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macrophage activating syndrome

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https://www.readbyqxmd.com/read/29353263/subcutaneous-adipose-tissue-biology-in-metabolic-syndrome
#1
REVIEW
Ishwarlal Jialal, Sridevi Devaraj
Metabolic syndrome (MetS) is a common global problem that comprises the cardio-metabolic cluster and predisposes to both diabetes and cardiovascular diseases. Although the pathogenic mechanisms have not been elucidated, both increased inflammation and insulin resistance play a pivotal role. It appears that both monocyte/macrophages and adipose tissue (AT) conspire to accentuate both the pro-inflammatory state and increased insulin resistance. Whilst there are scant data on visceral adipose tissue (VAT) and epicardial adipose tissue (EAT) biology, there are data on subcutaneous adipose tissue (SAT) dysregulation...
January 20, 2018: Hormone Molecular Biology and Clinical Investigation
https://www.readbyqxmd.com/read/29350173/membranoproliferative-glomerulonephritis-and-interstitial-nephritis-in-the-setting-of-epstein-barr-virus-related-hemophagocytic-syndrome%C3%A2
#2
Iolanda Godinho, Estela Nogueira, Sofia Jorge, António Teixeira Alves, António Gomes da Costa
BACKGROUND: Hemophagocytic syndrome (HPS) is a rare, aggressive disorder characterized by dysregulation of lymphocyte and macrophage activity, which culminates in tissue infiltration with hemophagocytosis and ultimately organ failure. Renal involvement frequently ensues and usually results in acute tubular necrosis with associated interstitial inflammation. Less frequently, glomerulopathy can also be found. CASE: We report a case of a 24-year-old Caucasian woman with previous asymptomatic hematuria, mild proteinuria, and normal renal function who presented to us with fever...
January 19, 2018: Clinical Nephrology
https://www.readbyqxmd.com/read/29348630/expression-of-inflammasome-proteins-and-inflammasome-activation-occurs-in-human-but-not-in-murine-keratinocytes
#3
Jennifer Sand, Eric Haertel, Thomas Biedermann, Emmanuel Contassot, Ernst Reichmann, Lars E French, Sabine Werner, Hans-Dietmar Beer
Inflammasomes are multimeric protein complexes that assemble upon sensing of a variety of stress factors. Their formation results in caspase-1-mediated activation and secretion of the pro-inflammatory cytokines pro-interleukin(IL)-1β and -18, which induce an inflammatory response. Inflammation is supported by a lytic form of cell death, termed pyroptosis. Innate immune cells, such as macrophages or dendritic cells, express and activate inflammasomes. However, it has also been demonstrated that human primary keratinocytes activate different types of inflammasomes in vitro, for example, upon UVB irradiation or viral infection...
January 18, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29345059/microrna-networks-associated-with-active-systemic-juvenile-idiopathic-arthritis-regulate-cd163-expression-and-anti-inflammatory-functions-in-macrophages-through-two-distinct-mechanisms
#4
Thuy Do, Rachel Tan, Mark Bennett, Mario Medvedovic, Alexei A Grom, Nan Shen, Sherry Thornton, Grant S Schulert
Systemic juvenile idiopathic arthritis (SJIA) is a severe childhood arthropathy with features of autoinflammation. Monocytes and macrophages in SJIA have a complex phenotype with both pro- and anti-inflammatory properties that combine features of several well characterized in vitro conditions used to activate macrophages. An important anti-inflammatory phenotype is expression of CD163, a scavenger receptor that sequesters toxic pro-inflammatory complexes that is highly expressed in both active SJIA and macrophage activation syndrome (MAS)...
January 2018: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/29342508/features-treatment-and-outcomes-of-macrophage-activation-syndrome-in-childhood-onset-systemic-lupus-erythematosus
#5
R Ezequiel Borgia, Maya Gerstein, Deborah M Levy, Earl D Silverman, Linda T Hiraki
OBJECTIVE: To describe the features and treatment of macrophage activation syndrome (MAS) in a childhood-onset systemic lupus erythematosus (cSLE) single centre cohort, and compare the cSLE manifestations and outcomes between those with and without MAS. METHODS: We included all cSLE patients followed at SickKids Hospital from 2002-12, and identified those also diagnosed with MAS. Demographic, clinical and laboratory features of MAS and SLE, medication use, hospital and PICU admissions as well as damage indices and mortality were extracted from the Lupus database...
January 17, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29337166/cd163-knockout-pigs-are-fully-resistant-to-highly-pathogenic-porcine-reproductive-and-respiratory-syndrome-virus
#6
Huaqiang Yang, Jian Zhang, Xianwei Zhang, Junsong Shi, Yongfei Pan, Rong Zhou, Guoling Li, Zicong Li, Gengyuan Cai, Zhenfang Wu
Porcine reproductive and respiratory syndrome virus (PRRSV) causes severe economic losses to current swine production worldwide. Highly pathogenic PRRSV (HP-PRRSV), originated from a genotype 2 PRRSV, is more virulent than classical PRRSV and further exacerbates the economic impact. HP-PRRSV has become the predominant circulating field strain in China since 2006. CD163 is a cellular receptor for PRRSV. The depletion of CD163 whole protein or SRCR5 region (interaction site for the virus) confers resistance to infection of several PRRSV isolates in pigs or cultured host cells...
January 11, 2018: Antiviral Research
https://www.readbyqxmd.com/read/29332923/heart-failure-as-an-aging-related-phenotype
#7
Hiroyuki Morita, Issei Komuro
The molecular pathophysiology of heart failure, which is one of the leading causes of mortality, is not yet fully understood. Heart failure can be regarded as a systemic syndrome of aging-related phenotypes. Wnt/β-catenin signaling and the p53 pathway, both of which are key regulators of aging, have been demonstrated to play a critical role in the pathogenesis of heart failure. Circulating C1q was identified as a novel activator of Wnt/β-catenin signaling, promoting systemic aging-related phenotypes including sarcopenia and heart failure...
January 15, 2018: International Heart Journal
https://www.readbyqxmd.com/read/29331073/cerebrospinal-fluid-macrophage-biomarkers-in-amyotrophic-lateral-sclerosis
#8
A G Thompson, E Gray, M-L Thézénas, P D Charles, S Evetts, M T Hu, K Talbot, R Fischer, B M Kessler, M R Turner
Objective The neurodegenerative disease amyotrophic lateral sclerosis (ALS) is a heterogeneous clinical syndrome involving multiple molecular pathways. The development of biomarkers for use in therapeutic trials is a priority. We sought to use a high-throughput proteomic method to identify novel biomarkers in individual cerebrospinal fluid samples. Methods Liquid chromatography-tandem mass spectrometry with label-free quantification was used to identify cerebrospinal fluid proteins using samples from a well-characterised longitudinal cohort comprising patients with ALS (n=43), the upper motor neuron variant primary lateral sclerosis (PLS, n=6), cross-sectional healthy (n=20) and disease controls (Parkinsons's n=20, ALS mimic disorders n=12)...
January 13, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29327078/the-role-of-the-thioredoxin-thioredoxin-reductase-system-in-the-metabolic-syndrome-towards-a-possible-prognostic-marker
#9
REVIEW
Alexey A Tinkov, Geir Bjørklund, Anatoly V Skalny, Arne Holmgren, Margarita G Skalnaya, Salvatore Chirumbolo, Jan Aaseth
Mammalian thioredoxin reductase (TrxR) is a selenoprotein with three existing isoenzymes (TrxR1, TrxR2, and TrxR3), which is found primarily intracellularly but also in extracellular fluids. The main substrate thioredoxin (Trx) is similarly found (as Trx1 and Trx2) in various intracellular compartments, in blood plasma, and is the cell's major disulfide reductase. Thioredoxin reductase is necessary as a NADPH-dependent reducing agent in biochemical reactions involving Trx. Genetic and environmental factors like selenium status influence the activity of TrxR...
January 11, 2018: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/29326099/interleukin-18-diagnostically-distinguishes-and-pathogenically-promotes-human-and-murine-macrophage-activation-syndrome
#10
Eric S Weiss, Charlotte Girard-Guyonvarc'h, Dirk Holzinger, Adriana A de Jesus, Zeshan Tariq, Jennifer Picarsic, Eduardo J Schiffrin, Dirk Foell, Alexei A Grom, Sandra Ammann, Stephan Ehl, Tomoaki Hoshino, Raphaela Goldbach-Mansky, Cem Gabay, Scott W Canna
Hemophagocytic Lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS) are life-threatening hyperferritinemic systemic inflammatory disorders. Though profound cytotoxic impairment causes familial HLH (fHLH), the mechanisms driving non-fHLH and MAS are largely unknown. MAS occurs in patients with suspected rheumatic disease, but the mechanistic basis for its distinction is unclear. Recently, a syndrome of recurrent MAS with infantile enterocolitis caused by NLRC4 inflammasome hyperactivity highlighted the potential importance of Interleukin (IL)-18...
January 11, 2018: Blood
https://www.readbyqxmd.com/read/29324628/ulinastatin-protects-against-lps-induced-acute-lung-injury-by-attenuating-tlr4-nf-%C3%AE%C2%BAb-pathway-activation-and-reducing-inflammatory-mediators
#11
Chao Cao, Chengfen Yin, Songtao Shou, Jun Wang, Lechang Yu, Xuening Li, Yanfen Chai
Acute lung injury (ALI) and its severe form, acute respiratory distress syndrome (ARDS), remain the leading causes of morbidity and mortality in intensive care units. Ulinastatin (UTI), a serine protease inhibitor, possesses anti-inflammatory properties and has been suggested to modulate lipopolysaccharide (LPS)-induced sepsis; thus, it is now widely used in the treatment of pancreatitis, sepsis, and septic shock. Toll-like receptor 4 (TLR4), an essential LPS signaling receptor, plays a critical role in the activation of innate immunity...
January 10, 2018: Shock
https://www.readbyqxmd.com/read/29309057/macrophage-extracellular-trap-formation-promoted-by-platelet-activation-is-a-key-mediator-of-rhabdomyolysis-induced-acute-kidney-injury
#12
Koshu Okubo, Miho Kurosawa, Mako Kamiya, Yasuteru Urano, Akari Suzuki, Kazuhiko Yamamoto, Koji Hase, Koichiro Homma, Junichi Sasaki, Hiroaki Miyauchi, Tatsuo Hoshino, Matsuhiko Hayashi, Tanya N Mayadas, Junichi Hirahashi
Rhabdomyolysis is a serious syndrome caused by skeletal muscle injury and the subsequent release of breakdown products from damaged muscle cells into systemic circulation. The muscle damage most often results from strenuous exercise, muscle hypoxia, medications, or drug abuse and can lead to life-threatening complications, such as acute kidney injury (AKI). Rhabdomyolysis and the AKI complication can also occur during crush syndrome, an emergency condition that commonly occurs in victims of natural disasters, such as earthquakes, and man-made disasters, such as wars and terrorism...
January 8, 2018: Nature Medicine
https://www.readbyqxmd.com/read/29304824/patient-characteristics-associated-with-response-to-nsaid-monotherapy-in-children-with-systemic-juvenile-idiopathic-arthritis
#13
Anjali Sura, Christopher Failing, Julie Sturza, Jasmine Stannard, Meredith Riebschleger
BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA) is an auto-inflammatory disease characterized by fever, arthritis, and ≥1 of rash, generalized lymphadenopathy, hepato/splenomegaly, and serositis. Non-steroidal anti-inflammatory drugs (NSAIDs) are among the initial treatments of sJIA, but there is currently no evidence indicating which children should undergo a trial of NSAID monotherapy and which should not. Our objective is to identify presentation characteristics which are associated with response and lack of response to a trial of NSAID monotherapy...
January 5, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29302207/role-of-inflammasomes-in-inflammatory-autoimmune-rheumatic-diseases
#14
REVIEW
Young-Su Yi
Inflammasomes are intracellular multiprotein complexes that coordinate anti-pathogenic host defense during inflammatory responses in myeloid cells, especially macrophages. Inflammasome activation leads to activation of caspase-1, resulting in the induction of pyroptosis and the secretion of pro-inflammatory cytokines including interleukin (IL)-1β and IL-18. Although the inflammatory response is an innate host defense mechanism, chronic inflammation is the main cause of rheumatic diseases, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), ankylosing spondylitis (AS), and Sjögren's syndrome (SS)...
January 2018: Korean Journal of Physiology & Pharmacology
https://www.readbyqxmd.com/read/29296904/soluble-interleukin-2-receptor-is-a-sensitive-diagnostic-test-in-adult-hlh
#15
Anna Hayden, Molly Lin, Sujin Park, Morris Pudek, Marion Schneider, Michael B Jordan, Andre Mattman, Luke Y C Chen
Serum soluble interleukin-2 receptor (sIL-2r) is an important disease marker in hemophagocytic lymphohistiocytosis (HLH), but there are no published data on its diagnostic value in adults. We conducted a single-center retrospective study of 78 consecutive adults who had sIL-2r measured for suspected HLH. Serum sIL-2r levels were measured by enzyme-linked immunosorbent assay (adult reference range, 241-846 U/mL). There were 38 patients with HLH and 40 with a non-HLH diagnosis (such as sepsis, liver disease, histiocyte disorders, autoimmune disease, leukemia, or lymphoma)...
December 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296875/vaccination-with-autologous-myeloblasts-admixed-with-gm-k562-cells-in-patients-with-advanced-mds-or-aml-after-allogeneic-hsct
#16
Vincent T Ho, Haesook T Kim, Natalie Bavli, Martin Mihm, Olga Pozdnyakova, Matthias Piesche, Heather Daley, Carol Reynolds, Nicholas C Souders, Corey Cutler, John Koreth, Edwin P Alyea, Joseph H Antin, Jerome Ritz, Glenn Dranoff, Robert J Soiffer
We report a clinical trial testing vaccination of autologous myeloblasts admixed with granulocyte-macrophage colony-stimulating factor secreting K562 cells after allogeneic hematopoietic stem cell transplantation (HSCT). Patients with myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML) with ≥5% marrow blasts underwent myeloblast collection before HSCT. At approximately day +30, 6 vaccines composed of irradiated autologous myeloblasts mixed with GM-K562 were administered. Tacrolimus-based graft-versus-host disease (GVHD) prophylaxis was not tapered until vaccine completion (∼day 100)...
November 14, 2017: Blood Advances
https://www.readbyqxmd.com/read/29295842/unopposed-il-18-signaling-leads-to-severe-tlr9-induced-macrophage-activation-syndrome-in-mice
#17
Charlotte Girard-Guyonvarc'h, Jennifer Palomo, Praxedis Martin, Emiliana Rodriguez, Sabina Troccaz, Gaby Palmer, Cem Gabay
The term macrophage activation syndrome (MAS) defines a severe, potentially fatal disorder characterized by overwhelming inflammation and multiorgan involvement. Interleukin (IL)-18 is a pro-inflammatory cytokine belonging to the IL-1 family, the activity of which is regulated by its endogenous inhibitor IL-18 binding protein (IL-18BP). Elevated IL-18 levels have been reported in patients with MAS. Herein we show that upon repeated toll-like receptor (TLR)9 stimulation with CpG, IL-18BP-/- mice display severe MAS manifestations, including increased weight loss, splenomegaly, anemia, thrombocytopenia, hyperferritinemia and bone marrow hemophagocytosis as compared to wild-type mice...
January 2, 2018: Blood
https://www.readbyqxmd.com/read/29291352/recurrent-ecsit-mutation-encoding-v140a-triggers-hyperinflammation-and-promotes-hemophagocytic-syndrome-in-extranodal-nk-t-cell-lymphoma
#18
Haijun Wen, Huajuan Ma, Qichun Cai, Suxia Lin, Xinxing Lei, Bin He, Sijin Wu, Zifeng Wang, Yan Gao, Wensheng Liu, Weiping Liu, Qian Tao, Zijie Long, Min Yan, Dali Li, Keith W Kelley, Yongliang Yang, Huiqiang Huang, Quentin Liu
Hemophagocytic syndrome (HPS) is a fatal hyperinflammatory disease with a poorly understood mechanism that occurs most frequently in extranodal natural killer/T cell lymphoma (ENKTL). Through exome sequencing of ENKTL tumor-normal samples, we have identified a hotspot mutation (c.419T>C) in the evolutionarily conserved signaling intermediate in Toll pathway (ECSIT) gene, encoding a V140A variant of ECSIT. ECSIT-V140A activated NF-κB more potently than the wild-type protein owing to its increased affinity for the S100A8 and S100A9 heterodimer, which promotes NADPH oxidase activity...
January 1, 2018: Nature Medicine
https://www.readbyqxmd.com/read/29285314/tlr2-myd88-pathway-dependent-regulation-of-dendritic-cells-by-dengue-virus-promotes-antibody-dependent-enhancement-via-th2-biased-immunity
#19
Junu Aleyas George, Seong Bum Kim, Jin Young Choi, Ajit Mahadev Patil, Ferdaus Mohd Altaf Hossain, Erdenebelig Uyangaa, Jin Hur, Sang-Youel Park, John-Hwa Lee, Koanhoi Kim, Seong Kug Eo
Possible risk mediators in primary dengue virus (DenV) infection that favor secondary DenV infection to life-threatening dengue hemorrhagic fever (DHF) and shock syndrome (DSS) via antibody-dependent enhancement (ADE) have not yet been described. Here, DenV infection enhanced the expression of inflammatory mediators and activation molecules in dendritic cells (DCs) through TLR2/MyD88 pathway. TLR2 appeared to facilitate DenV infection in DCs that were less permissive than macrophages for viral replication. In experiments using separate evaluations of DenV-infected and uninfected bystander DCs, infected DCs showed impaired maturation accompanied with TLR2-dependent production of inflammatory cytokines, by which uninfected bystander DCs showed increased expression of co-stimulatory molecules...
December 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/29279647/influenza-b-virus-triggering-macrophage-activation-syndrome-in-an-infant
#20
K Jayashree, Suchetha Rao, Nutan Kamath
Macrophage activation syndrome (MAS) is a potentially fatal complication caused by excessive activation and expansion of macrophages and T lymphocytes. It can be triggered by various infections and is characterized by the development of cytopenias, hyperferritinemia, liver dysfunction, and coagulopathy. We report a 10-month-old female infant with fever, convulsions, and hepatosplenomegaly. Laboratory data of bicytopenia, low erythrocyte sedimentation rate, and elevated liver enzymes suggested MAS. This was supported by the presence of hyperferritinemia with hypertriglyceridemia...
November 2017: Indian Journal of Critical Care Medicine
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