tbuckley/cabot-search huggingface dataset:
AB0255 The Association between Rheumatoid Factor Positivity and Effects of Treatment with A First Biologic Agent in Rheumatoid Arthritis
Authors: Y. Ogawa, T. Kojima, N. Takahashi, K. Funahashi, N. Ishiguro
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> The presence of rheumatoid factor (RF) plays an important role in diagnosis for patients suspected with rheumatoid arthritis (RA) [1]. However, it remains unclear whether RF positivity is related to the effects of treatment with biologic disease-modifying antirheumatic drugs (DMARDS). <h3>Objectives</h3> The objective of the current study was to determine whether RF positivity was associated with the effects of treatment with biologic DMARDS among bio-naïve RA patients. <h3>Methods</h3> We used demographic and clinical data from the Tsurumai Biologics Communication Registry, which comprises Nagoya University and 20 affiliated hospitals in Japan. Patients aged 20–80 years who fulfilled the ACR 1987 revised classification criteria or the 2010 ACR/EULAR classification criteria for RA were selected; those with prior exposure to biologic DMARDS were excluded. The RF status was divided into negative (0–15 IU/ml) or positive (&gt;15 IU/ml). Multivariate logistic regression analysis was used to assess the association between RF positivity and the possibility of sufficient response to biologic DMARDS treatment, as defined by good response at week 16 in the EULAR response criteria, based on DAS28. Crude and adjusted odds ratios (ORs) and corresponding 95% confidence intervals (95% CIs) were calculated. Adjustment variables included sex, age, whether or not tumor necrosis factor inhibitor had been used, baseline use of methotrexate (MTX) and glucocorticoids, and baseline stage and class as defined by the Steinbrocker classification. <h3>Results</h3> The demographic and disease characteristics of the patients at baseline were comparable between RF-negative (n=120) and RF-positive (n=557) groups. The mean age was 57 years; 81% were women. Patients had longstanding RA (mean 9.3 years), and the baseline DAS28 was 5.1. Among the patients, 80% and 61% received MTX and glucocorticoids, respectively. A good response as per EULAR response criteria was identified in 73 (60.8%) RF-negative patients and 268 (48.1%) RF-positive patients. The crude OR for the association between RF positivity and the possibility of good response as per EULAR response criteria was 0.60 (95% CI 0.40–0.89) compared with RF negativity. After adjustment for the aforementioned covariates, RF-positivity was associated with a decreased possibility of good response to biologic DMARDS compared with RF-negativity (OR 0.64, 95% CI 0.41–0.98). <h3>Conclusions</h3> This study demonstrated that RF-positivity was independently associated with a decreased possibility of good response to biologic DMARDS in bio-naïve RA patients. <h3>References</h3> P.Y. Chang, C.T. Yang, C.H. Cheng, K.H. Yu, Diagnostic performance of anti-cyclic citrullinated peptide and rheumatoid factor in patients with rheumatoid arthritis, Int J Rheum Dis, (2015). <h3>Disclosure of Interest</h3> None declared
Human skin dendritic cell fate is differentially regulated by the monocyte identity factor Kruppel-like factor 4 during steady state and inflammation
Authors: Jennifer Jurkin, Corinna Krump, René Köffel, Christina Fieber, Christopher Schuster, Patrick M. Brunner, Izabela Borek, Gregor Eisenwort, Clarice Lim, Jörg Mages, Roland Lang, Wolfgang Bauer, Diana Mechtcheriakova, Anastasia Meshcheryakova, Adelheid Elbe-Bürger, Georg Stingl, Herbert Strobl
Journal: Journal of Allergy and Clinical Immunology (2016)

Langerhans cell (LC) networks play key roles in immunity and tolerance at body surfaces. LCs are established prenatally and can be replenished from blood monocytes. Unlike skin-resident dermal DCs (dDCs)/interstitial-type DCs and inflammatory dendritic epidermal cells appearing in dermatitis/eczema lesions, LCs lack key monocyte-affiliated markers. Inversely, LCs express various epithelial genes critical for their long-term peripheral tissue residency.Dendritic cells (DCs) are functionally involved in inflammatory diseases; however, the mechanisms remained poorly understood.In vitro differentiation models of human DCs, gene profiling, gene transduction, and immunohistology were used to identify molecules involved in DC subset specification.Here we identified the monocyte/macrophage lineage identity transcription factor Kruppel-like factor 4 (KLF4) to be inhibited during LC differentiation from human blood monocytes. Conversely, KLF4 is maintained or induced during dermal DC and monocyte-derived dendritic cell/inflammatory dendritic epidermal cell differentiation. We showed that in monocytic cells KLF4 has to be repressed to allow their differentiation into LCs. Moreover, respective KLF4 levels in DC subsets positively correlate with proinflammatory characteristics. We identified epithelial Notch signaling to repress KLF4 in monocytes undergoing LC commitment. Loss of KLF4 in monocytes transcriptionally derepresses Runt-related transcription factor 3 in response to TGF-β1, thereby allowing LC differentiation marked by a low cytokine expression profile.Monocyte differentiation into LCs depends on activation of Notch signaling and the concomitant loss of KLF4.
OP0254-PARE Rheumatoid Arthritis Patient Characteristics and Willingness To Participate in Research among Members of The Creakyjoints Arthritis Patient Community: Results from A Patient Survey
Authors: W.B. Nowell, N. Gerber, J.R. Curtis, B. Johnson, K. Saag, M. Safford, J. Willig, S. Ginsberg
Journal: Annals of the Rheumatic Diseases (2016)

Background  CreakyJoints (CJ) is an international online arthritis patient community offering support, blogs, education, advocacy and the opportunity to participate in research.  Objectives  To identify willingness to participate in research among rheumatoid arthritis (RA) patients in an online community.  Methods  CJ patient community completed an online form preparatory to launch of a PCORI-funded research network (ArthritisPower.org). The online survey captured self-reported demographic, diagnosis, treatment, and willingness to participate in research.  Results  As of January 2016, a total of 8923 patients had registered. Among respondents, 2147 identified themselves as patients who had RA; a subgroup of 970 completed the survey on willingness to participate in research.  Demographics (N=2147):  Respondents were mostly female (93%) and white, non-Hispanic (85%) with mean age of 51.7 years (SD 11.6).  Conditions & Treatments (N=2147):  The most frequent concomitant conditions reported by RA respondents were osteoarthritis (32%), fibromyalgia (31%), and osteoporosis (13%). A majority reported taking a classical DMARD (e.g. MTX) (59%) or a biologic (50%) including anti-TNF biologics (33%); only 23% indicated they were taking neither.  Willingness to Participate in Research (N=970):  Most patients (68%) had never taken part in research. Among U.S. respondents, a majority (75%) would refuse to participate in research that requires sharing Social Security Number (SSN) and 50% for the last 4 digits of SSN compared with only 20% for email and 34% for medical records. Top 3 reasons patients would not be interested in being part of medical research were: project not recommended by doctor (30%), individual results may not be kept private/confidential (28%), and project will take a lot of time (25%). Table 1 shows percentage of patients interested in research based on the source of the invitation.  Conclusions  Results from this patient community survey indicate substantial willingness to participate in rheumatology research yet identify a number of potential barriers. Considering patients as unique stakeholders is vital to maximize the relevance and patient-centeredness of future research endeavors.  Disclosure of Interest  None declared
Prevalence and characteristics of<i>TERT</i>and<i>TERC</i>mutations in suspected genetic pulmonary fibrosis
Authors: Raphael Borie, Laure Tabèze, Gabriel Thabut, Hilario Nunes, Vincent Cottin, Sylvain Marchand-Adam, Grégoire Prevot, Abdellatif Tazi, Jacques Cadranel, Herve Mal, Lidwine Wemeau-Stervinou, Anne Bergeron Lafaurie, Dominique Israel-Biet, Clement Picard, Martine Reynaud Gaubert, Stephane Jouneau, Jean-Marc Naccache, Julie Mankikian, Christelle Ménard, Jean-François Cordier, Dominique Valeyre, Marion Reocreux, Bernard Grandchamp, Patrick Revy, Caroline Kannengiesser, Bruno Crestani
Journal: European Respiratory Journal (2016)

Telomerase reverse transcriptase (TERT) or telomerase RNA (TERC) gene mutation is a major monogenic cause of pulmonary fibrosis. Sequencing of TERT/TERC genes is proposed to patients with familial pulmonary fibrosis. Little is known about the possible predictors of this mutation and its impact on prognosis.We retrospectively analysed all the genetic diagnoses made between 2007-2014 in patients with pulmonary fibrosis. We evaluated the prevalence of TERT/TERC disease-associated variant (DAV), factors associated with a DAV, and the impact of the DAV on survival.237 patients with pulmonary fibrosis (153 with familial pulmonary fibrosis, 84 with telomere syndrome features without familial pulmonary fibrosis) were tested for TERT/TERC DAV. DAV was diagnosed in 40 patients (16.8%), including five with non-idiopathic interstitial pneumonia. Prevalence of TERT/TERC DAV did not significantly differ between patients with familial pulmonary fibrosis or with only telomere syndrome features (18.2% versus 16.4%). Young age, red blood cell macrocytosis, and low platelet count were associated with the presence of DAV; the probability of DAV was increased for patients 40-60 years. Transplant-free survival was lower with than without TERT/TERC DAV (4.2 versus 7.2 years; p=0.046).TERT/TERC DAV were associated with specific clinical and biological features and reduced transplant-free survival.
AB0086 Anti-Citrullinated Protein Antibodies in Patients with Cardiovascular Disease without RA
Authors: D. Van Der Woude, M.P. Hermans, D. van der Velden, L.A. Trouw, T.W. Huizinga, J. Kuiper, M.J. Schalij, W.J. Jukema, R.E. Toes
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Anti-citrullinated protein antibodies (ACPA) are thought to be highly specific for RA. ACPA are associated with risk factors for RA and with joint destruction, and are therefore presumed to be involved in RA pathogenesis. ACPA-positive RA patients also have increased cardiovascular mortality (1). In cardiovascular disease, inflammatory changes occur in vessel walls, raising the question whether ACPA (presumed to be pro-inflammatory) may contribute to this process. <h3>Objectives</h3> To investigate the prevalence and prognostic implications of ACPA in patients with cardiovascular disease without RA. <h3>Methods</h3> ACPA were determined by anti-CCP3 commercial assay in baseline sera from 290 patients with ST-elevation myocardial infarction participating in the MISSION intervention study (2). Patients with RA were excluded. The association between ACPA and long-term mortality was investigated. <h3>Results</h3> 30/290 (10.3%) of the non-RA patients with cardiovascular disease were ACPA-positive. ACPA-positive non-RA patients had a significantly increased long-term cardiac mortality compared to ACPA-negative non-RA patients (Figure). Corrected for age, ACPA positivity was independently associated with long-term mortality [HR 2.4 (CI 1.1–5.4) p-Value=0.026]. <h3>Conclusions</h3> ACPA can be detected in a considerable proportion of non-RA patients with cardiovascular disease. This challenges the presumed specificity of ACPA for RA. In both RA and cardiovascular disease, ACPA are associated with a worse disease outcome possibly by an ACPA-specific enhancement of inflammation. Future studies into ACPA in patients with cardiovascular disease offer the opportunity to dissect which risk factors are associated with ACPA in RA versus non-RA patients. This may supply crucial insights into the development of this autoimmune reaction. <h3>References</h3> Humphreys JH et al. Arthritis care &amp; research 2014;66(9):1296–301. Liem et al. American Heart Journal 2007;153(1):11. <h3>Disclosure of Interest</h3> None declared
P08.10 Postoperative hypofractionated accelerated intensity modulated radiotherapy with concurrent and adjuvant temozolomide in the management of patients with newly diagnosed glioblastoma: results of a pilot study
Authors: A. Biswas, P. K. Julka
Journal: Neuro-Oncology (2016)

was 39% (95% CI 21-57).No significant correlation was found between clinical outcome measures and the MGMT promoter methylation status or IDH1/2 mutation status.CONCLUSION: Axitinib has single-agent activity in patients with recurrent GB; upfront combination of axitinib and LOM did not significantly increase progression-free or overall survival in patients with recurrent glioblastoma.Adding CCNU to axitinib after progression seems to result in improved PFS compared to upfront combination therapy. P08.
Monocytes Induced to Differentiate Into Dendritic Cells After Overnight Transduction with Lentiviral Vectors Co-Expressing GM-CSF/ IL-4 and a Truncated Form of WT1: Preclinical Validation for Immunotherapy of High Risk Leukemia Patients After Allo-SCT
Authors: Renata Stripecke, Bala Sai Sundarasetty, Mareike Rickmann, Gustavo Salguero, Arnold Ganser
Journal: Blood (2011)
Prospective Randomized Comparison of High Dose AraC and Autologous Peripheral Blood Stem Cell Transplantation as Late Consolidation for Patients ≤60 Years with Standard Risk AML.
Authors: Arnold Ganser, Jürgen Krauter, Dieter Hoelzer, Oliver Ottmann, Hans Martin, Michael Lübbert, Jürgen Finke, Wolf Heit, Walter Fiedler, Lothar Kanz, Günter Schlimok, Hartmut Kirchner, Aruna Raghavachar, Peter Brossart, Wolfram Brugger, Gerhard Heil
Journal: Blood (2005)
OP0154-HPR Spinal Mobility Is Impaired in Patients with Chronic Back Pain of Short Duration, but Does Not Discriminate between Patients with Axial Spondyloarthritis and Other Forms of CBP: Table 1.
Authors: C. Fongen, H. Dagfinrud, I.J. Berg, S. Ramiro, F. van Gaalen, R. Landewé, R. Ramonda, M. van Oosterhout, D. van der Heijde, K.M. Fagerli
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Ankylosing spondylitis is associated with reduced spinal mobility. Little is known about the spinal mobility in early axial spondyloarthritis (axSpA) and how it compares to patients with other forms of chronic back pain (CBP). <h3>Objectives</h3> The aim was to compare spinal mobility in patients with CBP of short duration with reference mobility curves from the general population and to examine whether frequently used spinal mobility measures (SMM) can discriminate between axSpA and other forms of CBP. <h3>Methods</h3> The SPACE-cohort includes patients with CBP (≥3 months, ≤2 years, onset &lt;45 years) recruited from 5 centres in Europe. The CBP patients were classified as axSpA (fulfilling the ASAS criteria), possible axSpA (possSpA) (not fulfilling ASAS criteria but ≥1 axSpA feature) or not having SpA (noSpA). Spinal mobility was assessed with lateral spinal flexion (LSF), chest expansion (CE), intermalleolar distance (IMD), modified Schober (MS), cervical rotation (CR) and occiput-to-wall distance (OWD). SMM, except OWD, were defined as impaired if below the 5th percentile reference curve from general population, given their age and for CE and IMD also height, following the equations of mobility curves for each measures<sup>1</sup>. OWD &gt;0 was considered impaired. The proportion of patients with impaired spinal mobility for each measure was compared across the patient groups using Chi square. <h3>Results</h3> In total 393 CBP-patients were included: 142 axSpA, 139 possSpA and 112 noSpA. Some characteristics of patients with axSpA were statistically significant different compared to possSpA and noSpA: a larger proportion (%) was male 48/28/27, reported less back pain (NRS 0–10) median 4.0/6.0/6.0 and patients with axSpA were taller (cm) than patients with possSpA mean 177/173/174. In all CBP patients, the most frequently impaired mobility measure was LSF 40% followed by CE 22%, CR 18%, IMD 17%, MS 15% and OWD 11% (table 1). No statistically significant differences in proportion of patients with impaired spinal mobility were found between the CBP subgroups. <h3>Conclusions</h3> Among patients with CBP of short duration, between15–40% had a spinal mobility below the 5th percentile in the general population depending on the measurement used. The most affected SMM was LSF. Impaired spinal mobility was equally prevalent in patients with axSpA and other causes of CBP. Our findings suggest that the SMM recommended for axSpA may be of little differential diagnostic value. <h3>References</h3> Ramiro ARD 2015 <h3>Disclosure of Interest</h3> None declared
DIPSS-Plus: A Refined Dynamic International Prognostic Scoring System (DIPSS) for Primary Myelofibrosis That Incorporates Karyotype, Platelet Count and Transfusion Status
Authors: Naseema Gangat, Domenica Caramazza, Rakhee Vaidya, Geeta George, Kebede Begna, Susan Schwager, Daniel L. Van Dyke, Curtis Hanson, Wenting Wu, Animesh Pardanani, Francisco Cervantes, Francesco Passamonti, Ayalew Tefferi
Journal: Blood (2010)
Autoclave Sterilization of PEDOT:PSS Electrophysiology Devices
Authors: Ilke Uguz, Mehran Ganji, Adel Hama, Atsunori Tanaka, Sahika Inal, Ahmed Youssef, Roisin M. Owens, Pascale P. Quilichini, Antoine Ghestem, Christophe Bernard, Shadi A. Dayeh, George G. Malliaras
Journal: Advanced Healthcare Materials (2016)

Autoclaving, the most widely available sterilization method, is applied to poly(3,4-ethylenedioxythiophene) doped with polystyrene sulfonate (PEDOT:PSS) electrophysiology devices. The process does not harm morphology or electrical properties, while it effectively kills E. coli intentionally cultured on the devices. This finding paves the way to widespread introduction of PEDOT:PSS electrophysiology devices to the clinic. As a service to our authors and readers, this journal provides supporting information supplied by the authors. Such materials are peer reviewed and may be re-organized for online delivery, but are not copy-edited or typeset. Technical support issues arising from supporting information (other than missing files) should be addressed to the authors. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.
Phase II Study of Decitabine in Combination with Imatinib Mesylate in Patients with Accelerated (AP) or Blastic Phase (BP) of Chronic Myeloid Leukemia (CML).
Authors: Yasuhiro Oki, Hagop M. Kantarjian, Susan O’Brien, Srdan Verstovsek, Vazaganush Gharibyan, Jorge Cortes, Gail M. Morris, Guillermo Garcia-Manero, Jean-Pierre Issa
Journal: Blood (2005)
Skin manifestations in ultramarathon runners: experience in the Marathon des Sables 2014
Authors: V. Descamps, Y.-E. Claessens, B. Doumenc
Journal: British Journal of Dermatology (2016)

Ultra-marathons are becoming increasingly popular (1). In 2014 (4th to 14th April), the 29th Sultan Marathon des Sables (MDS) took place in Morocco. Overall, 1100 runners, men and women aged 16 to 80, met this challenge. Their goal was an endurance race of around 250 km in 5 self-sufficient stages in the desert over 6 days. Each runner carried their own rucksack of 7-10 kg with all of the necessary equipment and food (except water). Ultra-marathons are associated with well-known problems including dehydration, changes in body mass, loss of skeletal muscle mass, rhabdomyolysis with renal failure, and an increase in total body water. Lower limb and foot injuries are frequent in ultra-marathon runners, but specific skin manifestations may occur as a consequence of the extreme conditions of the MDS: high temperature with high rate of perspiration and salt loss, sun exposure, and contact with the sand.

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Sequential Regimen of Clofarabine, Cytarabine and Reduced Intensity Conditioning (RIC) Prior to Allogeneic Stem Cell Transplantation (allo-SCT) for Acute Myeloid Leukemia (AML) in Primary Treatment Failure
Authors: Mohamad Mohty, Florent Malard, Didier Blaise, Noel Milpied, Gerard Socie, Anne Huynh, Reman Oumedaly, Ibrahim Yakoub-Agha, Sabine Furst, Thierry Guillaume, Reza Tabrizi, Jacques Delaunay, Philippe Moreau, Myriam Labopin, Patrice Chevallier
Journal: Blood (2014)
AB0890 Neuropsychiatric Manifestations in Pediatric-Onset Systemic Lupus Erythematosus: Experience of A spanish Tertiary Center: Table 1.
Authors: W.A. Sifuentes Giraldo, S. Garrote Corral, A.L. Boteanu, M.L. Gámir Gámir, A. Zea Mendoza
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> The neuropsychiatric (NP) manifestations are an important cause of morbidity and mortality in patients with pediatric-onset systemic lupus erythematosus (pSLE). The prevalence of these manifestations ranges from 29% to 44% in different series, but data in the spanish pediatric population are scarce. <h3>Objectives</h3> To analyze the clinical and immunological features of patients with pSLE and NP followed in a Spanish tertiary center. <h3>Methods</h3> We performed a retrospective study of 49 patients with pSLE diagnosed between 1985 and 2005 in our center. The ACR NPSLE case definitions were used for classification. Demographic, clinical and immunological data were obtained through review of their medical charts. <h3>Results</h3> Twenty six patients (53%) developed NP manifestations. Mean age was 13.6 years and female:male ratio was 2.7:1. NP manifestations were present at the beginning of the disease in 7 cases (26.9%) and the mean time to develop them was 30.5 months. The most common presentations of NPSLE were seizures (50%), headache (26.9%), mood disorder/depression (26.9%), psychosis (19.2%), cerebrovascular disease (15.3%) and aseptic meningitis (15.3%). There was more than one NP manifestation in 15 cases (57.7%), with an average of 2.4 manifestations/patient. The comparison of patients with and without NPSLE demonstrated significant diffences (p&lt;0.05) in the number of males, titers of antinuclear antibodies (ANA) and anti-DNA antibodies, positivity for anti-β<sub>2</sub>-glicoprotein I (β<sub>2</sub>GPI) and cryoglobulins, high erythrosedimentation rate (ESR) and low complement (C3, C4) (Table 1). There were 2 cases of mortality in NPSLE (7.7%) during follow-up period, one as a result of infection of the central nervous system and another due to sepsis associated with intestinal thrombosis. <h3>Conclusions</h3> In our series &gt;50% of patients had NPSLE manifestations and frequently occurred early during the course of the disease. The clinical spectrum of NPSLE was wide in our cases and most of them had more than one manifestation. Patients with NPSLE showed a higher disease activity as measured by levels of autoantibodies, increased acute-phase reactants and low complement. Although NPSLE has been associated with antiphospholipid antibodies in other series, specifically anticardiolipin antibodies and lupus anticoagulant, we only found significant association with anti-β<sub>2</sub>GPI antibodies. Mortality in patients with NPSLE was high. <h3>Disclosure of Interest</h3> None declared
Efficacy and Safety of Deferasirox (Exjade®) in Reducing Cardiac Iron in Patients with β-Thalassemia Major: Results from the Cardiac Substudy of the EPIC Trial
Authors: Dudley Pennell, John B Porter, Maria Domenica Cappellini, Chi-Kong Li, Yesim Aydinok, Chan Lee Lee, Antonis Kattamis, Gillian Smith, Dany Habr, Gabor Domokos, Abdel Hmissi, Ali Taher
Journal: Blood (2008)
Ontogenic-Specific Increasesin HDAC1 Activity and Transcription Factor Association During the Maturation of Human Adult Erythroblasts in Vitro.
Authors: Giovanni Migliaccio, Carmela Dell’Aversana, Angela Nebbioso, Elena Alfani, Lilian arricchio, Antonello Mai, Pratima Chaurasia, Ronald Hoffman, James J. Bieker, Lucia Altucci, Anna Rita Migliaccio
Journal: Blood (2009)