tbuckley/cabot-search huggingface dataset:
Update of EULAR recommendations for the treatment of systemic sclerosis
Authors: Otylia Kowal-Bielecka, Jaap Fransen, Jerome Avouac, Mike Becker, Agnieszka Kulak, Yannick Allanore, Oliver Distler, Philip Clements, Maurizio Cutolo, Laszlo Czirjak, Nemanja Damjanov, Francesco del Galdo, Christopher P Denton, Jörg H W Distler, Ivan Foeldvari, Kim Figelstone, Marc Frerix, Daniel E Furst, Serena Guiducci, Nicolas Hunzelmann, Dinesh Khanna, Marco Matucci-Cerinic, Ariane L Herrick, Frank van den Hoogen, Jacob M van Laar, Gabriela Riemekasten, Richard Silver, Vanessa Smith, Alberto Sulli, Ingo Tarner, Alan Tyndall, Joep Welling, Frederic Wigley, Gabriele Valentini, Ulrich A Walker, Francesco Zulian, Ulf Müller-Ladner
Journal: Annals of the Rheumatic Diseases (2016)

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc.
AB0880 Macrophage Activation Syndrome – An Indian Experience
Authors: P.P. Giri, P. Pal
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Macrophage activation syndrome is a deadly complication of systemic rheumatic disease namely Systemic Juvenile Idiopathic arthritis (SJIA). But many other rheumatic diseases like SLE and Kawasaki disease (KD) can be complicated by MAS due to immune deregulation and extreme hypercytokinemia. Here we present a series of 24 cases of MAS that we have encountered in last 7 and half years. <h3>Objectives</h3> 1.To evaluate the clinical and laboratory characteristics of MAS 2.To assess the treatment responsiveness to different therapies 3. To identify the poor prognostic factors <h3>Methods</h3> Children fulfilled the recent 2014 diagnostic criteria of MAS that has been admitted in the time period of July 2008 to November 2015 has been included in the study. The details of clinical features, laboratory results and outcome were noted down. <h3>Results</h3> The total number of patient was 24 among them, Systemic JIA was 18, SLE was 5 and rest one had KD as a primary illness. The male: female ratio was 17:7 and the mean age at presentation was:7.9 years. CNS, cardiac and respiratory involvement were present respectively in 78, 52 and 35% of cases. 20 patients needed ICU admission, 10 needed mechanical ventilation. 16 survived and 7 expired with a mortality rate of around 30%. All of them received Pulse Methylprednisolne (30mg/kg) for 5–7 days +/− Intravenous immunoglobulin. 12 needed Cyclosporine. <h3>Conclusions</h3> 1. MAS is a deadly complication of pediatric rheumatological diseases with a high mortality rate of 30% in our series. 2. Features of acute liver failure along with CNS and cardiac involvement with extreme hyperferritenemia (ferritin &gt;50,000ng/ml) was associated with bad prognosis. 3. The effectiveness of IV pulse methylprednisolone was only 40%. <h3>References</h3> Alessandro Parodi et al .Macrophage Activation Syndrome inJuvenile Systemic Lupus Erythematosus A Multinational Multicenter Study of Thirty-Eight Patients. Arthritis &amp; Rheumatismvol. 60, No. 11, November 2009, pp 3388–3399 <h3>Disclosure of Interest</h3> None declared
Post-ASCT Consolidative Immunotherapy with RITUXIMAB, rINF-a2b and rIL2 In Poor-Risk DLBCL: Results of a Phase II Study
Authors: Jean-Richard Eveillard, Jean-Christophe Ianotto, Adrian Tempescul, Gaelle Guillerm, Hussam Saad, Florence Dalbiès, Ronan Le Calloch, Anaïg Dagorne, Christian Berthou
Journal: Blood (2010)
Allogeneic Stem Cell Transplantation Conditioning for MDS and AML with Clofarabine, Cytarabine and ATG
Authors: Michael G. Martin, Geoffrey L. Uy, Elizabeth Procknow, Keith Stockerl-Goldstein, Amanda F. Cashen, Peter Westervelt, Camille N. Abboud, Kristan Augustin, Jingqin Luo, John F. DiPersio, Ravi Vij
Journal: Blood (2008)
Circulating Endothelial Progenitor Cells Are Increased in Patients with Myelofibrosis and Do Not Harbor V617F JAK-2 or W515L MPL Mutations
Authors: Elisa Bonetti, Vittorio Rosti, Laura Villani, Rita Campanelli, Gaetano Bergamaschi, Margherita Massa, Alessandro Pecci, Alessandro M. Vannucchi, Francesco Frassoni, Giovanni Barosi
Journal: Blood (2007)
AB0531 The Role of Hydroxychloroquine in ANCA Positive and Negative Vasculitis:
Authors: A. Casian, S. Sangle, D. D'Cruz
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> There is an unmet need for a less toxic, corticosteroid sparing therapy in ANCA vasculitis (AAV), as up to 50% of patients relapse by 5 years and 20% have sub-optimal disease control. Hydroxychloroquine (HCQ) has been effective and safe in autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis. There is mechanistic rationale for the effectiveness of hydroxychloroquine in vasculitis, considering its effect on immune mediators involved in pathogenesis, including B cell activating factors, Toll-like receptors, autoreactive T cells and cytokines. <h3>Objectives</h3> To assess retrospectively the efficacy and safety of hydroxychloroquine (HCQ) in patients with systemic vasculitis <h3>Methods</h3> Patients were identified by searching our departmental vasculitis database including 248 patients in total and electronic clinical records. Twenty-six patients received hydroxychloroquine along with corticosteroids and immunosuppressants. We assessed the effect of hydroxychloroquine on clinical symptoms and the median dose of corticosteroids required. <h3>Results</h3> Twenty six patients with various vasculitides were treated with hydroxychloroquine (median dose 200 mg OD): 6 patients with Henoch Schonlein purpura (HSP), 6 urticarial vasculitis, 6 with ANCA+ vasculitis (AAV) (4 PR3-ANCA, 2 MPO-ANCA), 1 Eosinophilic Granulomatosis with Polyangiitis (EGPA), 2 Takayasu arteritis, 2 Behcet9s disease, 1 adult Still9s disease (AOSD), 1 relapsing polychondritis, 1 polyarteritis nodosa (PAN). The female: male ratio was 21:5. Median age was 53 years. The median duration of HCQ treatment was 3 years. Sixteen patients experienced a reduction in arthralgia, skin rashes improved in 8 patients and completely resolved in a further 4. Eight patients could reduce corticosteroid doses (from 9 mg to 6 mg) and 3 discontinued corticosteroids. Four patients developed fewer vasculitic relapses, 6 felt less fatigued, 2 patients no longer experienced abdominal pain and diarrhoea, 2 improved their mood and ability to think clearly. The 6 patients with AAV experienced improvement in arthralgia, reduced their prednisolone doses by a third, had fewer relapses and felt less tired. One patient developed asymptomatic QT prolongation and stopped the hydroxychloroquine, with no other adverse events reported. <h3>Conclusions</h3> All 26 patients reported symptomatic benefits associated with hydroxychloroquine treatment, especially improvement in joint pains, fatigue, rash. Vasculitic relapses were less frequent, with a reduction in corticosteroid doses. Hydroxychloroquine was generally well tolerated. <h3>Disclosure of Interest</h3> None declared
Mel-140 and TBI vs Mel-200 Prior to Autologous Peripheral Blood Stem Cell Transplantation for Multiple Myeloma - a Single Institution Experience.
Authors: Nalini Janakiraman, UshaSree Chamarthy, Radhika Acharya, Vijaya Donthireddy, Ginny Kamboj, Nancy Oja-Tebbe, George Divine
Journal: Blood (2004)
Reduced-Intensity Conditioning Is Associated with Shorter Duration of Chronic GVHD Than Myeloablative Conditioning and Provides Very Good Quality of Life for Long-Term Survivors after Allogeneic Stem Cell Transplantation.
Authors: Avichai Shimoni, Izhar Hardan, Noga Shem-Tov, Avital Rand, Elena Ribakovsky, Ronit Yerushalmi, Arnon Nagler
Journal: Blood (2007)
T Regulatory Cells Obtained from Cord Blood Are Greatly Expandable and Exert Potent Suppressive Function.
Authors: Giovanni F. Torelli, Roberta Maggio, Nadia Peragine, Barbarella Lucarelli, Maria S. De Propris, Maria Screnci, Simona Salvatori, Anna P. Iori, Filippo Milano, Veronica Valle, Emilia Iannella, Anna Guarini, Robert Foa
Journal: Blood (2007)
Airway inflammation in COPD after long-term withdrawal of inhaled corticosteroids
Authors: Lisette I.Z. Kunz, Nick H.T. ten Hacken, Thérèse S. Lapperre, Wim Timens, Huib A.M. Kerstjens, Annemarie van Schadewijk, Judith M. Vonk, Jacob K. Sont, Jiska B. Snoeck-Stroband, Dirkje S. Postma, Peter J. Sterk, Pieter S. Hiemstra
Journal: European Respiratory Journal (2016)

Long-term treatment with inhaled corticosteroids (ICS) might attenuate lung function decline and decrease airway inflammation in a subset of patients with chronic obstructive pulmonary disease (COPD), and discontinuing ICS treatment could result in further lung function decline. We hypothesised that airway inflammation increases after ICS withdrawal following long-term ICS treatment in COPD. In the GLUCOLD-1 study (GL1), 114 patients with moderate-severe COPD were randomised to 6-month or 30-month treatment with fluticasone propionate (500 µg twice daily), 30-month treatment with fluticasone/salmeterol (500/50 µg twice daily) or placebo. During the 5-year follow-up study (GL2), patients were followed prospectively while being treated by their physician. Bronchial biopsies and induced sputum were collected at baseline, at 30 months (end of GL1) and at 7.5 years (end of GL2) to assess inflammatory cell counts. Data were analysed using linear mixed-effects models. In patients using ICS during GL1 and using ICS 0–50% of the time during GL2 (n=61/85), there were significant increases in GL2 bronchial CD3 + (fold change per year calculated as GL2 minus GL1 2.68, 95% CI 1.87–3.84), CD4 + (1.91, 95% CI 1.33–2.75) and CD8 + cells (1.71, 95% CI 1.15–2.53), and mast cells (1.91, 95% CI 1.36–2.68). The sputum total cell counts increased significantly in GL2 (1.90, 95% CI 1.42–2.54), as did counts of macrophages (2.10, 95% CI 1.55–2.86), neutrophils (1.92, 95% CI 1.39–2.65) and lymphocytes (2.01, 95% CI 1.46–2.78). ICS discontinuation increases airway inflammation in patients with moderate-severe COPD, suggesting that the anti-inflammatory effects of ICS in COPD are not maintained after ICS discontinuation.
Rituximab Treatment Affords Long Term Control in Thrombotic Thrombocytopenic Purpura (TTP): The University of Kansas Experience.
Authors: Meenakshi Thirunavu, Delva Deauna-Limayo, Jigar Patel, David C. Bodensteiner, Barry S. Skikne, Siddhartha S. Ganguly, Norma Lacy, Mervyn A. Sahud
Journal: Blood (2006)
Chemokine receptors in allergic diseases
Authors: L. Castan, A. Magnan, G. Bouchaud
Journal: Allergy (2016)

Under homeostatic conditions, as well as in various diseases, leukocyte migration is a crucial issue for the immune system that is mainly organized through the activation of bone marrow-derived cells in various tissues. Immune cell trafficking is orchestrated by a family of small proteins called chemokines. Leukocytes express cell-surface receptors that bind to chemokines and trigger transendothelial migration. Most allergic diseases, such as asthma, rhinitis, food allergies, and atopic dermatitis, are generally classified by the tissue rather than the type of inflammation, making the chemokine/chemokine receptor system a key point of the immune response. Moreover, because small antagonists can easily block such receptors, various molecules have been developed to suppress the recruitment of immune cells during allergic reactions, representing potential new drugs for allergies. We review the chemokines and chemokine receptors that are important in asthma, food allergies, and atopic dermatitis and their respectively developed antagonists.
Safety and Tolerability of CSL112, a Reconstituted, Infusible, Plasma-Derived Apolipoprotein A-I, After Acute Myocardial Infarction
Authors: C. Michael Gibson, Serge Korjian, Pierluigi Tricoci, Yazan Daaboul, Megan Yee, Purva Jain, John H. Alexander, P. Gabriel Steg, A. Michael Lincoff, John J.P. Kastelein, Roxana Mehran, Denise M. D’Andrea, Lawrence I. Deckelbaum, Bela Merkely, Maciej Zarebinski, Ton Oude Ophuis, Robert A. Harrington
Journal: Circulation (2016)

Human or recombinant apolipoprotein A-I (apoA-I) has been shown to increase high-density lipoprotein-mediated cholesterol efflux capacity and to regress atherosclerotic disease in animal and clinical studies. CSL112 is an infusible, plasma-derived apoA-I that has been studied in normal subjects or those with stable coronary artery disease. This study aimed to characterize the safety, tolerability, pharmacokinetics, and pharmacodynamics of CSL112 in patients with a recent acute myocardial infarction.The AEGIS-I trial (Apo-I Event Reducing in Ischemic Syndromes I) was a multicenter, randomized, double-blind, placebo-controlled, dose-ranging phase 2b trial. Patients with myocardial infarction were stratified by renal function and randomized 1:1:1 to CSL112 (2 g apoA-I per dose) and high-dose CSL112 (6 g apoA-I per dose), or placebo for 4 consecutive weekly infusions. Coprimary safety end points were occurrence of either a hepatic safety event (an increase in alanine transaminase >3 times the upper limit of normal or an increase in total bilirubin >2 times the upper limit of normal) or a renal safety event (an increase in serum creatinine >1.5 times the baseline value or a new requirement for renal replacement therapy).A total of 1258 patients were randomized, and 91.2% received all 4 infusions. The difference in incidence rates for an increase in alanine transaminase or total bilirubin between both CSL112 arms and placebo was within the protocol-defined noninferiority margin of 4%. Similarly, the difference in incidence rates for an increase in serum creatinine or a new requirement for renal replacement therapy was within the protocol-defined noninferiority margin of 5%. CSL112 was associated with increases in apoA-I and ex vivo cholesterol efflux similar to that achieved in patients with stable coronary artery disease. In regard to the secondary efficacy end point, the risk for the composite of major adverse cardiovascular events among the groups was similar.Among patients with acute myocardial infarction, 4 weekly infusions of CSL112 are feasible, well tolerated, and not associated with any significant alterations in liver or kidney function or other safety concern. The ability of CSL112 to acutely enhance cholesterol efflux was confirmed. The potential benefit of CSL112 to reduce major adverse cardiovascular events needs to be assessed in an adequately powered phase 3 trial.URL: https://clinicaltrials.gov. Unique identifier: NCT02108262.
Determination of BCR-ABL Overexpression by Correlating qRT-PCR and IP-FISH in Imatinib Treated CML Patients.
Authors: Michele Bianchini, Patricia Gargallo, Fernanda Alú, Paula Barreyro, Belen Rosales, Raquel Bengió, Carlos De Brasi, Irene Larripa
Journal: Blood (2007)
Mutations of Myeloid Transcription Factors AML1, CEBPα and PU.1 in Patients with De Novo Myelodysplastic Syndrome: An Analysis of Paired Bone Marrow Samples at Diagnosis and at AML Transformation.
Authors: Lee-Yung Shih, Der-Cherng Liang, Chein-Fuang Huang, Jin-Hou Wu, Po-Nan Wang, Ming-Chung Kuo, Po Dunn, Tzung-Chih Tang, Tung-Liang Lin, Hung Chang
Journal: Blood (2005)
Freezing and Cryostorage of Hematopoietic Progenitor Cells (HPC) Apheresis Using 5% Dimethyl Sulfoxide (DMSO) without Hydroxyethyl Starch (HES) in Cryocyte Freezing Bags and in Cryogenic Vials.
Authors: Wai Shun Chan, Andrew L. Pecora, Suzi Nadasi, Lester Myers, Margarita Munoz, Besa Jashari, Robert A. Preti
Journal: Blood (2006)
Refining Trauma-Focused Treatments for Servicemembers and Veterans With Posttraumatic Stress Disorder
Authors: Charles W. Hoge, Daniel J. Lee, Carl A. Castro
Journal: JAMA Psychiatry (2016)

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FRI0080 Effects of IL-6 Receptor Inhibition Therapy on The Serum Levels of IL-33 and IL-6 in Patients with Rheumatoid Arthritis
Authors: I.A. Choi, S.J. Lee, W. Park, S.H. Park, S.-C. Shim, H.J. Baek, D.-H. Yoo, H.A. Kim, S.K. Lee, Y.J. Lee, Y.E. Park, H.-S. Cha, E.Y. Lee, E.B. Lee, Y.W. Song
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Several pro-inflammatory cytokines such as TNF-α, IL-1, IL-6, IL-8 and IL-15 are known to be critical in synovial inflammatory process in RA and successful results have been obtained in RA treatment with targeting pro-inflammatory cytokines including TNF-α, IL-1 and IL-6. <h3>Objectives</h3> This study sought to investigate the role of IL-33 and IL-6 in RA patients receiving IL-6 receptor inhibition therapy. <h3>Methods</h3> We analyzed the association of the IL-33 and IL-6 level with disease activity and serologic features in 83 patients with RA. We also measured the serum level of IL-33 and IL-6 before and after the administration of tocilizumab for 24 weeks in 40 patients. <h3>Results</h3> Serum IL-33 level showed significant correlation with RF (rho =0.660, p&lt;0.001) but did not correlate with DAS28, ESR, hsCRP or RA duration. IL-6 level was significantly correlated with hsCRP (rho =0.482, p&lt;0.001) but not correlate with DAS28, ESR, RF or RA duration. There was no correlation between serum IL-6 and IL-33 levels. Serum IL-33 level significantly decreased after 24 weeks of IL-6 receptor inhibition in patients with RA (p&lt;0.001). When comparing subgroups according to ACR20 response, serum IL-33 levels were significantly decreased after 24 weeks of IL-6 receptor inhibition therapy in ACR20 responders (p&lt;0.001) but not in the non-responders (p=0.084). Baseline IL-33 levels were not significantly different between the two subgroups (p=0.765). Serum IL-6 levels were not significantly changed after 24 weeks of IL-6 receptor inhibition therapy (median 7.1 to 8.9 pg/mL, p=0.503). Changes of IL-6 levels were insignificant both in ACR20 responders and non-responders after 24 weeks of IL-6 receptor inhibition therapy. Baseline IL-6 levels were not different between ACR20 responders and non-responders. <h3>Conclusions</h3> The use of IL-6 receptor inhibitor decreased the serum level of IL-33 and this effect seems to be led by the responder group. IL-33 could be a useful indicator to monitor the response in IL-6 receptor inhibition therapy. <h3>Disclosure of Interest</h3> None declared
ENESTnext Final Results: Deep Molecular Response (MR) with Nilotinib (NIL) in Patients (pts) with Newly Diagnosed Chronic Myeloid Leukemia in Chronic Phase (CML-CP)
Authors: Jesus G. Berdeja, Michael Heinrich, Shaker Dakhil, Stuart L. Goldberg, Martha Wadleigh, Rosalind Catchatourian, Philip Kuriakose, Jorge E. Cortes, Jerald P. Radich, David A. Rizzieri, Gaetano Bonifacio, Ilva Dautaj, Ghulam Warsi, Michael J. Mauro
Journal: Blood (2015)
Donor CMV Status Influence On the Outcome of Allogeneic Stem Cell Transplantation (HSCT); A Study by the Infectious Diseases Working Party (IDWP) of the EBMT
Authors: Per T. Ljungman, Ronald Brand, Rafael De La Camara, Catherine Cordonnier, Hermann Einsele, Jan Styczynski, Katherine N. Ward, Simone Cesaro
Journal: Blood (2012)
Perceived Religiousness is Protective for Colorectal Cancer: Data from the Melbourne Colorectal Cancer Study
Authors: Gabriel A Kune, Susan Kune, Lyndsey F Watson
Journal: Journal of the Royal Society of Medicine (1993)

The perceived or self-reported degree of ‘religiousness’ was obtained by interview from 715 colorectal cancer patients and 727 age/sex matched community controls, as part of a large, comprehensive population-based study of colorectal cancer incidence, aetiology and survival (The Melbourne Colorectal Cancer Study) conducted in Melbourne, Australia. Self-reported or perceived ‘religiousness’, as defined in the study, was a statistically significant protective factor [relative risk (RR)=0.70, 95% confidence interval (CI)=0.6–0.9, P=0.002]. This statistically significant protection remained after the previously determined major risk factors found in the study, namely a family history of colorectal cancer, dietary risk factors, beer consumption, number of children and age at birth of the first child, were statistically corrected for ( P=0.004). There was no association between Dukes' staging of the cancer and perceived degree of ‘religiousness’ ( P=0.42). Although self-reported or perceived ‘religiousness’ was associated with a median survival time of 62 months compared with 52 months in those self-reporting as being ‘non-religious’, this difference was not statistically significant ( P=0.64).