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C3 nephritis

Angelika Url, Verena Krutak, Anna Kübber-Heiss, Sonja Chvala-Mannsberger, Nadia Robert, Nora Dinhopl, Peter Schmidt, Chris Walzer
According to previous studies in captive cheetah ( Acinonyx jubatus ) populations, one of the most threatening diseases besides amyloidosis, myelopathy, veno occlusive disease, and gastritis, is renal failure. Contrary to captive cheetahs in North America and South Africa, morphological data concerning renal lesions in the cheetah European Endangered Species Program (EEP) are lacking. This study details the histological characterization as well as immunohistochemical and morphometrical analysis of nephropathies in 35 captive cheetahs from the EEP, which were necropsied between 1985 and 2003...
September 2016: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
Ranjan Gupta, Akhilesh Yadav, Amita Aggarwal
Urinary MCP-1 (uMCP-1) levels reflect lupus nephritis (LN) disease activity. However, long-term prospective studies evaluating it as a biomarker are lacking. SLE patients with active nephritis (AN), active disease without nephritis (ANR), and inactive disease (ID) were enrolled. AN patients were followed up every 3 months for 1 year. Urine and serum samples were collected at baseline from all and at follow-up visits in AN group. Urine samples from healthy subjects (HC), rheumatoid arthritis (RA), and diabetic nephropathy (DM) patients (20 each) served as controls...
November 2016: Clinical Rheumatology
Qingjun Pan, Linjie Guo, Jing Wu, Jun Cai, Huanjin Liao, Qiaofen Lan, Yanxia Peng, Yiming He, Hua-Feng Liu
In order to investigate the association between IgG4 autoantibody and complement abnormalities in systemic lupus erythematosus (SLE), 72 newly diagnosed SLE patients, 67 rheumatoid arthritis (RA) patients, and 41 healthy normals were employed. Serum levels of antinuclear IgG4 and IgG4-specific IgM-rheumatoid factor (RF) were measured, and the correlations between serum levels of antinuclear IgG4 and several clinical parameters were analyzed. Also, the levels of IgG subclasses, C1q, and C3 deposition in lupus nephritis (LN) were detected...
2016: Mediators of Inflammation
Samantha A Chalmers, Jing Wen, Justine Shum, Jessica Doerner, Leal Herlitz, Chaim Putterman
Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease that can affect multiple end organs. Kidney and brain are two of the organs most commonly involved in SLE. Past studies have suggested the importance of macrophages in the pathogenesis of lupus nephritis (LN). Furthermore, as the immune effectors of the brain, microglia have been implicated in pathways leading to neuropsychiatric SLE (NPSLE). We depleted macrophages and microglia using GW2580, a small colony stimulating factor-1 receptor (CSF-1R) kinase inhibitor, in MRL-lpr/lpr (MRL/lpr) mice, a classic murine lupus model that displays features of both LN and NPSLE...
August 25, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Huai-Chia Chuang, Yi-Ming Chen, Wei-Ting Hung, Ju-Pi Li, Der-Yuan Chen, Joung-Liang Lan, Tse-Hua Tan
Systemic lupus erythematosus (SLE) is a complex autoimmune disease that is characterized by systemic inflammation and multiple organ failures. Dysregulation of T cells plays a critical role in SLE pathogenesis. Our previous study indicates that JKAP (also named DUSP22) inhibits T-cell activation and that JKAP knockout mice develop spontaneous autoimmunity; therefore, we investigated whether JKAP downregulation is involved in SLE patients. JKAP protein levels in purified T cells were examined by immunoblotting using blood samples from 43 SLE patients and 32 healthy controls...
August 19, 2016: Oncotarget
Sveta Janković, Marijana Nikolić, Aleksandra Simović, Ana Vujić
INTRODUCTION: Henoch-Schönlein purpura (HSP) is a small blood vessel vasculitis, which usually manifests during childhood. The exact cause of the disease is unknown. CASE REPORT: We reported a 14-year-old girl who had been admitted to our clinic due to the appearance of red macules on her extremities and face, vomiting, and pain in the abdomen and joints. The patient was initially diagnosed with Henoch-Schönlein purpura. At the end of the fourth week of illness, larvae of Strongyloides stercoralis were detected in stool samples...
May 2016: Vojnosanitetski Pregled. Military-medical and Pharmaceutical Review
Gabriella Moroni, Andrea Doria, Elisa Giglio, Enrico Imbasciati, Chiara Tani, Margherita Zen, Francesca Strigini, Barbara Zaina, Angela Tincani, Mariele Gatto, Federica de Liso, Claudia Grossi, Pier Luigi Meroni, Gianfranca Cabiddu, Piergiorgio Messa, Pietro Ravani, Marta Mosca
Retrospective studies reported a high incidence of maternal complications in pregnant women with lupus. In this paper we prospectively assessed the rate of risk and the risk factors of maternal outcome in women with stable lupus nephritis who received pre-pregnancy counseling. This prospective multicenter study includes 71 pregnancies in 61 women with lupus nephritis who became pregnant between 2006 and 2013. Complete renal remission was present before pregnancy in 56 cases (78.9%) and mild active nephritis in 15 cases...
June 30, 2016: Journal of Autoimmunity
Joshua M Thurman, Carla M Nester
The complement (C) cascade is an ancient system of proteins whose primary role is to initiate and modulate immune responses. During C activation, circulating proteins are cleaved and nascent cleavage fragments participate in a broad range of downstream innate and adaptive immune functions. Although the majority of these functions are either homeostatic or protective, a large body of experimental and clinical evidence also highlights a central role for the C system in the pathogenesis of many types of glomerular disease...
June 23, 2016: Clinical Journal of the American Society of Nephrology: CJASN
Yuko Sakuma, Tatsuo Nagai, Taku Yoshio, Shunsei Hirohata
OBJECTIVE: To explore the role of C5a in the pathogenesis of neuropsychiatric systemic lupus erythematosus (NPSLE) and lupus nephritis (LN). METHODS: Sera were obtained from 29 patients with NPSLE, 25 with LN, 26 without NPSLE or LN [SLE alone], and 21 healthy donors. Cerebrospinal fluid (CSF) was obtained from 29 NPSLE patients. C5a and C5 were measured by ELISA. Blood-brain barrier (BBB) function was evaluated by Q albumin ([CSF albumin/serum albumin] × 10(3))...
June 20, 2016: Modern Rheumatology
Èlia Ripoll, Laura de Ramon, Juliana Draibe Bordignon, Ana Merino, Nuria Bolaños, Montse Goma, Josep M Cruzado, Josep M Grinyó, Juan Torras
BACKGROUND: Lupus nephritis (LN) is a complex chronic autoimmune disease of unknown etiology characterized by loss of tolerance against several self-antigens. Cytokines are known to be central players in LN pathogenesis. The Janus kinase-signal transducer and activator of transcription (JAK-STAT) pathway is one important pathway that mediates signal transduction of several cytokines. In this study, we examined the pathogenic role of this pathway and how CP-690,550 treatment influences LN outcome...
2016: Arthritis Research & Therapy
R Nada, A Kumar, V G Kumar, K L Gupta, K Joshi
Renal biopsy interpretation requires histopathology, direct immunofluorescence (DIF) and electron microscopy. Formalin-fixed, paraffin-embedded tissue (FFPE) sent for light microscopy can be used for DIF after antigen retrieval. However, complement staining has not been satisfactory. We standardized DIF using proteinase-K for antigen retrieval in FFPE renal biopsies. A pilot study was conducted on known cases of membranous glomerulonephritis (MGN), membranoproliferative type-1 (MPGN-1), immunoglobulin A nephropathy (IgAN), and anti-glomerular basement disease (anti-GBM)...
May 2016: Indian Journal of Nephrology
Samantha A Chalmers, Jessica Doerner, Todd Bosanac, Sara Khalil, Dustin Smith, Christian Harcken, Janice Dimock, Evan Der, Leal Herlitz, Deborah Webb, Elise Seccareccia, Di Feng, Jay S Fine, Meera Ramanujam, Elliott Klein, Chaim Putterman
Lupus nephritis (LN) is a potentially dangerous end organ pathology that affects upwards of 60% of lupus patients. Bruton's tyrosine kinase (BTK) is important for B cell development, Fc receptor signaling, and macrophage polarization. In this study, we investigated the effects of a novel, highly selective and potent BTK inhibitor, BI-BTK-1, in an inducible model of LN in which mice receive nephrotoxic serum (NTS) containing anti-glomerular antibodies. Mice were treated once daily with vehicle alone or BI-BTK-1, either prophylactically or therapeutically...
2016: Scientific Reports
Martine T P Besouw, Johan G Vande Walle, Mohamad I Ilias, Ann M Raes, Agnieszka A Prytula, Lieve Claeys, Jo L Dehoorne
A 12-year-old Congolese girl presented with acute renal failure, edema, hypertension, hemoptysis, hematuria, and proteinuria after a history of throat infection. Renal ultrasound showed kidneys of normal size, with increased echogenicity of the cortical parenchyma and decreased corticomedullary differentiation. Other additional investigations showed pancytopenia with decreased complement (low C3 and C4). Antinuclear antibodies were strongly positive, including anti-double stranded DNA. Renal biopsy confirmed severe grade IV lupus nephritis...
May 12, 2016: Acta Clinica Belgica
Soltysiak Jolanta, Zachwieja Jacek, Benedyk Anna, Lewandowska-Stachowiak Maria, Nowicki Michal, Ostalska-Nowicka Danuta
BACKGROUND: The increase of circulating urokinase plasminogen activator receptor (suPAR) was demonstrated in various diseases showing its prognostic value as well as the link to the inflammatory reaction. In glomerular diseases suPAR was consider to be a causative factor of proteinuria. In present study we aimed to evaluate serum concentration of suPAR in children with primary and secondary glomerulonephritis (GN) and its association with disease severity. METHODS: The study involved 22 children with minimal change disease (MCD), 9 with primary focal segmental glomerulosclerosis (FSGS), 7 with Henoch-Schönlein nephritis (HSN), 7 with lupus nephritis (LN) and 16 controls...
April 12, 2016: Minerva Pediatrica
Shuhong Chi, Jing Xue, Feng Li, Caixia Zhu, Yunxia Yu, Haibo Li, Xuemei Wang, Yurong Zhang, Jijuan Yang, Shaolan Zhou, Lijuan Yang, Chen Ji, Xiaoming Liu
Background. Serum concentrations of soluble interleukin-7 receptor (sIL-7R) and anti-C1q antibody have recently been identified as unique serological markers for lupus nephritis (LN) in patients with systemic lupus erythematosus (SLE). In this study, we evaluated the correlation of serum sIL-7R and anti-C1q in SLE patients. Methods. Sera from 134 patients with SLE and 84 healthy cohorts were tested for levels of sIL-7R and anti-C1q antibodies in terms of ELISA. Correlations of the sIL-7R and anti-C1q autoantibodies were evaluated...
2016: Autoimmune Diseases
Mari Okada, Mai Sato, Masao Ogura, Koichi Kamei, Kentaro Matsuoka, Shuichi Ito
BACKGROUND: Advances in long-term parenteral nutrition via indwelling central venous catheter have improved the quality of life and mortality in patients with life-threatening gastrointestinal diseases complicated with severely impaired absorption. However, infection to central venous catheter is still a common and critical complication for such patients. We encountered two patients under long-term parenteral nutrition who developed glomerulonephritis associated with central venous catheter infection...
2016: BMC Research Notes
Stephen R Holdsworth, Poh-Yi Gan, A Richard Kitching
Biological therapeutics (biologics) that target autoimmune responses and inflammatory injury pathways have a marked beneficial impact on the management of many chronic diseases, including rheumatoid arthritis, psoriasis, inflammatory bowel disease, and ankylosing spondylitis. Accumulating data suggest that a growing number of renal diseases result from autoimmune injury - including lupus nephritis, IgA nephropathy, anti-neutrophil cytoplasmic antibody-associated glomerulonephritis, autoimmune (formerly idiopathic) membranous nephropathy, anti-glomerular basement membrane glomerulonephritis, and C3 nephropathy - and one can speculate that biologics might also be applicable to these diseases...
April 2016: Nature Reviews. Nephrology
R Gupta, A Aggarwal, S Sinha, L Rajasekhar, A Yadav, P Gaur, R Misra, V S Negi
OBJECTIVES: Urinary biomarkers may help in identification, treatment and assessment of response in patients with lupus nephritis (LN). Osteoprotegerin (OPG) is produced by the kidneys and lymphoid cells and may reflect renal disease activity better. The data on its utility are sparse. METHODS: Fifty-eight patients with active LN (AN), 24 with active non-renal disease (ANR) and 39 with inactive disease (ID) were included. Median disease duration was 32 (1-204) months and median age was 27 (12-50) years...
October 2016: Lupus
Thomas D Barbour, Guang Sheng Ling, Marieta M Ruseva, Liliane Fossati-Jimack, H Terence Cook, Marina Botto, Matthew C Pickering
C3 glomerulopathy is a complement-mediated renal disease that is frequently associated with abnormalities in regulation of the complement alternative pathway. Mice with deficiency of factor H (Cfh(-/-)), a negative alternative pathway regulator, are an established experimental model of C3 glomerulopathy in which complement C3 fragments including iC3b accumulate along the glomerular basement membrane. Here we show that deficiency of complement receptor 3 (CR3), the main receptor for iC3b, enhances the severity of spontaneous renal disease in Cfh(-/-) mice...
April 2016: Kidney International
Juliana B Mansur, Amélia R P Sabino, Sonia K Nishida, Gianna Mastroianni Kirsztajn
OBJECTIVE: To establish the occurrence and intensity of podocyturia and its relation to grade of disease activity, as defined by clinical and laboratory criteria. METHODS: Prospective, cross-sectional study involving 50 patients with lupus nephritis and 29 controls, which had podocyturia levels determined from random urine samples using an immunofluorescence technique. Disease activity was graded by BILAG (renal criteria) and an additional system used in the service (S2)...
2016: Renal Failure
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