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macrophagic activation syndrome

Yumi Tsuchida, Shuji Sumitomo, Hirofumi Shoda, Kanae Kubo, Keishi Fujio, Kazuhiko Yamamoto
No abstract text is available yet for this article.
October 25, 2016: Modern Rheumatology
Lisa R Young, Peter M Gulleman, Chelsi W Short, Harikrishna Tanjore, Taylor Sherrill, Aidong Qi, Andrew P McBride, Rinat Zaynagetdinov, John T Benjamin, William E Lawson, Sergey V Novitskiy, Timothy S Blackwell
Alveolar epithelial cell (AEC) dysfunction underlies the pathogenesis of pulmonary fibrosis in Hermansky-Pudlak syndrome (HPS) and other genetic syndromes associated with interstitial lung disease; however, mechanisms linking AEC dysfunction and fibrotic remodeling are incompletely understood. Since increased macrophage recruitment precedes pulmonary fibrosis in HPS, we investigated whether crosstalk between AECs and macrophages determines fibrotic susceptibility. We found that AECs from HPS mice produce excessive MCP-1, which was associated with increased macrophages in the lungs of unchallenged HPS mice...
October 20, 2016: JCI Insight
Sven Jarius, Marius Ringelstein, Jürgen Haas, Irina I Serysheva, Lars Komorowski, Kai Fechner, Klaus-Peter Wandinger, Philipp Albrecht, Harald Hefter, Andreas Moser, Eva Neuen-Jacob, Hans-Peter Hartung, Brigitte Wildemann, Orhan Aktas
BACKGROUND: Recently, we described a novel autoantibody, anti-Sj/ITPR1-IgG, that targets the inositol 1,4,5-trisphosphate receptor type 1 (ITPR1) in patients with cerebellar ataxia. However, ITPR1 is expressed not only by Purkinje cells but also in the anterior horn of the spinal cord, in the substantia gelatinosa and in the motor, sensory (including the dorsal root ganglia) and autonomic peripheral nervous system, suggesting that the clinical spectrum associated with autoimmunity to ITPR1 may be broader than initially thought...
October 24, 2016: Journal of Neuroinflammation
Osman Yeşilbaş, Hasan Serdar Kıhtır, Hamdi Murat Yıldırım, Nevin Hatipoğlu, Esra Şevketoğlu
BACKGROUND: Leptospirosis is a zoonotic infectious disease caused by pathogenic spirochetes of the genus Leptospira. Although it is usually asymptomatic and self-limited, severe potentially fatal illness accompanied by multi-organ failure may occur. CASE REPORT: Here we report an unusual case of severe leptospirosis successfully treated with continuous venovenous hemofiltration (CVVHF) and therapeutic plasma exchange (TPE). The patient presented with pericardial tamponade, renal failure and macrophage activation syndrome, and later suffered prolonged jaundice and sclerosing cholangitis during hospitalization in the pediatric intensive care unit (PICU)...
September 2016: Balkan Medical Journal
Akimune Kaga, Hiroshi Watanabe, Hiroki Miyabayashi, Takaya Metoki, Setsuko Kitaoka, Satoru Kumaki
Neonatal toxic shock syndrome-like exanthematous disease (NTED) is a newly recognized neonatal infectious disease, caused by the superantigen toxic shock syndrome toxin-1 (TSST-1). TSST-1 is mainly produced by methicillin-resistant Staphylococcus aureus, and the immune responses to TSST-1 are known to cause toxic shock syndrome, a life-threatening infectious disease. The clinical symptoms of NTED are skin rash, fever, and thrombocytopenia, but severe thrombocytopenia is rare in term infants with NTED. Although the cause of NTED is the same as that of toxic shock syndrome, the clinical symptoms of NTED are milder than toxic shock syndrome...
2016: Tohoku Journal of Experimental Medicine
Arun Agarwal, Samiksha Sharma, Mala Airun
Selective immunoglobulin M deficiency(sIgMD) is a rare form of dysgammaglobulinaemia characterized by an isolated low level of serum immunoglobulin M (IgM). It was an incidence of less than 0.03% in the general population and 1% in hospitalized patients. sIgMD may occur as a primary or secondary condition. sIgMD is much more common than primary .Hemophagocytic lymphohistiocytosis (HLH) is also a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes and can be primary or secondary, characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and hematologic alterations...
July 2016: Journal of the Association of Physicians of India
Jia Shi, Jian-Bo Yu, Wei Liu, Dan Wang, Yuan Zhang, Li-Rong Gong, Shu-An Dong, Da-Quan Liu
Acute respiratory distress syndrome (ARDS) is one of the most devastating complications of sepsis lacking of effective therapy. Mitochondrial dynamics undergoing continuous fusion and fission play a crucial role in mitochondrial structure and function. Fis1, as a small protein located on the outer membrane of mitochondria, has been thought to be an important protein mediated mitochondrial fission. During ARDS, alveolar macrophages suffer from increased oxidative stress and apoptosis, and also accompanied by disrupted mitochondrial dynamics...
October 14, 2016: Experimental Cell Research
Yukimi Otsuka, Yasushi Inoue
Acute acalculous cholecystitis (AAC) is a severe disease seen in critically ill patients, including those with autoimmune diseases. We herein report the case of a 41-year-old female who developed macrophage activation syndrome (MAS) accompanied by a recurrence of Kikuchi disease. Abdominal imaging revealed marked thickening of the gallbladder wall and pericholecystic fluid, typically found in AAC. Treatment with intravenous pulse methylprednisolone induced in a significant improvement in the gallbladder wall, resulting in no need for surgical intervention...
2016: Internal Medicine
Mark Wunderlich, Courtney Stockman, Mahima Devarajan, Navin Ravishankar, Christina Sexton, Ashish R Kumar, Benjamin Mizukawa, James C Mulloy
Transgenic expression of key myelosupportive human cytokines in immune-deficient mice corrects for the lack of cross-species activities of stem cell factor (SCF), IL-3, and GM-CSF. When engrafted with human umbilical cord blood (UCB), these triple-transgenic mice produce BM and spleen grafts with much higher myeloid composition, relative to nontransgenic controls. Shortly after engraftment with UCB, these mice develop a severe, fatal macrophage activation syndrome (MAS) characterized by a progressive drop in rbc numbers, increased reticulocyte counts, decreased rbc half-life, progressive cytopenias, and evidence of chronic inflammation, including elevated human IL-6...
September 22, 2016: JCI Insight
David Pulido, Maria Flor Garcia-Mayoral, Mohammed Moussaoui, Diego Velázquez, Marc Torrent, Marta Bruix, Ester Boix
Acute infection by Gram-negative pathogens can induce an exacerbated immune response that leads to lethal septic shock syndrome. Bacterial lipopolysaccharide (LPS) is a major pathogen-associated molecular pattern (PAMP) molecule that can initiate massive and lethal immune system stimulation. Therefore, the development of new and effective LPS-neutralizing agents is a top priority. The eosinophil cationic protein (ECP) is an antimicrobial protein secreted in response to infection, with a remarkable affinity for LPS...
October 3, 2016: FEBS Journal
Morten Stilund, Mikkel Carstensen Gjelstrup, Tove Christensen, Holger Jon Møller, Thor Petersen
OBJECTIVES: This study aimed to examine the levels of the macrophage marker sCD163 and other biomarkers at the time of diagnosis of patients with either clinically isolated syndrome (CIS) or relapsing-remitting multiple sclerosis (RRMS), and assess relation to clinical indicators of prognosis, disease activity (DA), and changes in the levels of these biomarkers at follow-up. MATERIALS AND METHODS: The clinical status and MRI were reevaluated in 56 patients more than 1 year after diagnosis with a median follow-up time of 2 years...
September 2016: Brain and Behavior
Eri Watanabe, Hitoshi Sugawara, Takeshi Yamashita, Akira Ishii, Aya Oda, Chihiro Terai
We report the case of a 71-year-old Japanese woman with adult-onset Still's disease (AOSD) in whom macrophage activation syndrome (MAS) developed despite therapy with oral high-dose prednisolone and intravenous methylprednisolone pulse therapy twice. She was successfully treated with tocilizumab (TCZ). Soon afterward, her fever ceased and high levels of both ferritin and C-reactive protein levels decreased. Her course was complicated by disseminated intravascular coagulation, cytomegalovirus infection, and Pneumocystis jirovecii pneumonia...
2016: Case Reports in Medicine
H Grønbaek, M Kreutzfeldt, K Kazankov, N Jessen, T Sandahl, S Hamilton-Dutoit, H Vilstrup, H J Møller
BACKGROUND: Autoimmune hepatitis (AIH) is characterised by liver inflammation with reversibility upon anti-inflammatory treatment. Soluble (s)CD163, a specific macrophage activation marker, is associated with inflammation in other liver diseases, but never investigated in AIH. AIM: To investigate sCD163 in patients with acute AIH and in complete and incomplete responders to standard anti-inflammatory pharmacotherapy, and during follow-up in treatment naive patients...
November 2016: Alimentary Pharmacology & Therapeutics
Piero Ruscitti, Paola Cipriani, Francesco Ciccia, Francesco Masedu, Vasiliki Liakouli, Francesco Carubbi, Onorina Berardicurti, Giuliana Guggino, Paola Di Benedetto, Salvatore Di Bartolomeo, Marco Valenti, Giovanni Triolo, Roberto Giacomelli
Macrophage activation syndrome (MAS) is a rare, life-threatening disease in which early diagnosis and aggressive therapeutic strategy may improve the outcome. Due to its rarity, epidemiologic data are still lacking. Hyperferritinemia is frequently associated with MAS and might modulate the cytokine storm, which is involved in the development of multiple organ failure. In this paper, we investigated clinical data, treatments, and outcome of a homogeneous cohort of 41 adult MAS patients, complicating autoimmune rheumatic diseases...
September 21, 2016: Autoimmunity Reviews
Zhibin Yu, Chen Huang, Qiong Zhang, Wen-Hai Feng
Porcine reproductive and respiratory syndrome virus (PRRSV) mainly infects monocyte/macrophage cells and modulates cytokine production to regulate host immune response. IL-12p40 is the basic subunit of IL-12, a heterodimeric cytokine, which plays key roles in the cell-mediated immune response. In the present study, we demonstrated that PRRSV infection induced IL-12p40 production in vitro and in vivo. Subsequently, we showed that inhibitors of p38 MAPK, JNK, and NF-κB dramatically reduced PRRSV-induced IL-12p40 expression...
October 2, 2016: Virus Research
M Hsairi, S Ben Ameur, S Alibi, Y Belfitouri, I Maaloul, A Znazen, I Chabchoub, A Hammami, M Hachicha
Since the Arab Spring, a resurgence of zoonotic diseases such as rickettsiosis, endemic in the Mediterranean basin, has been observed. It preferentially infects microvascular endothelial cells of mammalian hosts inducing vasculitis with endothelial injury. Rickettsioses are considered benign infectious diseases. Severe systemic manifestations have been reported and are often explained by a delay in diagnosis. We present a case of hemophagocytic syndrome occurring in a 4-year-old Libyan girl as a complication of Mediterranean spotted fever...
October 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Jiping Zhao, Hao Yu, Yudong Liu, Sara A Gibson, Zhaoqi Yan, Xin Xu, Amit Gaggar, Pui-Kai Li, Chenglong Li, Shi Wei, Etty N Benveniste, Hongwei Qin
Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) are diseases with high mortality. Macrophages and neutrophils are responsible for inflammatory responses in ALI and ARDS, which are characterized by excessive production of proinflammatory mediators in bronchoalveolar lavage fluid (BALF) and plasma. Aberrant activation of the JAK/STAT pathway is critical for persistent inflammation in many conditions such as infection and autoimmunity. Given the importance of the STAT3 transcription factor in activating macrophages and neutrophils, and augmenting inflammation, we investigated the therapeutic potential of inhibiting STAT3 activity using the small molecule STAT3 inhibitor, LLL12...
September 16, 2016: American Journal of Physiology. Lung Cellular and Molecular Physiology
Walker M McHugh, William W Russell, Andrew J Fleszar, Paul E Rodenhouse, Skyler P Rietberg, Lei Sun, Thomas P Shanley, Timothy T Cornell
Acute Respiratory Distress Syndrome (ARDS) remains a leading cause of morbidity and mortality in both adult and pediatric intensive care units. A key event in the development of ARDS is neutrophil recruitment into the lungs leading to tissue damage and destruction. Interleukin-8 (IL-8) is the major human chemokine responsible for neutrophil recruitment into the lungs. Protein phosphatase 2A (PP2A) has been shown to be a key regulator of the mitogen-activated protein kinase (MAPK) cascades, which control the production of IL-8...
September 16, 2016: American Journal of Physiology. Lung Cellular and Molecular Physiology
Oswald Moling, Andrea Piccin, Martina Tauber, Peter Marinello, Mariagrazia Canova, Marco Casini, Giovanni Negri, Bernd Raffeiner, Raffaella Binazzi, Latha Gandini, Cinzia Vecchiato, Giovanni Rimenti, Atto Billio
BACKGROUND: Silicone implants have been successfully used for breast augmentation and reconstruction in millions of women worldwide. The reaction to the silicone implant is highly variable; it can lead to local inflammatory symptoms, and sometimes to systemic symptoms and disease. Over 80 cases of anaplastic lymphoma kinase-negative anaplastic large cell lymphoma have been reported in patients with silicone breast implants and have been accepted as a new clinical entity. To the best of our knowledge, an intravascular large B-cell lymphoma associated with a silicone breast implant has not been reported previously...
2016: Journal of Medical Case Reports
Ouahiba Sihali-Beloui, Salima El-Aoufi, Boubekeur Maouche, Sergio Marco
The aim of our transmission electron microscope study was to show, for the first time, the alteration of liver cells involved in the evolution of steatosis to steatohepatitis on a murine model of the diet-induced metabolic syndrome, Psammomys obesus. This pathologic evolution was induced by using the standard laboratory diet during 10 months, and analyzed with metabolic studies and the immunohistochemistry technique. Four months later, hepatocytes charged with lipid vacuoles were involved in autophagy. Furthermore, in the sinusoids, we observed Kupffer cells, neutrophils and macrophages...
September 28, 2016: Comptes Rendus Biologies
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