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Daniel L Kenney-Jung, Annamaria Vezzani, Robert J Kahoud, Reghann G LaFrance-Corey, Mai-Lan Ho, Theresa Wampler Muskardin, Elaine C Wirrell, Charles L Howe, Eric T Payne
Febrile infection-related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with limited treatment options and an unclear etiology. Anakinra is a recombinant version of the human interleukin-1 receptor antagonist used to treat autoinflammatory disorders. This is the first report of anakinra for treatment of a child with super-refractory status epilepticus secondary to FIRES. Anakinra was well-tolerated and effective. Cerebral spinal fluid analysis revealed elevated levels of proinflammatory cytokines before treatment that normalized on anakinra, suggesting a potential pathogenic role for neuroinflammation in FIRES...
October 22, 2016: Annals of Neurology
Simon Tarp, Daniel E Furst, Anna Dossing, Mikkel Østergaard, Tove Lorenzen, Michael S Hansen, Jasvinder A Singh, Ernest H Choy, Maarten Boers, Maria E Suarez-Almazor, Lars E Kristensen, Henning Bliddal, Robin Christensen
OBJECTIVES: To summarize and compare the benefits and harms of biological agents used as monotherapy for rheumatoid arthritis (RA) in order to inform decisions for patients who are intolerant to conventional DMARD therapy. METHODS: We searched MEDLINE, EMBASE, CENTRAL, and other sources for randomised trials that compared biological monotherapy with methotrexate, placebo, or other biological monotherapies. Primary outcomes were ACR50 and the number of patients who discontinued due to adverse events...
September 14, 2016: Seminars in Arthritis and Rheumatism
Ingunn Holen, Diane V Lefley, Sheila E Francis, Sarah Rennicks, Steven Bradbury, Robert E Coleman, Penelope Ottewell
BACKGROUND: We have recently identified interleukin 1B (IL-1B) as a potential biomarker for predicting breast cancer patients at increased risk for developing bone metastasis. In mouse models, IL-1B and its receptor (IL-1R1) are upregulated in breast cancer cells that metastasise to bone compared with cells that do not. We have now investigated the functional role of IL-1 by blocking IL-1R signalling with the clinically licensed antagonist, anakinra. METHODOLOGY: 6-week old female BALB/c mice received a subcutaneous or intra-venous injection of MDA-MB-231-IV or MCF7 cells...
September 27, 2016: Oncotarget
Sarah E Petite
No abstract text is available yet for this article.
October 18, 2016: American Journal of Therapeutics
Mohammad Adawi, Bishara Bisharat, Abdalla Bowirrat
BACKGROUND: Erdheim-Chester disease (ECD) is an uncommon aggressive, multisystem form of non-Langerhans' cell histocytosis, which was firstly reported by Jakob Erdheim and William chester in 1930. The disease pathological features encompass an aberrant multiplication, overproduction and accumulation of white blood cells called histiocytes within multiple tissues and organs. Herein, we present a case of ECD owing to the rarity of this disease (roughly 550 cases have been described in the literature to date)...
October 2016: Medicine (Baltimore)
Gabriela Maria Wiedemann, Max Martin Ludwig Knott, Viola Katharina Vetter, Moritz Rapp, Sascha Haubner, Julia Fesseler, Benjamin Kühnemuth, Patrick Layritz, Raffael Thaler, Stephan Kruger, Steffen Ormanns, Doris Mayr, Stefan Endres, David Anz
In cancer patients, immunosuppression through regulatory T cells (Treg) is a crucial component of tumor immune evasion and contributes to disease progression. Tumor-infiltrating Treg in particular suppress local effector T cell responses and are associated with poor prognosis in tumors such as human pancreatic cancer or hepatocellular carcinoma (HCC). The chemokine CCL22 is known to recruit Treg into the tumor tissue and many types of human tumors are known to express high levels of CCL22. The mechanisms leading to intratumoral secretion of CCL22 are so far unknown...
2016: Oncoimmunology
Vanderlea Poeys Cabral, Carlos Augusto Ferreira de Andrade, Sonia Regina Lambert Passos, Maria de Fátima Moreira Martins, Yara Hahr Marques Hökerberg
A question is raised about an increased risk of severe infection from the use of biological drugs in patients with rheumatoid arthritis. This systematic review of observational studies aimed at assessing the risk of severe infection associated with the use of anakinra, rituximab, and abatacept in patients with rheumatoid arthritis. The following databases were searched: PubMed, Science Direct, Scopus, Web of Knowledge, Scirus, Cochrane, Exerpta Medica Database, Scielo, and Lilacs up to July 2010. Severe infections were defined as those life-threatening ones in need of the use of parenteral antibiotics or of hospitalization...
October 1, 2016: Revista Brasileira de Reumatologia
Kazuhiko Kotani, Michiaki Miyamoto, Hitoshi Ando
Rheumatoid arthritis (RA) is a chronic inflammatory disease with a potential cardiovascular (CV) risk. Flow-mediated vasodilation (FMD) is an ultrasonic method to evaluate endothelial function. RA is a contributor to endothelial dysfunction, a CV risk. Relevant insights on the improvement of the CV outcomes in RA patients may be obtained by a systematic review of trials that investigated the effects of RA treatment on FMD in RA patients. This review found that treatments with antirheumatic drugs and some non-antirheumatic drugs could improve the FMD in RA patients...
October 13, 2016: Current Vascular Pharmacology
Aditya Stanam, Katherine N Gibson-Corley, Laurie Love-Homan, Nnamdi Ihejirika, Andrean L Simons
Erlotinib has demonstrated poor clinical response rates for head and neck squamous cell carcinoma (HNSCC) to date and the majority of respondents acquire resistance to erlotinib relatively quickly. To elucidate novel pathways involved in erlotinib resistance, we compared the gene expression profiles of erlotinib-resistant (ER) vs. erlotinib-sensitive (ES) HNSCC cell lines. Enrichment analysis of microarray data revealed a deregulation of the IL-1 signaling pathway in ER versus ES-HNSCC cells. Gene expression of interleukin-1 alpha (IL1A) and interleukin-1 beta (IL1B) were significantly upregulated by > 2 fold in ER-SQ20B and ER-CAL 27 cells compared to their respective ES-cells...
October 12, 2016: Oncotarget
Seza Ozen, Jasmin B Kuemmerle-Deschner, Rolando Cimaz, Avi Livneh, Pierre Quartier, Isabelle Kone-Paut, Andrew Zeft, Steve Spalding, Ahmet Gul, Veronique Hentgen, Sinisa Savic, Ivan Foeldvari, Joost Frenkel, Luca Cantarini, Dony Patel, Jeffrey Weiss, Nina Marinsek, Ravi Degun, Kathleen G Lomax, Helen J Lachmann
OBJECTIVES: Periodic fever syndromes (PFS) are characterised by recurrent attacks of fever and localised inflammation. This study examined the diagnostic pathway and treatments at tertiary centres for FMF, TRAPS and MKD/HIDS. METHODS: PFS specialists at medical centres in the USA, European Union and Eastern Mediterranean participated in a retrospective chart review, providing de-identified data in an electronic case report form. Patients were treated between 2008-2012 with at least 1 year of follow-up, all had clinical and/or genetically proven disease, and were on/eligible for biologic treatment...
October 9, 2016: Arthritis Care & Research
R Andersen, G B E Jemec
Hidradenitis suppurativa (HS) is difficult to treat. Official guidelines have only recently been developed, and suggest that patients should be provided with both adjuvant, medical and surgical therapy. The guidelines are the result of resurgent interest in this disease, in which etiology and pathogenesis are only partially understood at present. Recent research has, however, identified possible targets for specific intervention using biologicals. In addition, classical clinically driven developments of new treatments continue to evolve, leading to several interesting new therapies for HS patients...
August 2016: Drugs of Today
Zdeněk Adam, Anna Šedivá, Renata Koukalová, Zdeněk Řehák, Hana Petrášová, Petr Szturz, Zdenka Adamová, Eva Vetešníková, Luděk Pour, Marta Krejčí, Viera Sandecká, Eva Pourová, Zdeňka Čermáková, Sabina Ševčíková, Zdeněk Král, Jiří Mayer
Schnitzlers syndrome is an acquired auto-inflammatory disease of still unclear origin. The Strasbourg criteria were adopted (non-infectious fever, chronic urticaria, changes in the bone structure, leukocytosis and higher values of inflammatory markers - CRP and presence of monoclonal immunoglobulin mostly of type IgM, very rarely of IgG) to establish this diagnosis. The first-choice therapy for this disease is the blocking of interleukin-1 effects. In practice, the interleukin-1 receptor antagonist, anakinra, is the most commonly used...
2016: Vnitr̆ní Lékar̆ství
Jana Van Wabeke, Eric Dhondt, Isabelle Peene, Yves Piette
Gout is a common cause of inflammatory arthritis. The classical treatment options in an acute gout attack are non-steroidal anti-inflammatory drugs, colchicine, and corticosteroids. Interleukin-1 inhibition has been shown to be an effective alternative when non-biologic therapies are ineffective or contraindicated. Herein, we report the case of a 69-year-old female who presented with polyarticular tophaceous gout treated successfully with anakinra.
October 7, 2016: Acta Clinica Belgica
Weimin Liu, Sucai Liu, Mukesh Verma, Iram Zafar, James T Good, Donald Rollins, Stephen Groshong, Magdalena M Gorska, Richard J Martin, Rafeul Alam
BACKGROUND: The mechanism of Th2/Th17-predominant and Th2/Th17-low asthma is unknown. OBJECTIVE: To study the immune mechanism of Th2/Th17-predominant and Th2/Th17-low asthma. METHODS: In a previously reported cohort of 60 asthmatic patients, 16 patients were immunophenotyped with Th2/Th17-predominant asthma and 22 patients with Th2/Th17-low asthma. We examined BAL leukocytes, cytokines, mediators, and epithelial cell function for these asthma subgroups...
October 1, 2016: Journal of Allergy and Clinical Immunology
Aurélie Gabrion, Isabelle Hmitou, Despina Moshous, Bénédicte Neven, Alain Lefèvre-Utile, Jean-Sébastien Diana, Félipe Suarez, Capucine Picard, Stéphane Blanche, Alain Fischer, Marina Cavazzana, Fabien Touzot
BACKGROUND: Chronic granulomatous disease (CGD) is primary immunodeficiency caused by defective production of reactive oxygen species in phagocytic cells that results in life-threatening infections and severe inflammatory manifestations. The treatment of inflammatory manifestations remains challenging, as it can be associated with an increased risk of infections. Previous studies have shown that CGD phagocytes display a defect in autophagy and a reactive oxygen species-independent activation of the inflammasome...
October 1, 2016: Journal of Allergy and Clinical Immunology
Stephanie R Harrison, Dennis McGonagle, Sharmin Nizam, Stephen Jarrett, Jeroen van der Hilst, Michael F McDermott, Sinisa Savic
BACKGROUND: Some adult patients presenting with unexplained pyrexia, serositis, skin rashes, arthralgia, myalgia, and other symptoms commonly found in autoinflammatory disorders may not fit a specific diagnosis, either because their clinical phenotype is nondiagnostic or genetic tests are negative. We used the term undifferentiated systemic autoinflammatory disorder (uSAID) to describe such cases. Given that well-defined autoinflammatory diseases show responses to IL-1 blockade, we evaluated whether anakinra was useful for both diagnosing and treating uSAID patients...
May 5, 2016: JCI Insight
Vanesa Calvo-Río, Montserrat Santos-Gómez, Inmaculada Calvo, Ma Isabel González-Fernández, Berta López Montesinos, Marina Mesquida, Alfredo Adán, María Victoria Hernández, Olga Maíz, Antonio Atanes, Beatriz Bravo, Consuelo Modesto, Gisela Díaz-Cordovés, Natalia Palmou-Fontana, Javier Loricera, M C González-Vela, Rosalía Demetrio-Pablo, J L Hernández, Miguel A González-Gay, Ricardo Blanco
OBJECTIVE: To assess the efficacy of Tocilizumab (TCZ) for Juvenile Idiopathic Arthritis (JIA)-associated uveitis. METHODS: Multicenter study of patients with JIA-associated uveitis refractory to conventional immunosuppressive drugs and anti-TNFα agents. RESULTS: We assessed 25 (21 women) patients (47 affected eyes); mean age 18.5±8.3 years. Uveitis was bilateral in 22. Cystoid macular edema (CME) was present in 9 patients. Ocular sequelae found at TCZ onset included: cataracts (13), glaucoma (7), synechiae (10), band keratopathy (12), maculopathy (9), and amblyopia (5)...
October 1, 2016: Arthritis & Rheumatology
Dror S Shouval, Amlan Biswas, Yu Hui Kang, Alexandra E Griffith, Liza Konnikova, Ivan D Mascanfroni, Naresh S Redhu, Sandra M Frei, Michael Field, Andria L Doty, Jeffrey D Goldsmith, Atul K Bhan, Anthony Loizides, Batia Weiss, Baruch Yerushalmi, Tadahiro Yanagi, Xiuli Lui, Francisco J Quintana, Aleixo M Muise, Christoph Klein, Bruce H Horwitz, Sarah C Glover, Athos Bousvaros, Scott B Snapper
IL10 receptor (IL10R)-deficient mice develop spontaneous colitis and similarly, patients with loss-of-function mutations in IL10R develop severe infant-onset inflammatory bowel disease (IBD). Loss of IL10R signaling in mouse and human macrophages is associated with increased production of interleukin 1 beta (IL1B). We demonstrated that innate immune production of IL1B mediates colitis in IL10R-deficient mice. Transfer of Il1r1(-/-) CD4(+) T cells into Rag1-/-/Il10rb-/- mice reduced the severity of their colitis (compared to mice that received CD4(+) T cells that express IL1R), accompanied by decreased production of interferon gamma, tumor necrosis factor, and IL17A...
September 27, 2016: Gastroenterology
Steven Vanderschueren, Annelien van der Veen
OBJECTIVES: The Schnitzler syndrome is a rare inflammatory disorder, with a chronic urticaria-like rash and an IgM (rarely IgG) monoclonal gammopathy as cardinal features. Interleukin-1 β is regarded as the key mediator and the interleukin-1 receptor antagonist anakinra has been proposed as first-line treatment. This case series of eleven patients is intended to enhance disease awareness and to compare our centre's experience with that of literature. METHODS: We describe the clinical features and disease course of 11 patients with a definite Schnitzler syndrome, according to the Strasbourg diagnostic criteria, encountered in the University Hospital, Leuven, Belgium, between 1995 and 2015...
August 31, 2016: Clinical and Experimental Rheumatology
Sunita Paudyal, Frances M Yang, Christopher Rice, Chen-Chun Chen, Michael Skelton, Monique Bethel, Shilpa Brown, Norris Stanley Nahman, Laura Carbone
OBJECTIVES: To determine the frequency of end-stage renal disease (ESRD) in patients with rheumatoid arthritis (RA), the causes of ESRD, and the treatment of RA in the setting of ESRD. METHODS: Cross-sectional study of RA (N = 3754) and non-RA (N = 326,776) patients in the United States Renal Data System (USRDS) during 2011 (N = 330,530). The epidemiology of ESRD in RA was determined and the etiology of ESRD in patients with and without RA was compared. The frequency of patients with RA with at least one filled prescription for prednisone/prednisolone, a DMARD, and/or a biologic in 2011 was determined...
August 2, 2016: Seminars in Arthritis and Rheumatism
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