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systemic lupus , glomerulonephritis

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
Susanna Brauner, Margarita Ivanchenko, Gudny Ella Thorlacius, Aurelie Ambrosi, Marie Wahren-Herlenius
Systemic rheumatic diseases are characterised by abnormal B cell activation with autoantibody production and hypergammaglobulinemia. Ro52/SSA, also denoted TRIM21, 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, hypergammaglobulinemia 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 immunisation with thymus-dependent (TD) and thymus-independent (TI) antigens...
September 3, 2018: Clinical and Experimental Immunology
Eric J Campbell, Anne Clarke, Rosalind Ramsey-Goldman
Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disorder with systemic manifestations and multi-organ 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...
September 1, 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
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...
October 2018: Annals of the Rheumatic Diseases
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
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
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
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
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
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...
August 2018: Clinical and Experimental Dermatology
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...
October 2018: Rheumatology International
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...
August 2018: Annals of the Rheumatic Diseases
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
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