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C3 nephritis

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https://www.readbyqxmd.com/read/28647487/does-pregnancy-have-any-impact-on-long-term-damage-accrual-and-on-the-outcome-of-lupus-nephritis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/28378099/increased-serum-sfas-strail-and-reduced-sfasl-in-juvenile-onset-systemic-lupus-erythematosus
#13
Bernadete L Liphaus, Maria Helena B Kiss, Solange Carrasco, Patrícia Palmeira, Claudia Goldenstein-Schainberg, Magda Carneiro-Sampaio
The aims of this study were to assess serum Fas, FasL, TRAIL, and Bcl-2 levels in patients with juvenile-onset systemic lupus erythematosus (JSLE) and to evaluate their relations with disease activity parameters and nephritis. Forty-eight JSLE patients, 33 juvenile idiopathic arthritis (JIA, inflammatory controls) patients and 40 healthy controls were enrolled. sFas, sFasL, sTRAIL, and sBcl-2 serum levels were measured by ELISA. Disease activity parameters included SLEDAI score, ESR, anti-dsDNA antibodies, C3, and C4 levels...
April 4, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28375199/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-in-childhood-onset-systemic-lupus-erythematosus-patients-a-multicenter-study
#14
Ana Paula Sakamoto, Clovis Artur Silva, Claudia Saad-Magalhães, Aline Nicácio Alencar, Rosa Maria Rodrigues Pereira, Katia Kozu, Cassia Maria Passarelli Lupoli Barbosa, Maria Teresa Terreri
OBJECTIVE: To assess Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in a large population of childhood-onset systemic lupus erythematosus (cSLE) patients. METHODS: Multicenter study including 852 cSLE patients followed in Pediatric Rheumatology centers in São Paulo, Brazil. SJS was defined as epidermal detachment below 10% of body surface area (BSA), overlap SJS-TEN 10-30% and TEN greater than 30% of BSA. RESULTS: SJS and TEN was observed in 5/852 (0...
March 26, 2017: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28373995/dickkopf-1-is-a-biomarker-for-systemic-lupus-erythematosus-and-active-lupus-nephritis
#15
Jing Xue, Jiali Yang, Lijuan Yang, Shaolan Zhou, Chen Ji, Xuemei Wang, Nan Yu, Xiaoming Liu, Shuhong Chi
An early diagnosis of lupus nephritis (LN) has an important clinical implication in guiding treatments of systemic lupus erythematosus (SLE) in clinical settings. In this study, the concentrations of Wnt-3A, Frizzled-8 (FZD-8), and Dickkopf-1 (DKK-1) of Wnt signaling, as well as their diagnostic values for accessing LN, were evaluated by ELISA in sera and urine of 111 SLE patients (31 with LN and 80 without LN) and 70 healthy cohorts. Significantly more abundances of DKK-1 protein were determined in both of sera and urine of SLE patients compared to healthy cohorts (p < 0...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28343355/anti-c1q-autoantibodies-as-markers-of-renal-involvement-in-childhood-onset-systemic-lupus-erythematosus
#16
Cécile Picard, Jean-Christophe Lega, Bruno Ranchin, Pierre Cochat, Natalia Cabrera, Nicole Fabien, Alexandre Belot
BACKGROUND: Childhood-onset systemic lupus erythematosus (cSLE) is rare, and considered more severe than its adult-onset counterpart. Lupus nephritis (LN) occurs more frequently in children, accounting for higher long-term morbidity and mortality compared with adults. Thus, reliable biological markers are needed to predict disease course. This study aimed to investigate the capacity of anti-C1q autoantibodies (Abs) to predict renal flare and global disease activity in cSLE patients, and association with disease activity and kidney involvement...
March 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28341889/complement-regulation-and-kidney-diseases-recent-knowledge-of-the-double-edged-roles-of-complement-activation-in-nephrology
#17
REVIEW
Masashi Mizuno, Yasuhiro Suzuki, Yasuhiko Ito
The complement activation system plays important roles to maintain homeostasis in the host and to fight foreign invaders to protect the host. Therefore, the complement system is considered a core part of innate immunity which also cross-talks to acquired immunity. In the history of nephrology, the complement system is familiar to us, because complement protein or fragment deposition, including C3, C4, C1q, and/or C4d, is routinely estimated by immunohistochemistry to diagnose renal pathologies. The relationships between pathological mechanisms and complement activation have been investigated for renal diseases such as post-infectious glomerulonephritis, lupus nephritis, and primary membranoproliferative glomerulonephritis, which are usually accompanied by hypocomplementemia...
March 24, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28334051/dissecting-the-relationships-of-igg-subclasses-and-complements-in-membranous-lupus-nephritis-and-idiopathic-membranous-nephropathy
#18
Woong Na, Kijong Yi, Young Soo Song, Moon Hyang Park
Membranous lupus nephritis (MLN) and idiopathic membranous nephropathy (IMN) are kidney diseases with similar morphology, but distinct etiologies, both producing glomeruli with immune deposits. Immunoglobulins and complements, the main components of the deposits, can be detected by immunofluorescence (IF) microscopy. Previous researches characterized the immune deposits only individually, but not the interactions between them. To study these relationships we analyzed an IF profile of IgG subclasses and complements (IgG1, IgG2, IgG3, IgG4, C3, C1q, and C4) in 53 and 95 cases of biopsy-confirmed MLNs and IMNs, respectively, mainly using information theory and Bayesian networks...
2017: PloS One
https://www.readbyqxmd.com/read/28320458/b-cell-epitope-of-human-cytomegalovirus-phosphoprotein-65-hcmv-pp65-induced-anti-dsdna-antibody-in-balb-c-mice
#19
Ao HoHsieh, Chin Man Wang, Yeong-Jian Jan Wu, Albert Chen, Ming-I Chang, Ji-Yih Chen
BACKGROUND: HCMV phosphoprotein 65 (HCMVpp65) is a putative immunogen that acts as an accelerator, inducing autoantibody and exacerbating autoimmune response in susceptible animals. The immunity to pp65336-439 instigates autoimmunity, suggesting that pp65336-439 contains crucial B cell epitope(s) for the development of nephritis. This study narrowed down the target epitope to pp65422-439 for immunization of BALB/c mice and mapping of B cell epitope. METHODS: The target epitope pp65422-439 reactivity and B cell epitope mapping was examined in serum from pp65422-439-immunized mice and patients with systemic lupus erythematosus (SLE)...
March 21, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28281463/what-is-the-clinical-significance-of-anti-sm-antibodies-in-systemic-lupus-erythematosus-a-comparison-with-anti-dsdna-antibodies-and-c3
#20
Alexandra Flechsig, Thomas Rose, Fidan Barkhudarova, Romy Strauss, Jens Klotsche, Cornelia Dähnrich, Wolfgang Schlumberger, Philipp Enghard, Gerd-Rüdiger Burmester, Falk Hiepe, Robert Biesen
OBJECTIVES: To investigate the clinical value of anti-Sm antibodies in diagnosis and monitoring of systemic lupus erythematosus (SLE) and their ability to predict lupus flares compared with that of anti-dsDNA antibody and complement (C3) assays. METHODS: Autoantibodies against Smith antigen (Sm) and double-stranded DNA (dsDNA) in sera from SLE (n=232), myositis (n=26), systemic sclerosis (n=81), Sjögren's syndrome (n=88), and rheumatoid arthritis patients (n=165) and healthy donors (n=400) were determined by using enzyme-linked immunosorbent assays (both from Euroimmun)...
March 3, 2017: Clinical and Experimental Rheumatology
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