Kozue Uchio-Yamada, Mayuri Tanaka, Noboru Manabe
C1s deficiency is strongly associated with the development of human systemic lupus erythematosus (SLE); however, the mechanisms by which C1s deficiency contributes to the development of SLE have not yet been elucidated in detail. Using ICR-derived-glomerulonephritis (ICGN) mouse strain that develops SLE and very weakly expresses C1s in the liver, we investigated the protective roles of C1s against SLE. A genetic sequence analysis revealed complete deletion of the C1s1 gene, a mouse homolog of the human C1s gene, with partial deletion of the C1ra and C1rb genes in the ICGN strain...
March 18, 2018: Genes and Immunity
https://www.readbyqxmd.com/read/29524578/impact-of-enos-27-bp-vntr-4b-a-gene-polymorphism-with-the-risk-of-systemic-lupus-erythematosus-in-south-indian-subjects
#20
Shiva Krishna Katkam, Bobbala Indumathi, Fatima S D Tasneem, Liza Rajasekhar, Vijay Kumar Kutala
OBJECTIVE: Endothelial nitric oxide synthase (eNOS) is constitutively expressed by vascular endothelium including glomerular endothelium. Functional polymorphisms, -786T>C (rs2070744) promoter variant, 27 bp VNTR (4b/a) in intron 4 and 894G>T (rs1799983) exon variant of eNOS are known to alter the eNOS expression and activity leading to altered NO levels and contribute to the development of vascular and renal disease risk. Thus it might have a role in SLE risk and development of glomerulonephritis...
June 5, 2018: Gene
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