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C3 nephritis

Saishree Pradhan, Sriram Krishnamurthy, Barath Jagadisan, Nachiappa G Rajesh, Subashini Kaliaperumal, Suryaprakash Ramasamy, Nandeeswari Subramanian
A 12-year-old girl born to third-degree consanguineous parents presented with recurrent episodes of haematuria for 8 months in association with peri-orbital and lower limb oedema for 20 days. There was no jaundice, hepatomegaly or neurological abnormality at presentation. An older brother had died following jaundice at 10 years of age. Urinalysis showed multiple dysmorphic erythrocytes without proteinuria and there was leucopenia, thrombocytopenia and hypo-albuminaemia (23 g/L). C3 component of complementaemia was low and anti-nuclear antibodies and anti-double-stranded DNA antibodies were strongly positive by immunofluorescence...
March 12, 2018: Paediatrics and International Child Health
Ryo Mukai, Yoko Okunuki, Deeba Husain, Clifford B Kim, John D Lambris, Kip M Connor
The complement system is a key component of innate immunity comprised of soluble components that form a proteolytic cascade leading to the generation of effector molecules involved in cellular clearance. This system is highly activated not only under general inflammatory conditions such as infections, collagen diseases, nephritis, and liver diseases, but also in focal ocular diseases. However, little is known about the role of the complement system in retinal homeostasis during aging. Using young (6-week-old) and adult (6-month-old) mice in wild type (C57BL/6) and complement knockout strains ( C1q -/- , Mbl a/c -/- , Fb -/- , C3 -/- , and C5 -/- ), we compared amplitudes of electroretinograms (ERG) and thicknesses of retinal layers in spectral domain optical coherence tomography between young and adult mice...
2018: Frontiers in Aging Neuroscience
Min-Hua Tseng, Shih-Hua Lin, Chao-Yi Wu, Hui-Ping Chien, Huang-Yu Yang, Yung-Chang Chen, Yu-Ching Chou, Jing-Long Huang
Although aberrant complement activation is involved in the pathogenesis of systemic lupus erythematosus (SLE), the role of complement regulatory proteins in disease activity of SLE remains limited. We enrolled the pediatric-onset SLE patients from our cohort study over 10 years. The clinical and laboratory data including SLEDAI disease activity score, and serum complement factor H (CFH), CFI, CD46, C5a, and C5b-9 in the active and remission phases were determined. Glomerular C5b-9 deposition as a complement activity marker was also examined...
February 2, 2018: Oncotarget
Uta Hardt, Anders Larsson, Iva Gunnarsson, Robert M Clancy, Michelle Petri, Jill P Buyon, Gregg J Silverman, Elisabet Svenungsson, Caroline Grönwall
BACKGROUND: Immunoglobulin M (IgM) autoreactivity to malondialdehyde (MDA) protein modifications is part of the natural antibody repertoire in health and may have beneficial functions. In contrast, IgG anti-MDA are increased in chronic inflammation and autoimmunity and may instead have pathogenic properties. METHODS: Herein, we investigated serum IgG anti-MDA levels by enzyme-linked immunosorbent assay (ELISA) in 398 systemic lupus erythematosus (SLE) patients in the Swedish Karolinska SLE cohort and compared these to findings in 225 US SLE patients from New York University and Johns Hopkins University...
February 26, 2018: Arthritis Research & Therapy
Huan Huan Xie, Hui Shen, Li Zhang, Mei Ying Cui, Li Ping Xia, Jing Lu
We measured the interleukin-34 (IL-34) level in sera from patients with systemic lupus erythematosus (SLE) and discoid lupus erythematosus (DLE) using an enzyme-linked immunosorbent assay (ELISA). Blood tests, including assays to determine C-reactive protein (CRP), complement (C) 3, C4, immunoglobulin (Ig) A, IgG, IgM, anti-double-stranded DNA antibody (Anti-dsDNA Ab) and hemoglobin (Hb) levels and white blood cell (WBC) and platelet (PLT) counts, were performed using standard methods. Lupus nephritis (LN) was diagnosed according to the American College of Rheumatology (ACR) renal criteria...
February 22, 2018: Scientific Reports
X W Dong, Z H Zheng, J Ding, X Luo, Z Q Li, Y Li, M Y Rong, Y L Fu, J H Shi, L C Yu, Z B Wu, P Zhu
Reliable markers for the rapid discrimination of severe renal damage remain a vital concern for lupus nephritis (LN). To determine a better tool for kidney damage detection, the present study compared the evaluation ability of novel urinary cytokines and chemokines (namely urinary monocyte chemoattractant protein 1 (uMCP-1), tumor necrosis factor-like weak inducer of apoptosis (uTWEAK)) with traditional serum or urinary markers (namely urinary alpha 1-microgrobulin (uα1-MG), beta 2-microglobulin (uβ2-MG) and serum complement C3 (C3), complement C4 (C4), creatinine (Cr), blood urea nitrogen (BUN) and cystatin C (Cys C)) in discriminating LN renal damage...
January 1, 2018: Lupus
C-B Choi, S Won, S-C Bae
Objectives Outcomes of systemic lupus erythematosus (SLE) have significantly improved over the years. However, when there is major organ involvement, the outcomes can still be unfavorable. Outcomes of multitarget therapy using mycophenolate mofetil (MMF) and tacrolimus in patients with SLE who were refractory to standard therapy were assessed. Methods We retrospectively reviewed the Hanyang BAE lupus cohort to identify patients with biopsy-confirmed lupus nephritis (classes III, IV, or V) who failed to either achieve complete response with standard induction therapy or those who had a lupus flare after achieving a complete response with conventional induction therapy and subsequently were switched to multitarget combination therapy with MMF and tacrolimus...
January 1, 2018: Lupus
Ratna Basak, Xiaotong Wang, Caitlin Keane, Robert Woroniecki
A 17-year-old girl presented with fever, myalgia, vomiting for 1 month and oliguria and dyspnoea for 4 days. She was tachycardic,hypertensive, with pedal oedema and decreased breath sounds. She had high serum creatinine (3 mg/dL), anaemia, thrombocytopenia, leucocytosis and eosinophilia with schistocytes. Lactate dehydrogenase, transaminases were high , with low haptoglobin and high ferritin (5269 ng/mL). Complement C3/C4 and fibrinogen were normal. Urinalysis showed large blood and protein and stool studies were negative...
February 11, 2018: BMJ Case Reports
Paola Italiani, Maria Laura Manca, Francesca Angelotti, Daniela Melillo, Federico Pratesi, Ilaria Puxeddu, Diana Boraschi, Paola Migliorini
BACKGROUND: Dysregulated production of cytokines has a critical role in systemic lupus. The aim of this work is to identify, by a comprehensive analysis of IL-1 family cytokines and receptors in serum, correlation between cytokines/receptors' levels and the clinical and serological features of the disease. METHODS: A full clinical evaluation was performed in 74 patients with systemic lupus erythematosus (SLE). C3, C4, anti-dsDNA and anti-C1q antibodies were measured...
February 8, 2018: Arthritis Research & Therapy
Yanwu You, Yueqiu Qin, Xu Lin, Fafen Yang, Junjie Wang, Fang Yuan, Suren R Sooranna, Liao Pinhu
OBJECTIVE: To investigate the expression levels of fractalkine (FKN) mRNA in peripheral blood mononuclear cells (PBMCs) and FKN protein in serum of patients with lupus nephritis (LN) from China, and to evaluate the associations between the expression of FKN and systemic lupus erythematosus disease activity index 2000 (SLEDAI-2 K), anti-double-stranded DNA and complement proteins in LN patients. METHODS: Real-time quantitative polymerase chain reaction and enzyme-linked immunosorbent assay were used to detect the expression levels of FKN mRNA in PBMCs and FKN protein in serum separately from 105 patients with LN and 52 healthy controls...
February 5, 2018: Cytokine
Shudan Wang, Ming Wu, Luis Chiriboga, Briana Zeck, H Michael Belmont
OBJECTIVE: To study membrane attack complex in lupus nephritis as a potential biomarker for disease intensity and prognostic indicator for response to treatment. METHODS: Immunohistochemistry was performed using unconjugated, murine anti-human complement C9 on kidney biopsies from 30 SLE patients who fulfilled 4 ACR or SLICC criteria. Clinical parameters were assessed at time of biopsy, 6 and 12 months. RESULTS: 30 renal biopsies were obtained from patients with Class II (2), III (5), IV (8), V (5), III+V (8) and IV+V (2)...
January 6, 2018: Seminars in Arthritis and Rheumatism
André R Alexandre, Pedro L Carreira, David A Isenberg
Lupus nephritis (LN) affects up to 50% of patients with Systemic Lupus Erythematosus (SLE) and is associated with a worse prognosis. LN usually develops within the first 5 years of the onset of the disease. We report three patients with very delayed LN (DLN) diagnosed after 15 or more years after SLE diagnosis. The three patients were non-Caucasian women with adolescent or adult-onset SLE. Each had antinuclear, anti-dsDNA and anti-Ro antibodies. Hydroxychloroquine was prescribed for each. Their disease courses were characterised by sporadic non-renal flares controlled by steroids and, in two cases, by one cycle of rituximab...
2018: Lupus Science & Medicine
Chi Chiu Mok, Samar Soliman, Ling Yin Ho, Fatma A Mohamed, Faten Ismail Mohamed, Chandra Mohan
BACKGROUND: The aim was to study urinary angiostatin, CXC chemokine ligand 4 (CXCL4) and vascular cell adhesion molecule-1 (VCAM-1) as biomarkers of renal disease in systemic lupus erythematosus (SLE). METHOD: Patients who fulfilled ≥ 4 American College of Rheumatology (ACR) criteria for SLE with active renal, active non-renal or inactive disease, and a group of healthy controls were studied. Urine samples were assayed for angiostatin, CXCL4 and VCAM-1 by ELISA, and normalized by creatinine...
January 11, 2018: Arthritis Research & Therapy
Tzu-Han Yang, Tsai-Hung Wu, Yuh-Lih Chang, Hsien-Tzung Liao, Chia-Chen Hsu, Chang-Youh Tsai, Yueh-Ching Chou
AIMS: This study aimed to assess retrospectively the efficacy and safety of cyclosporin A (CsA) therapy in patients with lupus nephritis (LN). MATERIALS AND METHODS: From September 2005 to August 2015, eligible patients with LN undergoing CsA treatment were enrolled in the study. Medical charts as well as clinical and laboratory data were retrospectively reviewed. The data were evaluated at 0, 1, 6, 12 month(s) after the start of CsA. Serum creatinine (SCr), estimated glomerular filtration rate (eGFR), urine protein/creatinine ratio (uPCR), complement components C3, C4, and anti-double stranded DNA antibody (anti-dsDNA) titers were recorded...
January 10, 2018: Clinical Nephrology
Hadi Karimzadeh, Shokrollah Hasani, Zahra Sayedbonakdar, Ali Mehrabi Koushki
Background: Renal involvement in systemic lupus erythematous is one of the most serious complications. The aim of this study was to compare the effects of treatment decisions based on clinical symptoms and renal biopsy on the outcome of patients with recurrent lupus nephritis. Materials and Methods: This descriptive study was conducted in 2012-13 in the Alzahra hospital on patients with lupus nephritis who had referred to the rheumatology clinic of this center due to lupus nephritis relapse...
2017: Advanced Biomedical Research
Tullia de Risi-Pugliese, Fleur Cohen Aubart, Julien Haroche, Philippe Moguelet, Sabine Grootenboer-Mignot, Alexis Mathian, Saskia Ingen-Housz-Oro, Miguel Hie, Noémie Wendremaire, Françoise Aucouturier, François Lepelletier, Makoto Miyara, Brigitte Bader-Meunier, Philippe Rémy, Nicole Fabien, Camille Francès, Stéphane Barete, Zahir Amoura
BACKGROUND: Bullous systemic lupus erythematosus (BSLE) is a rare blistering condition associated with systemic lupus erythematosus (SLE). PATIENTS AND METHODS: We conducted a multi-center retrospective study and literature review in order to describe the clinical, immunological, and histological presentations and outcomes of BSLE. The skin biopsies were centrally reviewed, and sera obtained during a flare of BSLE were analyzed for identification of circulating anti-basement membrane zone antibodies...
November 4, 2017: Seminars in Arthritis and Rheumatism
Valentina Živković, Tatjana Cvetković, Branka Mitić, Bojana Stamenković, Sonja Stojanović, Biljana Radovanović-Dinić, Vladimir Jurišić
There is a pivotal need for new markers to be tested in every day clinical practice for systemic lupus erythematosus (SLE) and lupus nephritis (LN). The levels of monocyte chemoattractant protein-1 (MCP-1) in the serum and urine of 72 SLE patients (27 with LN and 45 without LN involvement) and 30 healthy individuals were studied to establish their clinical significance. The SLE Disease Activity Index (SLEDAI) was used to establish the disease activity. Urine and serum MCP-1 was determined using the sandwich enzyme immunosorbent assay...
November 27, 2017: Rheumatology International
Wenge Zhao, Yanli Ma, Mingmin Wang, Changhe Shi, Jianping Sun, Kaiqiu Chu, Chuansheng Liu
Our study retrospectively investigated the expression of forkhead/winged helix transcription factor (Foxp3) in renal tissue and clinical features of patients with hepatitis B virus (HBV)-associated glomerulonephritis (HBV-GN). A total of 58 patients with HBV-GN were assigned to group A; 45 serum and renal tissue HBsAg-negative patients with nephritis were group B; 24 serum HBsAg-positive and renal tissue HBsAg-negative patients with slightly increased serum creatinine without nephritis were group C. Clinical manifestations, laboratory indices and renal biopsies were recorded...
November 2017: Experimental and Therapeutic Medicine
Michael J Boedigheimer, David A Martin, Zahir Amoura, Jorge Sánchez-Guerrero, Juanita Romero-Diaz, Alan Kivitz, Cynthia Aranow, Tak Mao Chan, Yip Boon Chong, Kit Chiu, Christine Wang, Winnie Sohn, Gregory E Arnold, Michael A Damore, Andrew A Welcher, Barbara A Sullivan, Brian L Kotzin, James B Chung
OBJECTIVE: To evaluate safety, pharmacokinetics and pharmacodynamics of anti-interferon (IFN)-γ monoclonal antibody AMG 811 in subjects with SLE without or with lupus nephritis (LN). METHODS: In this phase Ib, randomised, multiple-dose escalation study (NCT00818948), subjects without LN were randomised to subcutaneous AMG 811 (6, 20 or 60 mg) or placebo and subjects with LN were randomised to subcutaneous AMG 811 (20, 60 or 120 mg) or placebo every four weeks for three total doses...
2017: Lupus Science & Medicine
H Wang, J Xu, X Zhang, Y L Ren, M Cheng, Z L Guo, J C Zhang, H Cheng, G L Xing, S X Wang, F Yu, M H Zhao
Tubulointerstitial injury is found frequently in lupus nephritis. Immune complex deposits can occur in the tubular basement membranes (TBMs), although its significance in lupus nephritis patients remains unclear. This study assessed the clinical and prognostic features of lupus nephritis patients with TBM deposits in a large Chinese multicenter cohort. Complete data were collected from 195 patients with renal biopsy-proven lupus nephritis diagnosed in the Peking University First Hospital as the discovery cohort...
January 1, 2017: Lupus
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