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systemic lupus , glomerulonephritis

Laura S van Dam, Ton J Rabelink, Cees van Kooten, Y K Onno Teng
Neutrophil extracellular traps (NETs) are extracellular DNA structures covered with antimicrobial peptides, danger molecules, and autoantigens that can be released by neutrophils. NETs are an important first-line defense mechanism against bacterial, viral, fungal, and parasitic infections, but they can also play a role in autoimmune diseases. NETs are immunogenic and toxic structures that are recognized by the autoantibodies of patients with antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) (i...
February 2019: KI Reports
Yi Li Wong, Mei-Tzu Su, Akiko Sugahara-Tobinai, So Itoi, Dai Kezuka, Shota Endo, Masanori Inui, Toshiyuki Takai
Immune homeostasis is critically regulated by the balance between activating and inhibitory receptors expressed on various immune cells such as T and B lymphocytes, and myeloid cells. The inhibitory receptors play a fundamental role in the immune-checkpoint pathway, thus maintaining peripheral tolerance. We recently found that expression of leukocyte immunoglobulin-like receptor (LILR)B4, an inhibitory member of the human LILR family, is augmented in autoantibody-producing plasmablasts/plasma cells of systemic lupus erythematosus (SLE) patients...
February 15, 2019: International Immunology
Vasil V Vasilev, Maria Radanova, Valentin J Lazarov, Marie-Agnes Dragon-Durey, Veronique Fremeaux-Bacchi, Lubka T Roumenina
The complement component C3 is at the heart of the complement cascade. It is a complex protein, which generates different functional activated fragments (C3a, C3b, iC3b, C3c, C3d). C3b is a constituent of the alternative pathway C3 convertase (C3bBb), binds multiple regulators, and receptors, affecting thus the functioning of the immune system. The activated forms of C3 are a target for autoantibodies. This review focuses on the discovery, disease relevance, and functional consequences of the anti-C3b autoantibodies...
2019: Frontiers in Immunology
Kenta Imaruoka, Yuji Oe, Tomofumi Fushima, Emiko Sato, Akiyo Sekimoto, Hiroshi Sato, Junichi Sugawara, Sadayoshi Ito, Nobuyuki Takahashi
Systemic lupus erythematosus (SLE) increases the risk of preterm birth and preeclampsia (PE). The flares of SLE during pregnancy or after delivery are also problematic. We have previously demonstrated that nicotinamide (NAM), a non-teratogenic amide of vitamin B3, reduces inflammation and oxidative stress and improves PE-like phenotype and pregnancy outcomes in the mouse models of PE. The present study aimed to establish a model to investigate the pregnancy outcomes and flares of SLE in pregnant mice with SLE and to examine whether NAM is beneficial to pregnant mice with SLE...
February 7, 2019: Biochemical and Biophysical Research Communications
Lauren Feldman, Megha Dhamne, Yuebing Li
INTRODUCTION: Limited information is available describing the spectrum of neurological complications of cryoglobulinemia. METHODS: Single center retrospective review of patients with neurologic symptoms and elevated serum cryoglobulins, with their potential association being classified as definite, possible, or unlikely using defined criteria. RESULTS: Among 492 patients, 131 (87 classified as definite and 44 as possible) had neurologic symptoms associated with cryoglobulinemia...
January 25, 2019: Journal of the Neurological Sciences
Shijun He, Xing Liu, Zemin Lin, Yuting Liu, Lei Gu, Hu Zhou, Wei Tang, Jianping Zuo
BACKGROUND: Glomerulonephritis is one of the major complications and causes of death in systemic lupus erythematosus (SLE) and is characterized by glomerulosclerosis, interstitial fibrosis, and tubular atrophy, along with severe persistent proteinuria. DZ2002 is a reversible S-adenosyl-L-homocysteine hydrolase (SAHH) inhibitor with potent therapeutic activity against lupus nephritis in mice. However, the molecular events underlying the renal protective effects of DZ2002 remained unclear...
January 29, 2019: Arthritis Research & Therapy
R Popa, L A Lautaru, R Lucretiu, D C Ruiu, D Caragea, M Olteanu, A R Mihailovici, C Ene, V Padureanu, R Padureanu, V Cirlig
Glucosteroids (GS) are widely used drugs for various inflammatory pathologies (Nephrotic syndrome, Proliferative glomerulonephritis, Extramembrane glomerulonephritis, Nephropathy of the Nodous Poliarterita (PAN), Nephropathy from purple Henoch-Schonlein, lupus nephropathy (LN), Acute adrenal insufficiency Waterhouse-Friederichsen, Chronic adrenal insufficiency Addison, Systemic Lupus Erythematosus (SLE), Polymyositis and dermatomyositis, Chronic granulomatosis, Crohn's disease, Hemorrhagic rectocolitis, Hemolytic anemias, Acute leukemias and chronic lymphocytic leukemia, Hodgkin's lymphoma)...
July 2018: Current Health Sciences Journal
Samantha A Chalmers, Sayra J Garcia, Joshua A Reynolds, Leal Herlitz, Chaim Putterman
Immune-mediated glomerulonephritis is a serious end organ pathology that commonly affects patients with systemic lupus erythematosus (SLE). A classic murine model used to study lupus nephritis (LN) is nephrotoxic serum nephritis (NTN), in which mice are passively transferred nephrotoxic antibodies. We have previously shown that macrophages are important in the pathogenesis of LN. To further investigate the mechanism by which macrophages contribute to the pathogenic process, and to determine if this contribution is mediated by NF-κB signaling, we created B6 mice which had RelA knocked out in myeloid cells, thus inhibiting classical NF-κB signaling in this cell lineage...
January 3, 2019: Journal of Autoimmunity
Asim Sadaf, Moorani N Khemchand, Lateef Fouzia, Zubair Asia
Renal biopsy is an important tool for the diagnosis of acute and chronic glomerular diseases in children. We aimed to analyze the spectrum of clinical indications and histopathological patterns (HPP) in children who underwent renal biopsy (RB). This is a retrospective review of case records of 108 renal biopsies carried out from January 2010 to December 2015 at the Pediatric Nephrology Department, National Institute of Child Health Karachi, Pakistan. RB was performed under Ketamine-Midazolam sedation and real-time ultrasound...
November 2018: Saudi Journal of Kidney Diseases and Transplantation
Genhong Yao, Jingjing Qi, Zhuoya Zhang, Saisai Huang, Linyu Geng, Wenchao Li, Weiwei Chen, Xiaojun Tang, Shiying Wang, Lingyun Sun
AIM: Cardiovascular complications related to atherosclerosis are major causes of morbidity and mortality in patients with systemic lupus erythematosus (SLE). However, the underlying mechanisms are not fully understood. Endothelial dysfunction has been identified as having involvement in pathogenesis of cardiovascular diseases and SLE. This study aims to evaluate endothelial cell injury in mice with the combination of lupus and atherosclerosis. METHODS: The mouse model of accelerated atherosclerosis in lupus (gld...
December 21, 2018: International Journal of Rheumatic Diseases
Rhea Bhargava, George C Tsokos
PURPOSE OF REVIEW: Lupus nephritis (LN) is a serious manifestation of systemic lupus erythematosus and is characterized by proteinuria and renal failure. Proteinuria is a marker of poor prognosis and is attributed to podocyte loss and dysfunction. It is often debated whether these cells are innocent bystanders or active participants in the pathogenesis of glomerulonephritis. RECENT FINDINGS: Podocytes share many elements of the innate and adaptive immune system...
March 2019: Current Opinion in Rheumatology
Saúl Pampa-Saico, Roberto Marcén-Letosa, Ana Fernández-Rodríguez, Laura E Díaz-González, Sara Jiménez-Álvaro, Fernando Liaño García
INTRODUCTION: The outcome and prognosis of systemic lupus erythematosus (SLE) in long-term kidney transplantation (KT) is variable. The objective of this study was to analyse the survival of the graft and the patient, comparing rates with a control group (primary glomerulonephritis [PGN]). MATERIALS AND METHODS: Forty-three patients receiving a KT with diagnosis of lupus nephritis (LN) and 367 patients with PGN were compared between January 1980 and December 2014...
November 27, 2018: Medicina Clínica
Melek Kharroubi, Lilia Ben Fatma, Lamia Rais, Hela Jebali, Ikram Mami, Mohamed Karim Zouaghi
BACKGROUND: Glomerular deposits are a major pathologic feature of a wide range of human glomerulonephritis and may be located in the mesangial, subepithelial, and subendothelial regions. Rare cases of primary glomerulonephritis definied by exclusive or predominant mesangial IgG deposits were reported. We reviewed the pathologic findings for the 848 renal biopsies examined in our department between 2007 and 2016, one case of primary mesangial IgG glomerulonephritis (MIG) in the absence of any evidence of systemic lupus erythematous (SLE), of other systemic diseases or of Infections...
July 2018: La Tunisie Médicale
Ahmad Alflaiw, Ghormallah Alghamdi, Khalid Alsaad, Noura Aloudah
We describe here a case of a 53-year-old Saudi female who presented to the emergency room with shortness of breath progressive in nature for the previous one month, associated with a cough and occasional greenish sputum. She turned out to be a case of immunocomplex mediated glomerulonephritis presenting as rapidly progressive glomerulonephritis in a female patient with a history of systemic lupus erythematosus (SLE) in her daughter.
September 2018: Saudi Journal of Kidney Diseases and Transplantation
Chun-Ling Liang, Weihui Lu, Jiu-Yao Zhou, Yuchao Chen, Qunfang Zhang, Huazhen Liu, Feifei Qiu, Zhenhua Dai
BACKGROUND/AIMS: Lupus nephritis (LN) is an autoimmune glomerulonephritis that frequently develops secondary to systemic lupus erythematosus. Patients with LN require extensive treatments with global immunosuppressive agents. However, long-term treatment with conventional immunosuppressants may cause various side effects. Therefore, it's important to seek alternative drugs for treating LN. Here we aimed to investigate the immunoregulatory effects of mangiferin (MG), an ingredient that was originally extracted from natural herbs, including Mangifera Indica Linn...
2018: Cellular Physiology and Biochemistry
Yiming Luo, Yumeng Wen, Ana Belen Arevalo Molina, Punya Dahal, Lorenz Leuprecht, Makda Bsrat
Macrophage activation syndrome (MAS) is a rare manifestation of systemic lupus erythematosus (SLE) with potentially life-threatening consequences. To the best of our knowledge, this is the first case reported in literature for a constellation of MAS, glomerulonephritis, pericarditis, and retinal vasculitis as initial presentation of SLE. Despite extensive multisystem involvement of his disease, the patient responded well to initial steroid treatment, with mycophenolate mofetil successfully added as a steroid-sparing agent...
2018: Case Reports in Medicine
Alaa A Ali, Dana A Sharif, Safa E Almukhtar, Kais Hasan Abd, Zana Sidiq M Saleem, Michael D Hughson
BACKGROUND: Estimates of the incidence of glomerulonephritis (GN) and end-stage renal disease (ESRD) in an Iraqi population are compared with the United States (US) and Jordan. METHODS: The study set consist of renal biopsies performed in 2012 and 2013 in the Kurdish provinces of Northern Iraq. The age specific and age standardized incidence of GN was calculated from the 2011 population. ESRD incidence was estimated from Sulaimaniyah dialysis center records of patient's inititating hemodialysis in 2017...
October 11, 2018: BMC Nephrology
Ying Tan, Ming-Hui Zhao
Complement activation has been identified to play a vital role in the pathogenesis of many glomerulonephritis, either as direct complement activation-driven factor in thrombotic microangiopathy and C3 glomerulopathy, and/or as an important contributor in lupus nephritis and anti-neutrophil cytoplasmic antibody-associated vasculitis. Recent studies indicated that complement activation may also play roles in the pathogenesis of immunoglobulin A nephropathy and focal segmental glomerulosclerosis. Interestingly, monoclonal immunoglobulins/light chains from patients with monoclonal gammopathy may interfere with complement activation and thus indirectly result in complement-mediated glomerulonephritis...
October 2018: Nephrology
Zhenlu Chong, Chunjing Bao, Jia He, Tianxiao Chen, Lijia Zhong, Gaopeng Li, Huanle Li, Lutong Fang, Yinjing Song, Guoxiang Fu, Xuyan Yang, Lihua Lai, Yang Liu, Qingqing Wang
Systemic lupus erythematosus (SLE) is a systemic autoimmune disease, and the pathogenesis of SLE has not been fully elucidated. The E3 ubiquitin ligase FBXW7 has been well characterized in cancer as a tumor suppressor that can promote the ubiquitination and subsequent degradation of various oncoproteins; however, the potential role of FBXW7 in autoimmune diseases is unclear. In the present study, we identified that FBXW7 is a crucial exacerbating factor for SLE development and progression in a mouse model induced by 2, 6, 10, 14-tetramethylpentadecane (TMPD)...
October 1, 2018: Cellular & Molecular Immunology
Melissa A Bates, Peyman Akbari, Kristen N Gilley, James G Wagner, Ning Li, Anna K Kopec, Kathryn A Wierenga, Daven Jackson-Humbles, Christina Brandenberger, Andrij Holian, Abby D Benninghoff, Jack R Harkema, James J Pestka
Ectopic lymphoid structures (ELS) consist of B-cell and T-cell aggregates that are initiated de novo in inflamed tissues outside of secondary lymphoid organs. When organized within follicular dendritic cell (FDC) networks, ELS contain functional germinal centers that can yield autoantibody-secreting plasma cells and promote autoimmune disease. Intranasal instillation of lupus-prone mice with crystalline silica (cSiO2 ), a respirable particle linked to human lupus, triggers ELS formation in the lung, systemic autoantibodies, and early onset of glomerulonephritis...
2018: Frontiers in Immunology
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