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systemic lupus , glomerulonephritis

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
Qianqian Yuan, Yan Li, Jiangxia Li, Xianli Bian, Feng Long, Ruonan Duan, Xiaochun Ma, Fei Gao, Shang Gao, Shijun Wei, Xi Li, Wenjie Sun, Qiji Liu
Genome-wide association studies have recently illuminated that WDFY4 is genetically associated with systemic lupus erythematosus (SLE) susceptibility in various ethnic groups. Despite strong genetic evidence suggesting a role of WDFY4 in SLE pathogenesis, its functional relevance is largely unknown. In this study, we generated Wdfy4 B lymphocyte conditional knockout ( Wdfy4 -CKO) mice and found that loss of Wdfy4 led to a decrease in number of total B cells and several subpopulations of B cells in the periphery and a defect in the transition from the pro- to pre-B cell stage in bone marrow...
September 26, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Li Wang, Hua Chen, Jing Shi, Hao Tang, Hang Li, Wenjie Zheng, Fengchun Zhang
RATIONALE: Castleman disease (CD) is a nonclonal lymphoproliferative disorder sometimes manifested systemic inflammatory symptoms. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized with multi-system involvement as well as broad spectrum of serum autoantibodies. When these two conditions happened to have similar clinical spectrum features, the confusion with each other occurred. PATIENT CONCERNS: A 46-year-old man suffered from chronic fever, nephrotic syndrome, acute kidney injury, anemia, thrombocytopenia and serositis, as well as hypocomplementemia and negative anti-nuclear antibody...
September 2018: Medicine (Baltimore)
L T Pontes, D T Camilo, M R De Bortoli, R S S Santos, W M Luchi
We report an original case of a 27-year-old transgender woman who developed lupus nephritis after male-to-female sex reassignment surgery. The patient had been taking hormones to induce feminization since the age of 18. She was admitted with malar "butterfly" rash, anasarca and hypertension, associated with an increase in serum creatinine (1.7 mg/dl). Renal involvement was characterized by nephritic and nephrotic syndrome. Autoantibody tests were positive for antinuclear antibodies and anti-double-stranded DNA, and complement levels were markedly reduced...
November 2018: Lupus
Can Hüzmeli, Ferhan Candan, Ayşe Şeker Koçkara, Demet Alaygut, Mansur Kayataş
Antiphospholipid syndrome (APS) is a disorder characterized by antiphospholipid antibody positivity, arterial or venous thrombosis, and fetal loss. In APS, renal as well as vascular and glomerular involvement is observed. Systemic lupus erythematosus and other connective tissue diseases should be excluded to diagnose primary APS. Immunoglobulin M (IgM) nephropathy is characterized by single or dominant IgM deposition in glomerular mesangium. It often presents with hematuria and proteinuria. In a 45-year-old female patient admitted to our clinic with diabetes mellitus and proteinuria, fundus examination did not reveal diabetic retinopathy but a high anticardiolipin IgM and venous thrombosis in the upper extremity were observed...
July 2018: European Journal of Rheumatology
S Brauner, M Ivanchenko, G E Thorlacius, A Ambrosi, M Wahren-Herlenius
Systemic rheumatic diseases are characterized by abnormal B cell activation with autoantibody production and hypergammaglobulinaemia. Ro52/SSA, also denoted tripartite motif (TRIM)21, is a major autoantigen in Sjögren's syndrome and systemic lupus erythematosus. Interestingly, TRIM21-deficient mice develop systemic autoimmunity with B cell-driven manifestations such as autoantibodies, hypergammaglobulinaemia and glomerulonephritis following tissue injury. The mechanisms by which Trim21-deficiency leads to enhanced B cell activation and antibody production are, however, not well understood, and to further elucidate the role of TRIM21 in systemic autoimmunity, we investigated the B cell phenotype and antibody responses of Trim21-/- mice following immunization with thymus-dependent (TD) and thymus-independent (TI) antigens...
September 3, 2018: Clinical and Experimental Immunology
Eric J Campbell, Ann E Clarke, Rosalind Ramsey-Goldman
Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder with systemic manifestations and multiorgan involvement. Although primarily diagnosed, and managed in the outpatient setting, it can occasionally present with life-threatening complications that require rapid assessment and urgent aggressive therapy. Areas covered: In our review, we explore three organ systems that are often affected in SLE, but have the potential to present as medical emergencies; these are the kidney, the central nervous system, and the hematologic system...
October 2018: Expert Review of Clinical Immunology
Manish R Balwani, Charulata Bawankule, Prakash Khetan, Vishal Ramteke, Priyanka Tolani, Vivek Kute
We report a case of systemic lupus erythematosus (SLE) who presented with rapidly progressive renal failure (RPRF) with positive antinuclear antibody (ANA) and anti-double-stranded DNA (dsDNA) antibody and active urinary sediment in the form of microscopic hematuria and proteinuria. Provisional clinical diagnosis of lupus nephritis was made. Renal biopsy showed pauci-immune crescentic glomerulonephritis, the diagnosis of which was supported by positive serum anti-MPO antibody. Renal biopsy in SLE patients can sometimes reveal varied pathological entities such as antinuclear cytoplasmic antibodies (ANCAs) positive vasculitis, as in our case, which modified our treatment protocol...
July 2018: Saudi Journal of Kidney Diseases and Transplantation
Meherzad Kutky, Sarah Aloudat
Rationale: Late-onset systemic lupus erythematosus (SLE) represents a specific subgroup of SLE, and although there is no strict age cut-off, 50 years is commonly used as the minimum age for disease onset. In this report, we present a case of a 74-year-old male with late-onset SLE and biopsy-proven lupus nephritis (LN). Presenting concerns of the patient: A 74-year-old male was referred to the nephrology clinic with a rapidly rising creatinine from a baseline of 60 µmol/L to 176 µmol/L...
2018: Canadian Journal of Kidney Health and Disease
M Ciszek, B Kisiel, J Czerwinski, E Hryniewiecka, D Lewandowska, S Borczon, W Tlustochowicz, L Paczek
Chronic kidney disease (CKD) is a common complication of rheumatic disorders. We analyzed the incidence of different rheumatic conditions as a primary diagnosis of end-stage renal disease (ESRD) in kidney transplant recipients in Poland. Data were received from the national waiting list for organ transplantation (Poltransplant) registries. Primary diagnosis leading to ESRD were analyzed in 15,984 patients who received kidney transplants between 1998 and 2015. There was no information about primary diagnosis in 4981 cases (31%) and in 1482 cases (9%) the diagnosis was described as unknown...
July 2018: Transplantation Proceedings
Debasis Pore, Emily Huang, Dina Dejanovic, Neetha Parameswaran, Michael B Cheung, Neetu Gupta
Genetic deletion of the Src family tyrosine kinase Lyn in mice recapitulates human systemic lupus erythematosus, characterized by hyperactive BCR signaling, splenomegaly, autoantibody generation, and glomerulonephritis. However, the molecular regulators of autoimmunity in Lyn-deficient mice and in human lupus remain poorly characterized. In this study, we report that conditional deletion of the membrane-cytoskeleton linker protein ezrin in B cells of Lyn-deficient mice (double knockout [DKO] mice) ameliorates B cell activation and lupus pathogenesis...
September 1, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Aram Musaelyan, Sergey Lapin, Vladimir Nazarov, Olga Tkachenko, Boris Gilburd, Alexandra Mazing, Lilia Mikhailova, Yehuda Shoenfeld
Vimentin is a protein of intermediate filament family, which is expressed in all mesenchymal cells. Vimentin plays a key role in the physiology of the cell, cellular interactions and the functioning of the immune system. Post-translationally modified and native forms of vimentin are involved in the pathogenesis of inflammation and many autoimmune diseases: rheumatoid arthritis, sarcoidosis, systemic lupus erythematosus, antiphospholipid syndrome, Crohn's disease, ankylosing spondyloarthritis and idiopathic pulmonary fibrosis...
September 2018: Autoimmunity Reviews
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
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
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