tbuckley/cabot-search huggingface dataset:
FRI0254 The Effect of Cyclophosphamide on Lung Function in Different Stages of Interstitial Lung Disease Associated To Systemic Sclerosis: Table 1.
Authors: W.M.T. Van Den Hombergh, E. Teesselink, H.K.A. Knaapen-Hans, S.O. Simons, F.H.J. van den Hoogen, J. Fransen, M.C. Vonk
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> The primary cause of death in patients with systemic sclerosis (SSc) is interstitial lung disease (ILD). Several retrospective studies have shown some effect of cyclophosphamide (CYC) on ILD. In one randomized controlled trial a small but significant effect on pulmonary function was shown after oral CYC (Taskin,2006). Hoyles (2006) showed a trend towards improvement of forced vital capacity after monthly CYC infusions. In our centre, intravenous CYC pulses are the first choice of therapy in SSc-ILD. <h3>Objectives</h3> To analyze 1) the effects of iv CYC pulses (750mg/m<sup>2</sup>) on pulmonary function in SSc-ILD in our cohort, and 2) whether this effect is dependent on the extent of ILD. <h3>Methods</h3> Patients with SSc-ILD receiving CYC pulses between 2003 to 2015 were classified by the Goh criteria (2008) in either limited or extensive ILD, using HRCT at baseline judged by two raters. Pulmonary function tests were performed at 0, 6 and 12 months. Missing outcome data due to drop-out, but not death, were replaced by last observation carried forward. <h3>Results</h3> 79 patients were included, 42 with limited ILD, 37 with extensive ILD. There were no differences in age, gender, SSc subtype classification, disease duration or autoantibody status between the two groups. Baseline FVC (median 80% vs. 91%) and DLCO (39% vs. 50%) were lower in extensive ILD (p&lt;0.05). After 12 months, FVC (p=0.08)), and DLCO (p=0.62) did not worsen compared to baseline values. There were no differences in the degree of change in FVC and DLCO between limited and extensive ILD. Forty-eight patients completed 12 cycles of CYC. Per protocol analyses showed not significant differences between limited and extended ILD. <h3>Conclusions</h3> 1. CYC pulse therapy does not worsen pulmonary function in SSc-related ILD after 1 year. 2. The extent of ILD does not influence the effect of CYC. <h3>Disclosure of Interest</h3> None declared
New Interleukin-15 Superagonist (IL-15 SA) Combined with Donor Lymphocyte Infusion (DLI) Significantly Enhances Graft-Versus-Tumor Activity after Allogeneic Hematopoietic Stem Cell Transplantation
Authors: Cavan Bailey, Michelle M Panis, Cihangir Buyukgoz, Tulin Budak-Alpdogan, Neal Flomenberg, Onder Alpdogan
Journal: Blood (2014)
Perivascular cells and tissue engineering: Current applications and untapped potential
Authors: Elisa Avolio, Valeria V. Alvino, Mohamed T. Ghorbel, Paola Campagnolo
Journal: Pharmacology & Therapeutics (2016)

The recent development of tissue engineering provides exciting new perspectives for the replacement of failing organs and the repair of damaged tissues. Perivascular cells, including vascular smooth muscle cells, pericytes and other tissue specific populations residing around blood vessels, have been isolated from many organs and are known to participate to the in situ repair process and angiogenesis. Their potential has been harnessed for cell therapy of numerous pathologies; however, in this Review we will discuss the potential of perivascular cells in the development of tissue engineering solutions for healthcare. We will examine their application in the engineering of vascular grafts, cardiac patches and bone substitutes as well as other tissue engineering applications and we will focus on their extensive use in the vascularization of engineered constructs. Additionally, we will discuss the emerging potential of human pericytes for the development of efficient, vascularized and non-immunogenic engineered constructs.
Mitochondria Protection after Acute Ischemia Prevents Prolonged Upregulation of IL-1β and IL-18 and Arrests CKD
Authors: Hazel H. Szeto, Shaoyi Liu, Yi Soong, Surya V. Seshan, Leona Cohen-Gould, Viacheslav Manichev, Leonard C. Feldman, Torgny Gustafsson
Journal: Journal of the American Society of Nephrology (2016)

The innate immune system has been implicated in both AKI and CKD. Damaged mitochondria release danger molecules, such as reactive oxygen species, DNA, and cardiolipin, which can cause NLRP3 inflammasome activation and upregulation of IL-18 and IL-1β It is not known if mitochondrial damage persists long after ischemia to sustain chronic inflammasome activation. We conducted a 9-month study in Sprague-Dawley rats after 45 minutes of bilateral renal ischemia. We detected glomerular and peritubular capillary rarefaction, macrophage infiltration, and fibrosis at 1 month. Transmission electron microscopy revealed mitochondrial degeneration, mitophagy, and deformed foot processes in podocytes. These changes progressed over the study period, with a persistent increase in renal cortical expression of IL-18, IL-1β, and TGF-β, despite a gradual decline in TNF-α expression and macrophage infiltration. Treatment with a mitoprotective agent (SS-31; elamipretide) for 6 weeks, starting 1 month after ischemia, preserved mitochondrial integrity, ameliorated expression levels of all inflammatory markers, restored glomerular capillaries and podocyte structure, and arrested glomerulosclerosis and interstitial fibrosis. Further, helium ion microscopy vividly demonstrated the restoration of podocyte structure by SS-31. The protection by SS-31 was sustained for ≥6 months after treatment ended, with normalization of IL-18 and IL-1β expression. These results support a role for mitochondrial damage in inflammasome activation and CKD and suggest mitochondrial protection as a novel therapeutic approach that can arrest the progression of CKD. Notably, SS-31 is effective when given long after AKI and provides persistent protection after termination of drug treatment.
SAT0239 The Cumulative Number of Micro-Haemorrhages and Micro-Thrombosis in Nailfold Videocapillaroscopy Is A Good Predictor of Disease Activity in Systemic Sclerosis: A Validation Study of Nemo Score
Authors: N. Del Papa, R. Irace, R. Andracco, D. Sambataro, S. Vettori, F. Pignataro, R. Ferrara, E. Zaccara, W. Maglione, C. Vitali, G. Valentini
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> In a previous study it has been shown shown that, in patients with systemic sclerosis (SSc), the cumulative number of micro-haemorrhages (MH) and micro-thrombosis (MT), computed in eight finger nailfold videocapillaroscopy (NVC), and called NEMO score, was strongly predictive of disease activity (DA) measured by means of European Scleroderma Study Group (ESSG) score. Even the number of giant capillaries (GC) was correlated to a lesser extent with the ESSG score, and then a modified (m) NEMO score was proposed in which the combined presence of a MH/MT and GC was considered [1]. <h3>Objectives</h3> The present study was aimed at validating NEMO score in different cohorts of patients. <h3>Methods</h3> NEMO score and the total number of GC were assessed by eight finger NVC in 122 patients with SSc referring to the Rheumatic Disease Unit of the “Istituto G.Pini” of Milan, where the preliminary study had been carried out (internal validation). The same procedure was performed in 99 patients collected in a different centre, i.e., the Rheumatic Dept. of the II University of Naples (external validation). In all of the patients DA, measured by ESSG score, was assessed at the same time of NVC performance. <h3>Results</h3> Demographic and clinical characteristics of the patients were summarised in Table 1. Table 2 shows the main statistical results obtained by the analysis of the two cohorts. The mNEMO score does not add anything in terms of sensitivity and specificity to the performance of the simple NEMO score in predicting DA. <h3>Conclusions</h3> This validation study confirms that the presence of a certain number of MH plus that of MT in eight finger NVC may represent a simple screening method to predict the presence of DA in patients with SSc. <h3>References</h3> Sambataro D, et al. Arthritis Res &amp; Ther 2014;16:462. <h3>Disclosure of Interest</h3> None declared
Interferon-γ Enhances PML Protein Expression in Acute Promyelocytic Leukemia Cells and Cooperates with All-Trans Retinoic Acid to Induce Maturation of NB4 and MR2 Cells
Authors: Pengcheng He, Mei Zhang, Xiaoning Wang, Jun Qi, Jieying Xi, Jing Li, Di Wu, Yunxin Cao
Journal: Blood (2008)
Single Cell Analysis of IG Genes in CLL: Cases with Multiple IGH Rearrangements Are Constituted of Several Independent Clones Even When Indistinguishable By Flow Cytometry
Authors: Kamila Brazdilova, Karla Plevova, Hana Skuhrova Francova, Helena Kockova, Magdalena Chmelikova, Marek Borsky, Katerina Burckova, Barbara Kantorova, Boris Tichy, Hana Skabrahova, Yvona Brychtova, Jiri Mayer, Michael Doubek, Sarka Pospisilova
Journal: Blood (2015)
High-Dose Chemotherapy and Autologous Peripheral-Blood Stem-Cell Transplantation for Relapsed Metastatic Germ Cell Tumors: The Indiana University Experience
Authors: Nabil Adra, Rafat Abonour, Sandra K. Althouse, Costantine Albany, Nasser H. Hanna, Lawrence H. Einhorn
Journal: Journal of Clinical Oncology (2017)

Purpose Patients with relapsed metastatic germ cell tumor (GCT) can be cured with second-line and even third-line regimens. We report survival outcomes of patients treated with high-dose chemotherapy (HDCT) and peripheral-blood stem-cell transplantation (PBSCT) at Indiana University between 2004 and 2014. Patients and Methods We conducted a retrospective analysis of 364 consecutive patients with GCT who progressed after cisplatin-based combination chemotherapy and were subsequently treated with HDCT and PBSCT. Three hundred forty-one patients received two consecutive courses of HDCT consisting of 700 mg/m 2 carboplatin and 750 mg/m 2 etoposide, each for 3 consecutive days, and each followed by PBSCT. Twenty-three patients received only a single course of HDCT because of progressive disease or toxicity. Cox proportional hazards models were used to test predictors of disease progression. Results The median age was 32 years (range, 17 to 70 years). With a median follow-up of 3.3 years, the 2-year progression-free survival (PFS) was 60% (95% CI, 55% to 65%) and the 2-year overall survival was 66% (95% CI, 60% to 70%). Three hundred three patients received HDCT as second-line therapy with a 2-year PFS of 63% (95% CI, 57% to 68%), and 61 patients received HDCT as third-line or later therapy with a 2-year PFS of 49% (95% CI, 36% to 61%). In a multivariable analysis, factors associated with disease progression included use of HDCT as third-line or later therapy, platinum-refractory disease, mediastinal primary tumor site, nonseminoma histology, intermediate- or poor-risk disease at the time of GCT diagnosis, and human chorionic gonadotropin ≥ 1,000 mIU/mL at initiation of HDCT. There were nine treatment-related deaths. Secondary leukemia developed in five patients. Conclusion This large single-institution study demonstrates that patients with relapsed metastatic GCT are curable by HDCT plus PBSCT even when used in third-line or later therapy.
Capturing the biology of disease severity in a PSC-based model of familial dysautonomia
Authors: Nadja Zeltner, Faranak Fattahi, Nicole C Dubois, Nathalie Saurat, Fabien Lafaille, Lei Shang, Bastian Zimmer, Jason Tchieu, Mohamed A Soliman, Gabsang Lee, Jean-Laurent Casanova, Lorenz Studer
Journal: Nature Medicine (2016)
AB0236 The Role of Vitamin D Receptor in Rheumatoid Arthritis
Authors: P. Nemec, M. Sefcik, J. Sevcikova, L. Prochazkova, M. Pavkova-Godbergova
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Rheumatoid arthritis (RA) is a disease characterized by chronic inflammation of synovial tissue, and progressive destruction of the joints. In RA patients a high prevalence of serum vitamin D deficiency/insufficiency was described. Vitamin D has a central role in the immune system and also correlates with increased levels of estrogen receptor α. <h3>Objectives</h3> The study analyzed the relationships between polymorphisms in the gene for vitamin D receptor (VDR) and the development and/or progression of the disease. <h3>Methods</h3> A total of 156 patients were enrolled with a diagnosis of RA (median age 55.6 years; 78% of them women; control group of 50 subjects). Development of the disease was scored by positive antibodies against cyclic citrullinated peptide (anti-CCP), rheumatoid factor (RF), CRP. Disease progression was assessed by the modified Sharp-van der Heijdeovou method Total Sharp Score (TSS). Polymorphisms were detected by PCR and subsequent restriction analysis (rs4516035 EcoRV; rs7975232: ApaI; rs731236: TaqI). <h3>Results</h3> The results show the relation of polymorphism VDR rs4516035 to seropositivity for anti-CCP in the distribution of genotypes (P=0.03), with a higher prevalence of the TT genotype when compared to the CC + CT variants (p=0.007, OR =3.2). The relationship was found also in the frequency of alleles (p=0.04). Further, a borderline relationship of this polymorphism to RF IgA levels (P=0.06) was detected. A relationship of polymorphism VDR rs731236 with the disease progression evaluated by TSS (p=0.02) was demonstrated, with the highest TSS in the patients carrying the GG variant. <h3>Conclusions</h3> The results show that the GG variant of VDR rs731236 polymorphism was associated with a worse prognosis of RA. Moreover, the VDR rs4516035 polymorphism was related to the seropositivity for anti-CCP, with a significant prevalence of TT genotype in positive RA patients. <h3>Disclosure of Interest</h3> None declared
SAT0372 Impact of Dose Tapering of Tumour Necrosis Factor-Blockers on Radiographic Progression in The Ankylosing Spondylitis – A 4 Year Prospective Follow Up from Single-Centre Cohort
Authors: J.W. Park, H.M. Kwon, J.K. Park, E.B. Lee, Y.W. Song, E.Y. Lee
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Although dose tapering of tumour necrosis factor (TNF)-blockers in ankylosing spondylitis (AS) showed a comparable clinical efficacy with favourable safety and cost in some studies, there has been no investigation comparing the radiographic progression of patients with tapering regimen versus patients with standard-dose treatment. <h3>Objectives</h3> To investigate the impact of dose reduction of TNF-blockers on radiographic progression in the AS over 4 years <h3>Methods</h3> One hundred sixty five patients treated with etanercept or adalimumab were selected from single-centre, prospective observational cohort based on the availability of cervical and lumbar radiographs at baseline and after 2 and/or 4 years of the treatment. Radiographs were scored using modified Stokes AS spinal score (mSASSS) by two independent readers. Radiographic progression of patients treated with standard-dose (standard-dose group, n=49) was compared with that of patients who tapered the dosage during the TNF-blocker treatment (tapering group, n=116) using linear mixed models. <h3>Results</h3> Demographic and clinical factors at baseline between two groups were not significantly different except for higher initial BASDAI (7.1 vs. 6.3, p=0.003) in the standard-dose group. Baseline mSASSS (SD) of the standard-dose group and the tapering group were 17.3 (17.7) and 11.0 (16.0), respectively (p=0.059). At 2 years after the treatment, mean dose quotient (SD) of the tapering group was 0.59 (0.17) and time to first dose reduction (SD) was 36.6 (28.4) weeks. During the observation period, the rate of radiographic progression in the overall patients was 0.90 mSASSS unit/year. Rate of progression was significantly higher in patients with longer disease duration (≥10 years), elderly patients (≥40 years old), smokers, or patients with a hip involvement at baseline. Radiographic progression over 4 years between two treatment strategies was similar after the adjustment for baseline status. However, in the subgroup of patients who have syndesmophytes at baseline, it occurred significantly faster in the tapering group (1.30 mSASSS unit/year vs. 1.82 mSASSS unit/year, p=0.008) (figure). This result was concordant when radiographic progression was assessed by the development of new syndesmophytes (0.52/year vs. 0.73/year, p=0.047). <h3>Conclusions</h3> Dose tapering strategy of TNF-blockers is associated with more rapid radiographic progression in AS patients who have syndesmophytes at baseline. <h3>References</h3> Závada J, et al. A tailored approach to reduce dose of anti-TNF drugs may be equally effective, but substantially less costly than standard dosing in patients with ankylosing spondylitis over 1 year: a propensity score-matched cohort study. Ann Rheum Dis 2016;75:96–102. <h3>Disclosure of Interest</h3> None declared
Lack of IL-7 Receptor Alpha Chain (CD127) Expression In T Cells Is a Hallmark of T-Cell Immunodeficiency In Schimke Immuno-Osseous Dysplasia (SIOD)
Authors: Mrinmoy Sanyal, Kira Y Dionis, Alireza Baradaran-Heravi, Benjamin Dekel, Arend Bökenkamp, Cornelius F. Boerkoel, David B. Lewis
Journal: Blood (2010)
PTU-144 EUS in The Diagnosis and Staging of Lung Cancer: Single Centre Experience at Glasgow Royal Infirmary
Authors: C Harrington, S Paterson, A Stanley
Journal: Gut (2016)

<h3>Introduction</h3> Lung cancer is the leading cause of cancer related death in the Western World.<sup>1</sup> Accurate staging is required to identify those patients with localised Non Small Cell Lung Cancer (NSCLC) who have resectable disease. Patients with Small Cell Lung Cancer (SCLC) or T4,N2-3 or M1 NSCLC are not suitable for surgery. Endoscopic ultrasound (EUS) with fine needle aspiration (FNA) can help obtain a tissue diagnosis where Endobronchial Ultrasound (EBUS)-FNA or CT guided biopsy is not possible.<sup>2</sup> <h3>Methods</h3> We performed a retrospective search of prospectively recorded data on all patients who were referred for EUS-FNA between 2012 and 2015 for the following indications: Suspicious lung mass with or without associated lymphadenopathy. This group has been subdivided into those who underwent EUS-FNA to obtain a tissue diagnosis and those who already had a tissue diagnosis and the EUS-FNA was performed for tumour staging. Suspicious mediastinal or epigastric lymph nodes alone. This group has been subdivided into those for whom EUS-FNA was performed to investigate possible recurrence of previously resected lung cancer and those who had unexplained mediastinal or epigastric lymphadenopathy. Procedure: The EUS-FNA procedures were all undertaken by AJS or SP, with a 22 g FNA needle (Cook Ltd), using a standard linear echoendoscope with ultrasound (Pentax Ltd &amp; Hitachi Ltd). We collected data on patient demographics, EUS findings and procedures, cytology and prior pathology and radiology using our electronic clinical reporting system <h3>Results</h3> 35 patients were referred for EUS-FNA for the diagnosis or staging of lung cancer during the study period. 2 patients were unsuitable candidates. 25 patients had a suspicious lung mass and 8 had suspicious lymph nodes alone. In the group with a suspicious lung mass, EUS-FNA led to a diagnosis of lung cancer in 15 patients with 7 patients having benign pathology. 3 patients already had a diagnosis of lung cancer; EUS-FNA led to upgrading of tumour stage in 1 patient. In the group with suspicious lymph nodes alone, 3 diagnoses of lung cancer were made. Of the remaining 5 patients, 4 had benign pathology and 1 had gastric cancer. No procedural complications were encountered in any patient. EUS-FNA therefore led to a diagnosis of lung cancer in 60% (18/30) of the cases referred without a prior diagnosis. <h3>Conclusion</h3> EUS-FNA is a useful modality in the diagnosis and staging of lung cancer when EBUS and/or CT guided biopsy fail to obtain a tissue diagnosis. <h3>References</h3> 1 Ferlay J, Shin HR, Bray F, <i>et al</i>. Estimates of worldwide burden of cancer in 2008: GLOBOCAN 2008. <i>Int J Cancer</i>. <b>2010</b>;127:2893–917. 2 Colella S, Vilmann P, Konge L, <i>et al</i>. Endoscopic ultrasound in the diagnosis and staging of lung cancer. <i>Endoscopic Ultrasound</i>. 2014;<b>3</b>(4):205–212. <h3>Disclosure of Interest</h3> None Declared
Comparison Between First-Generation 4-Color Vs. Second-Generation 8-Color Multiparameter Flow Cytometry (MFC) to Monitor Minimal Residual Disease (MRD) in Multiple Myeloma (MM)
Authors: Paula Arana, Bruno Paiva, Maria Belen Vidriales, Juan A Flores-Montero, Noemi Puig, Teresa Cedena, Lourdes Cordon, Joaquin Martinez-López, Enrique M. Ocio, Miguel T Hernandez, Ana Isabel Teruel, Mercedes Gironella, M Asuncion Echeveste, Laura Rosiñol, Joan Blade, Juan Jose Lahuerta, Jacques J.M. van Dongen, Alberto Orfao, Maria-Victoria Mateos, Jesus San Miguel
Journal: Blood (2015)
Nilotinib Combined with Multi-Agent Chemotherapy for Adult Patients with Newly Diagnosed Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia: Interim Results of Korean Adult ALL Working Party Phase 2 Study
Authors: Dae-Young Kim, Young Don Joo, Je-Hwan Lee, Jung-Hee Lee, Dong Hwan Kim, Chul Won Jung, Inho Kim, Sung-Soo Yoon, Deok-Hwan Yang, Je-Jung Lee, Yang Soo Kim, Yeung-Chul Mun, Hawk Kim, Sang Kyun Sohn, Won Sik Lee, Jong-Ho Won, Myung Soo Hyun, Jae Hoon Lee, Hyun-Sook Chi, Kyoo-Hyung Lee
Journal: Blood (2011)
SAT0126 Comorbidities after Daily Concomitant Treatment with 10mg Prednisone or Placebo in The 2-Year Computer Assisted Management in Early Rheumatoid Arthritis Trial-Ii: Table 1.
Authors: M.J. De Hair, M. Safy, N. Ijff, J.W. Jacobs, J.M. van Laar
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> In the second Computer Assisted Management in Early Rheumatoid Arthritis (CAMERA–II) trial, patients initiated treatment with methotrexate (MTX) with randomized 10 mg/d prednisone (pred) or placebo (plac), given for 2 years [1]. Significantly more patients in the MTX+pred strategy arm than in the MTX+plac arm were erosion free at 2 years, and disease activity improved faster. The frequency of adverse events (AE) during the 2 year trial was comparable between the groups. There is paucity of data on long-term AEs after treatment with medium dose of glucocorticoids, however. <h3>Objectives</h3> To investigate the incidence of long-term AEs/comorbidities in early rheumatoid arthritis patients, who had been treated additionally with pred10 mg/day or plac in CAMERA-II. <h3>Methods</h3> After the 2 year trial, patients were followed according to a protocol in clinical care. The occurrence of AEs/comorbidities and the use of pred (it was the strategy to taper and stop it) was collected retrospectively from the medical charts. The incidence of long-term AEs/comorbidities in the former MTX+pred group was tested versus that in the former MTX+plac group using Fisher9s Exact tests. <h3>Results</h3> Of the 236 patients included in the CAMERA-II trial, follow-up data was available of 218 patients: MTX+pred N=107, MTX+plac N=111; no longer followed according to protocol N=18. The median follow-up time after the end of the 2-year trial period was 6 (range 0–9) and 6 (0–11) years, respectively. 86 (80%) patients treated with pred could discontinue it after a median of 9 months after the end of the trial end. The percentage of patients with AEs/comorbidities is shown in Table 1. No statistically significant differences between the two groups were found. <h3>Conclusions</h3> Of patients in the MTX+pred group during CAMERA-II, 80% could discontinue pred after the trial. There was no increased incidence of long-term AEs/comorbidities in the former MTX+pred group. <h3>References</h3> Bakker MF, Jacobs JWG, Welsing PMJ, et al. Low-dose prednisone inclusion in a methotrexate-based, tight control strategy for early rheumatoid arthritis: a randomized trial. Ann Intern Med 2012;156:329–39. <h3>Disclosure of Interest</h3> None declared