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C3 nephritis

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https://www.readbyqxmd.com/read/28880016/-5r-5-hydroxytriptolide-ameliorates-anti-glomerular-basement-membrane-glomerulonephritis-in-nzw-mice-by-regulating-fc%C3%AE-receptor-signaling
#1
Qing Qi, Heng Li, Ze-Min Lin, Xiao-Qian Yang, Feng-Hua Zhu, Yu-Ting Liu, Mei-Juan Shao, Lu-Yao Zhang, Yan-Sheng Xu, Yu-Xi Yan, Lan-Lan Sun, Shi-Jun He, Wei Tang, Jian-Ping Zuo
(5R)-5-hydroxytriptolide (LLDT-8) is a novel triptolide analog that has been identified as a promising candidate for treating autoimmune diseases and has been shown to be effective in treating murine collagen-induced arthritis and lupus nephritis. In the present study, we investigated the therapeutic effect and possible mechanism of action of LLDT-8 in a murine anti-glomerular basement membrane (GBM) glomerulonephritis model. NZW mice were injected with rabbit anti-GBM serum (500 μL, ip). The mice were orally treated with LLDT-8 (0...
September 7, 2017: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/28830653/recombinant-human-igg1-based-fc-multimers-with-limited-fcr-binding-capacity-can-effectively-inhibit-complement-mediated-disease
#2
Haoping Sun, Henrik S Olsen, Emmanuel Y Mérigeon, Edward So, Erin Burch, Susan Kinsey, John C Papadimitriou, Cinthia B Drachenberg, Søren M Bentzen, David S Block, Scott E Strome, Xiaoyu Zhang
There is a lack of effective targeted therapies for the treatment of complement dependent diseases. We developed two recombinant Fc multimers, G207 and G211, with limited ability to interact with low/moderate affinity FcγRs, but with high avidity for C1q. These drugs effectively inhibited complement dependent cytotoxicity (CDC) in vitro, and prevented the deposition of C1q, C3b and MAC, on the surface of Ab-opsonized cells. Importantly, these inhibitory effects were both C1q dependent and independent. In order to determine the biologic relevance of our findings, we evaluated the clinical efficacy of these drugs in three different animal models, acute RBC hemolysis, anti-Thy-1 nephritis and passive Heymann's nephropathy (PHN), in which disease pathophysiology relies preferentially on complement activation...
August 19, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28757276/value-of-immunohistochemical-expression-of-podocalyxin-in-active-lupus-nephritis
#3
Maha Abdelmoneim Behairy, Marwa Mosaad Shakweer, Tamer Wahid El Said, Nermin Hassan ElGharbawy
Podocalyxin is an electronegative sialoglycoprotein that prevents the podocyte foot process from collapsing. The aim of this study was to detect an association between the glomerular immunohistochemical (IHC) expression of podocalyxin and the degree of podocyte effacement detected by electron microscopy, and to evaluate the role of podocalyxin IHC expression as a novel marker for disease activity in lupus nephritis (LN). METHODS: Thirty-two renal biopsies of active lupus nephritis patients were studied...
July 27, 2017: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/28753077/low-dose-cyclosporine-a-in-the-treatment-of-resistant-proliferative-lupus-nephritis
#4
Marjan Sheikholeslami, Mehrzad Hajialilo, Seyed Sadreddin Rasi Hashemi, Aida Malek Mahdavi, Morteza Gojazadeh, Alireza Khabbazi
OBJECTIVE: This study aimed to evaluate long-term efficacy of low dose cyclosporine A (CsA) in the treatment of resistant proliferative lupus nephritis. METHODS: In this retrospective study, patients with biopsy proven proliferative lupus nephritis who were unresponsive to combination therapy with steroid plus mycophenolate mofetil (MMF) or cyclophosphamide (CYC) and had been treated with CsA were included. Efficacy monitoring was based on the systemic lupus erythematosus (SLE) disease activity index, dose of prednisolone, serum complement, anti-double stranded DNA (anti-dsDNA) titration, urine analysis, proteinuria, creatinine clearance, remission of the renal disease, renal survival and involvement of other organs...
July 28, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28745692/-changes-in-the-complement-system-in-membranoproliferative-glomerulonephritis
#5
V A Yurova, L A Bobrova, N L Kozlovskaya, Yu V Korotchaeva, A G Serova, L V Kozlov, S S Andina, K A Demyanova, A M Kuchieva, S V Roshchupkina
AIM: To compare the clinical manifestations membranoproliferative glomerulonephritis (MPGN) in its idiopathic variant, lupus nephritis (LN), and C3 glomerulopathy (C3-GP), by comparing them with changes in the complement system. SUBJECTS AND METHODS: The clinic of nephrology followed up 42 patients with different types of MPGN in 2013 to 2015. The study included 35 patients divided into 3 groups: 1) 8 patients with C3-GP, 2) 13 with idiopathic MPGN; 3) 14 with Class IV LN...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28732643/development-and-internal-validation-of-a-prediction-model-to-estimate-the-probability-of-needing-aggressive-immunosuppressive-therapy-with-cytostatics-in-de-novo-lupus-nephritis-patients
#6
Mauricio Restrepo-Escobar, Paula Andrea Granda-Carvajal, Fabián Jaimes
OBJECTIVE: To develop a multivariable clinical prediction model for the requirement of aggressive immunosuppression with cytostatics, based on simple clinical record data and lab tests. The model is defined in accordance with the result of the kidney biopsies. METHODS: Retrospective study conducted with data from patients 16 years and older, with SLE and nephritis with less than 6 months of evolution. An initial bivariate analysis was conducted to select the variables to be included in a multiple logistic regression model...
July 18, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28683778/unusual-acute-lupus-hemophagocytic-syndrome-a-test-of-diagnostic-criteria-a-case-report
#7
Wijetunga Mudalige Udai Akalanka Wijetunga, Ravindra Laxman Satarasinghe, Balasuriya Mudiyanselage Dayananda, Ganhewage Kokila Darshani
BACKGROUND: Hemophagocytic lymphohistiocytosis is an aggressive life-threatening syndrome of excessive immune activation. Hemophagocytic lymphohistiocytosis due to systemic lupus erythematosus is described as acute lupus hemophagocytic syndrome. Acute lupus hemophagocytic syndrome presenting with negative antinuclear antibody is uncommon. CASE PRESENTATION: A 57-year-old Sri Lankan woman presented with intermittent fever, weight loss, episodic confusion, and alopecia for 3 months...
July 7, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28658762/role-of-immunofluorescence-in-adult-onset-nephrotic-syndrome-a-study-in-a-tertiary-care-centre-of-western-india
#8
Sharada Rane, Prerana Mutyal, Nicholas Dcunha, Mayur Parkhi, Meenal Jadhav
INTRODUCTION: Study of renal Immunofluorescence (IF) is an ancillary but essential technique in evaluation of renal biopsies in glomerulopathies and also it enlightens on the pathogenesis of nephrotic syndrome. AIM: To determine the role of IF in evaluating definite subtyping and diagnosis of adult onset nephrotic syndrome and attempting clinicopathological correlation. MATERIALS AND METHODS: A total of 52 patients of adult onset nephrotic syndrome were evaluated clinically and with pertinent investigations; and subjected to USG guided percutaneous renal biopsy which was processed and stained for light microscopy and for immunofluorescence by direct method (DIF) using antibodies against IgG, IgM, IgA and C3...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28647487/does-pregnancy-have-any-impact-on-long-term-damage-accrual-and-on-the-outcome-of-lupus-nephritis
#9
Davide Gianfreda, Silvana Quaglini, Giulia Frontini, Francesca Raffiotta, Piergiorgio Messa, Gabriella Moroni
No data are available about the impact of pregnancy on the long-term outcome of lupus nephritis. Thirty-two women with lupus nephritis with a 10-year follow-up after their first pregnancy ("women who gave birth") and 64 matched controls with the same follow-up and who never had pregnancies ("controls") were compared for the occurrence of SLE flares, chronic kidney disease (CKD), and SLICC/ACR Damage Index (SDI) in the post pregnancy period. The same evaluations were done before and after pregnancy in women who gave birth...
June 21, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28626064/monitoring-c3ar-expression-using-a-floxed-tdtomato-c3ar-reporter-knock-in-mouse
#10
Katharina M Quell, Christian M Karsten, Anna Kordowski, Larissa Nogueira Almeida, Daria Briukhovetska, Anna V Wiese, Jing Sun, Fanny Ender, Konstantina Antoniou, Torsten Schröder, Inken Schmudde, Johann L Berger, Peter König, Tillman Vollbrandt, Yves Laumonnier, Jörg Köhl
C3a exerts multiple biologic functions through activation of its cognate C3a receptor. C3(-/-) and C3aR(-/-) mice have been instrumental in defining important roles of the C3a/C3aR axis in the regulation of acute and chronic inflammatory diseases, including ischemia/reperfusion injury, allergic asthma, autoimmune nephritis, and rheumatoid arthritis. Surprisingly little is known about C3aR expression and function in immune and stromal cells. To close this gap, we generated a floxed tandem-dye Tomato (tdTomato)-C3aR reporter knock-in mouse, which we used to monitor C3aR expression in cells residing in the lung, airways, lamina propria (LP) of the small intestine, brain, visceral adipose tissue, bone marrow (BM), spleen, and the circulation...
June 16, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28614243/different-types-of-glomerulonephritis-associated-with-the-dysregulation-of-the-complement-alternative-pathway-in-2-brothers-a-case-report
#11
Pei Chen, Li Zhu, Feng Yu, Sha-Sha Han, Si-Jun Meng, Wei-Yi Guo, Hong Zhang, Yan Song
RATIONALE: C3 glomerulonephritis (C3GN) and complement-mediated hemolytic uremic syndrome (HUS) both result from the abnormal regulation of the complement system. A significant number of patients with C3GN or complement-mediated HUS have mutations of more than 1 complement protein. This discovery has had a major impact on identifying the underlying cause of familial C3GN or complement-mediated HUS. PATIENT CONCERNS: We report the cases of 2 brothers (herein referred to as patient II-1 and patient II-9), both with complement disorders that differed in their clinical and genetic features...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28612243/increased-expression-of-bruton-s-tyrosine-kinase-in-peripheral-blood-is-associated-with-lupus-nephritis
#12
Wei Kong, Wei Deng, Yue Sun, Saisai Huang, Zhuoya Zhang, Bingyu Shi, Weiwei Chen, Xiaojun Tang, Genhong Yao, Xuebing Feng, Lingyun Sun
Systemic lupus erythematosus (SLE) is an autoimmune disease manifested by multiorgan impairment. It is reported that B cells participate in the onset of SLE. Bruton's tyrosine kinase (Btk), as a downstream signaling molecule of B cell antigen receptor (BCR) signaling pathway, is involved in the development, activation, and survival of B cells. The aim of our study was to explore the specific role of Btk in lupus nephritis (LN). We determined the percentages of Btk+ B cells in peripheral blood mononuclear cells (PBMCs) from SLE patients by flow cytometry and analyzed the correlation between the percentage of Btk+ B cells and lupus-related clinical indexes...
June 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28540909/unusual-presentation-of-systemic-lupus-erythematosus
#13
E Mahesh, Parampalli Rakesh Madhyastha, Vijay Varma, K C Gurudev, M S Gireesh, Sujeeth Reddy Bande
Bullous systemic lupus erythematosus is a rare distinctive subepidermal blistering disorder that can occur in patients with systemic lupus erythematosus (SLE). It is histologically characterized by a neutrophil-predominant infiltrate in the subepidermal region with deposition of immunoglobulins (IgG, IgA, IgM), C3, and auto-antibodies against collagen VII. Herein, we report a case of a 13-year-old girl who presented with bullous SLE and Class III lupus nephritis, which is extremely rare at this age.
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28491039/daily-moderate-exercise-is-beneficial-and-social-stress-is-detrimental-to-disease-pathology-in-murine-lupus-nephritis
#14
Saba I Aqel, Jeffrey M Hampton, Michael Bruss, Kendra T Jones, Giancarlo R Valiente, Lai-Chu Wu, Matthew C Young, William L Willis, Stacy Ardoin, Sudha Agarwal, Brad Bolon, Nicole Powell, John Sheridan, Naomi Schlesinger, Wael N Jarjour, Nicholas A Young
Daily moderate exercise (DME) and stress management are underemphasized in the care of patients with lupus nephritis (LN) due to a poor comprehensive understanding of their potential roles in controlling the inflammatory response. To investigate these effects on murine LN, disease progression was monitored with either DME or social disruption stress (SDR) induction in NZM2410/J mice, which spontaneously develop severe, early-onset LN. SDR of previously established social hierarchies was performed daily for 6 days and DME consisted of treadmill walking (8...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28487790/delayed-manifestation-of-shunt-nephritis-a-case-report-and-review-of-the-literature
#15
Michael Babigumira, Benjamin Huang, Sherry Werner, Wajeh Qunibi
We present an unusual case of shunt nephritis in a 39-year-old male who presented 21 years after placement of a ventriculoperitoneal (VP) shunt. He complained of fevers, headaches, dizziness, and urticarial plaques on arms, trunks, and legs and was found to have anemia, low complement levels, elevated serum creatinine, proteinuria, and new onset microhematuria. Blood and urine cultures were negative. Renal biopsy showed features of acute tubulointerstitial nephritis attributed to vancomycin use. Glomeruli showed increased mesangial hypercellularity and segmental endocapillary proliferation...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28480787/clinical-and-immunological-characteristics-of-150-systemic-lupus-erythematosus-patients-in-jamaica-a-comparative-analysis
#16
K C Maloney, T S Ferguson, H D Stewart, A A Myers, K De Ceulaer
Background Epidemiological studies in systemic lupus erythematosus have been reported in the literature in many countries and ethnic groups. Although systemic lupus erythematosus in Jamaica has been described in the past, there has not been a detailed evaluation of systemic lupus erythematosus patients in urban Jamaica, a largely Afro-Caribbean population. The goal of this study was to describe the clinical features, particularly disease activity, damage index and immunological features, of 150 systemic lupus erythematosus subjects...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28426384/a-2-year-old-boy-with-circulatory-failure-owing-to-streptococcal-toxic-shock-syndrome-case-report
#17
Werner Keenswijk, Johan Vande Walle
A 2-year-old boy presented with severe hypotension and acute kidney injury after a prodrome of non-bloody diarrhoea and fever in the preceding 3 days. He had a mild Ebstein cardiac anomaly but otherwise a normal past history and growth. On examination, he looked ill, his temperature was 37.5 °C, circulation was poor, and there were several purpuric lesions on the face, hands and scrotum. Haemoglobin was 7.8 g/dL (11-14), total white cell count 27 × 10(9)/L, platelets 62 × 10(9)/L, blood urea nitrogen 20...
April 20, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28403670/genetic-variants-in-fh-are-associated-with-renal-histopathologic-subtypes-of-lupus-nephritis-a-large-cohort-study-from-china
#18
M Tan, J-B Hao, H Chu, F-M Wang, D Song, L Zhu, F Yu, Y-Z Li, Y Song, M-H Zhao
Objective Genetic variants in FH (complement factor H) were reported to associate with susceptibility to systemic lupus erythematosus (SLE). This study proposed that the genetic defects of FH in the susceptibility and in the development of lupus nephritis might be different. Methods This study enrolled 334 lupus nephritis patients, 269 SLE patients without clinical renal involvement and 350 controls. Two-step genotyping was performed. First, all the exons of the FH gene were fully sequenced in 100 lupus nephritis patients and 100 healthy controls...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28400421/kidney-outcomes-and-risk-factors-for-nephritis-flare-de-novo-in-a-multiethnic-cohort-of-pregnant-patients-with-lupus
#19
Jill P Buyon, Mimi Y Kim, Marta M Guerra, Sifan Lu, Emily Reeves, Michelle Petri, Carl A Laskin, Michael D Lockshin, Lisa R Sammaritano, D Ware Branch, T Flint Porter, Allen Sawitzke, Joan T Merrill, Mary D Stephenson, Elisabeth Cohn, Jane E Salmon
BACKGROUND AND OBJECTIVES: Kidney disease is a critical concern in counseling patients with lupus considering pregnancy. This study sought to assess the risk of renal flares during pregnancy in women with previous lupus nephritis in partial or complete remission, particularly in those with antidouble-stranded DNA antibodies and low complement levels, and the risk of new-onset nephritis in patients with stable/mildly active SLE. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We assessed active nephritis (renal flares and de novo kidney disease) and associated predictors during pregnancy in patients with lupus with urine protein ≤1000 mg and serum creatinine <1...
June 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28385126/do-classic-blood-biomarkers-of-jsle-identify-active-lupus-nephritis-evidence-from-the-uk-jsle-cohort-study
#20
E M D Smith, A L Jorgensen, M W Beresford
Background Lupus nephritis (LN) affects up to 80% of juvenile-onset systemic lupus erythematosus (JSLE) patients. The value of commonly available biomarkers, such as anti-dsDNA antibodies, complement (C3/C4), ESR and full blood count parameters in the identification of active LN remains uncertain. Methods Participants from the UK JSLE Cohort Study, aged <16 years at diagnosis, were categorized as having active or inactive LN according to the renal domain of the British Isles Lupus Assessment Group score...
January 1, 2017: Lupus
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