tbuckley/cabot-search huggingface dataset:
SAT0608 How to differentiate adult onset still’s disease from overall other causes of fever of unknown origin: results of a prospective study from a tertiary referral centre
Authors: E. Bilgin, A. Erden, B. Armagan, L. Kiliç, A. Sari, G. Yardimci, M.K. Hayran, A. Akdoğan, Ö. Karadağ, S. Apraş Bilgen, I. Ertenli, S. Kiraz, U. Kalyoncu
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Adult onset Still's disease (AOSD) is a rare, auto-inflammatory disease that commonly presents as fever of unknown origin (FUO), and most common rheumatologic cause of FUO. Clinical and/or laboratory parameters that can discriminate AOSD from other causes of FUO need to be clarified in current literature. <h3>Objectives</h3> To determine clinical and/or laboratory parameters that help to differentiate AOSD from other causes of FUO and demonstrating a clinician-friendly algorithm for this purpose. <h3>Methods</h3> Data from patients who admitted to Hacettepe University Hospitals, inpatients sections of department of internal medicine with the complaint of FUO, who eventually had a certain diagnosis, collected prospectively during 30 months. AOSD patients followed at Hacettepe University department of rheumatology were included. Clinical and laboratory data were collected at the time of diagnosis of AOSD and time of admission of patients with FUO. <h3>Results</h3> Total 156 patients (n=69, for AOSD; n=87, for FUO) were included. FUO group were also divided into three subgroups: rheumatologic (n=31, 35.6%), infectious (n=28, 32.2%) and malignant (n=28, 32.2%) causes. While 51 (74%) patients were female in AOSD group, 43 (49,4%) patients were female in FUO group (p=0.03). Frequency of rash, arthralgia, arthritis, sore throat, fever at night (p&lt;0.001 for each), history of hemophagocytosis (p=0.037) were significantly higher in AOSD group. Fever peak number equal and/or higher than 3, presence of lymphadenopathy (p=0002 and p=0,001,respectively) were significantly higher in FUO group. While leukocytosis, neutrophilia, thrombocytosis, hyperferritinemia, higher lactate dehydrogenase and complement 3 levels (p&lt;0.001 for each) were significantly more frequent in AOSD group, albumin levels lower than 3 g/dl and positive rheumatoid factor (p=0.009 and p=0.002,respectively) were significantly more frequent in FUO group. Results of univariate and multivariate analysis are given in table 1. Algorithm for discrimination of AOSD and FUO is given at Abstract SAT0608 – figure 1. <h3>Conclusions</h3> Presence of arthralgia, hyperferritinemia, sore throat and neutrophilia strongly favour AOSD in patients presenting as FUO. This study demonstrates a clinician-friendly algorithm for the first time in current literature. <h3>Disclosure of Interest</h3> None declared
Abstract 4936: Endovascular Perfusion of Pluripotent Mesenchymal Stem Cells Stabilizes Already-formed Aortic Aneurysms in a Rat Model
Authors: Fabrice Schneider, Jian Ping Dai, Marianne Taurel, Pascal Desgranges, Jean-Pierre Becquemin, Éric Allaire
Journal: Circulation (2009)

Introduction: Endovascular cell therapy using catheter-based delivery of Vascular Smooth Muscle Cells (VSMCs) has been shown to stabilize diameter of expanding Abdominal Aortic Aneurysms (AAAs) in rat. Mesenchymal Stem Cells (MSCs) are able to differentiate into VSMCs after stimulation by Platelet Derived Growth Factor-BB (PDGF-BB). Our goal was to assess the impact of MSCs endovascular perfusion with or without pre-differentiation by PDGF-BB stimulation on aneurysm growth. Materials and methods: After selection by plastic adhesion, bone marrow derived-MSCs were stimulated or not by PDGF-BB and phenotyped by immunohistochemistry. AAAs obtained by xenograft degeneration were perfused endovascularly with 1 million of VSMCs (n=5), MSCs (n=6), PDGF-MSCs (n=6) and medium culture as controls (n=4). Rats were euthanized 1 week later or 4 weeks later (non differentiated MSCs (n=6) and culture medium (n=5)) for diameter measurement and AAA harvesting for mRNA (MMP-9, TIMP-1) quantification. Results: MSCs were CD90 + , CD44 + , CD45 − and CD11 b − and after PDGF stimulation strongly expressed calponin, α -actine and myosin. At 1 week, MSC infusion decreased aneurysm growth compared to SMCs and control group (% diameter increase: 6.5%+/−9.7, 25.5%+/−17.2 and 53.4%+/−14.4 respectively; p&lt;0,05) whereas PDGF-MSCs increased aortic growth rate (55.6%+/−26.6; p&lt;0,05) and induced rupture (n=3/6 AAAs). Perfusion of MSC decreased expression of MMP-9 and increased TIMP1 expression compared to controls. At 4 weeks, MSC signicantly decreased aneurysm growth compared to control group (9.2%+/−13.8 and 76.2%+/−17.4, respectively; p&lt;0.05) Conclusion: Pluripotent MSCs stabilized growth of expanding AAAs more efficiently than VSMCs and decreased proteolytic activity in the aortic wall. Predifferentiation suppressed the stabilization properties of MSCs, suggesting the importance of pluripotency of progenitors in aneurysm repair.
AB0576 Features of neurological manifestations of systemic lupus erythematosus in the kyrgyz cohort of patiens
Authors: G. Koilubaeva, E. Aseeva, S. Soloviev, T. Reshetnyak
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> The development of a variety of neuropsychiatric symptoms in SLE patients starts with the dysregulation of the immune system. Involvement of the CNS in the pathological process during the onset of the disease can significantly worsen the patient's condition and complicate the prognosis. <h3>Objectives</h3> To study the features of neurological manifestations of SLE in the Kyrgyz cohort of patients. <h3>Methods</h3> The basis of the study were the results of an initial examination of 460 patients with prospective observation, with a reliable diagnosis of SLE. Out of them, 112 patients were neurological, at the age of 30 (median) [26, 41], predominantly of Kyrgyz nationality (87), with duration of the disease from 6 months to 7 years (median – 2.0) treated in the National Cardiology and Therapy Centre named after M. Mirrakhimov (NCTC) from January 2012 to December 2017. Neurologic manifestations of SLE were diagnosed by clinical examination by a neurologist using some instrumental methods of investigation. To assess the neuropsychiatric state of patients, the classification criteria by the American College of Rheumatology (1999) were used. Neuropsychiatric disorders were diagnosed through a clinical examination by the psychotherapist using the classification of mental disorders and behavioural disorders according to ICD (International Classification of Diseases)−10. <h3>Results</h3> The damage of the nervous system was observed in 112 (24,305%) Kyrgyz patients from 460, mainly of the central nervous system – in 77 (68.75%), to a lesser extent – the peripheral nervous system (PNS) – in 35 (31.25%) out of 112. In half of the observed patients with CNS lesions, various neuropsychiatric disorders were registered – in 39 (50.65%) out of 77, with psychosis prevalence with visual and auditory hallucinatory syndrome – in 34 (87.18%) out of 39 patients. There were 14 patients with cerebrovascular infection (18.18%) out of 77, mainly with ischaemic stroke in the SMA basin. There was one patient with aseptic meningoencephalitis with manifestations of mild hemiparesis and with the disruption of the function of the pelvic organs in the form of mandatory urges. Symptoms of myelopathy with malfunction of the pelvic organs were registered in 9 (11.69%) patients out of 77. Five patients had symptoms of combined CNS and PNS lesions. In one of them cases the patient had encephalopolyneuroradiculopathy of the Guillain-Barre type with the phenomena of coarse tetraparesis and with disruption of the function of pelvic organs in the form of delay, with a single epicprism. <h3>Conclusions</h3> Neurological manifestations of SLE in the majority of Kyrgyz patients were mainly caused by CNS lesions (68.75%), mostly in the form of neuropsychiatric disorders (50.65%), with a predominance of psychosis with visual and auditory hallucinatory syndrome (87.18%). <h3>Disclosure of Interest</h3> None declared
THU0255 Low bone mineral density is common in axial spondyloarthropathy
Authors: G. Fitzgerald, T. Anachebe, F. O’ Shea
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Osteoporosis is a known consequence of inflammatory arthritis (IA). In the general population and IA such as rheumatoid arthritis, the impact of osteoporosis is well outlined. However, it is often ignored in axial spondyloarthropathy (axSpA), a form of IA centred on sacroiliac joints and the spine, as axSpA predominantly affects men, in whom osteoporosis is often not considered. As a result, osteoporosis prevalence figures are unclear, with wide variation in the literature. Accurate epidemiology regarding bone mineral density (BMD) in axSpA is crucial to begin understanding the impact of low BMD in this cohort. <h3>Objectives</h3> 1. Investigate the prevalence of low BMD in a well-characterised axSpA cohort 2. Explore relationships (demographic, disease-related, laboratory) between BMD and axSpA. <h3>Methods</h3> A detailed assessment was performed on axSpA patients, including demographics, clinical characteristics and laboratory investigations. Disease severity was assessed with tools validated in axSpA: ASDAS-CRP and BASDAI (disease activity), BASMI (spinal mobility) and BASFI (function). BMD was assessed using DXA of the spine, hip and radius. Lateral vertebral assessment (LVA) was also performed. The WHO criteria were used to classify low BMD. SPSS was used for statistical analysis. <h3>Results</h3> One hundred and four patients with axSpA were consecutively recruited: 77.9% (n=81) male, 98.1% (n=102) Caucasian, mean (SD) age 51<sup>12</sup> years, disease duration 26<sup>13</sup> years. The mean (SD) ASDAS-CRP was 2.3 (1), BASDAI was 3.9 (2.2), BASMI was 4.3 (1.9) and BASFI was 3.8 (2.5), reflecting mild to moderate disease burden. A history of fracture was present in 42.3% (n=44) of the cohort, with only 3 fragility fractures reported. Of the cohort, 42.3% (n=44) had osteopenia and 16.3% (n=17) had osteoporosis. Low BMD was most prevalent at the spine, with 44% of the cohort affected, followed by the femoral neck (30.1%, n=22). Low BMD at the radius was uncommon (&lt;10% of the cohort). Only 6.4% of the cohort had a prior diagnosis of osteoporosis and only 39.4% had a previous DXA. Three vertebral fractures were detected on LVA – all patients were unaware of these fractures prior to the study. Female gender, higher BASFI, lower BMI and lower urate levels were significantly associated with bone loss at both the spine and the hip. ASDAS-CRP and BASDAI had no impact on low BMD. Additionally, longer disease duration was associated with spine BMD loss. Non-obese patients were more likely to have low BMD at any site than obese patients (62.3% v 40%, OR 2.5, p=0.04). The use of biologics didn't influence BMD. <h3>Conclusions</h3> Low BMD is common in this axSpA cohort, with over 50% of patients affected. Most cases of low BMD were undiagnosed prior to this study and less than half of the cohort had ever had a DXA, suggesting a continued low awareness of the risk of osteoporosis in a male-dominated disease. <h3>Disclosure of Interest</h3> None declared
SAT0487 A cohort study of macrovascular involvement in patients with systemic sclerosis
Authors: C. Caimmi, S. De Marchi, G. Astorino, E. Bertoldo, P. Caramaschi, M. Rossini
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> It is well known that systemic sclerosis (SSc) affects microvessels, but data on macrovascular involvement are still lacking or debatable. <h3>Objectives</h3> Aim of this study was to estimate the prevalence of atherosclerotic plaques and their possible determinants in a large cohort of SSc patients. <h3>Methods</h3> One-hundred and four outpatients with SSc were enrolled. Data about disease characteristics and cardiovascular risk factors (diabetes, hypertension, dyslipidemia, smoke) were collected and patients underwent a full ecocolorDoppler ultrasonography of arteries of the neck and lower limbs (LL). <h3>Results</h3> Mean ageof our cohort was 62±13 years and 17 (16.3%) patients were male. Seventy patients (67.3%) had a limited subset and the mean disease duration was 12.7±6.8 years. A previous history of digital ulcers was found in 27 cases (26.0%). Thirty-eight patients (36.5%) were on ongoing or previous treatment with immunosuppressive drugs. Regarding cardiovascular risk factors, 22 (21.2%) had hypertension, 7 (6.7%) diabetes, 52 (50%) dyslipidemia and 45 (43.3%) were active or past smokers. Fifty-seven (54.2%) patients had plaques at carotids, 1 (1%) at LL and 37 (35.9%) at UL. Prevalences were higher in older patients, as expected. Patients with carotid plaques were older (p&lt;0.001), with hypertension (p=0.057), a limited disease subset (p=0.005), a more severe disease accordingly to Medsger severity score (0.048), worse renal function (p=0.012), higher glucose blood levels (p≤0.001), homocysteinemia (p=0.006) and ESR (p=0.004) and less often on immunosuppresors (p=0.048) but more often on steroids (p=0.050). Patients with LL plaques were older (p&lt;0.001), male (p=0.003), treated with statins (p=0.056), with a worse renal function (p=0.001), higher glucose blood levels (p=0.038) and homocysteinemia (p=0.006). In multivariate analysis with all variables with a p&lt;0.010 in univariate, patients with carotid plaques were found to be older (p=0.003) and with a limited disease subset (p=0.012; OR 5.6, CI95% 1.5 to 21.5). These data were confirmed even after correcting also for other well-known risk factors for atherosclerosis. No variables were found to be statistically different between patients with and without LL plaques. <h3>Conclusions</h3> in this study we performed one of the most complete evaluation of macrovascular involvement in one of the most numerous cohort of SSc patients present in literature. The prevalence of carotid and LL plaques did not seem to be higher in SSc patients as compared to what reported in healthy subjects. Intriguing is that patients with limited disease have an increased risk of having carotid plaques even after correcting for possible confounders. <h3>Disclosure of Interest</h3> None declared
Erratum to “A new HDV mouse model identifies mitochondrial antiviral signaling protein (MAVS) as a key player in IFN-β induction” [J Hepatol 67 (2017) 669–679]
Authors: Lester Suárez-Amarán, Carla Usai, Marianna Di Scala, Cristina Godoy, Yi Ni, Mirja Hommel, Laura Palomo, Víctor Segura, Cristina Olagüe, Africa Vales, Alicia Ruiz-Ripa, Maria Buti, Eduardo Salido, Jesús Prieto, Stephan Urban, Francisco Rodríguez-Frias, Rafael Aldabe, Gloria González-Aseguinolaza
Journal: Journal of Hepatology (2018)
AB1314 Lack of association between abo blood groups and rheumatic diseases in china: a single-centreexperience
Authors: J. Qi, Q. lv, Z. chen, X. Zhang, M. Yang, Y. Zhang, J. Gu
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Various genetic and environmental risk factors have been shown to be associated with the incidence of rheumatic diseases. Several studies have shown associations of ABO blood groups with various diseases. <h3>Objectives</h3> Our study aimed to determine whether there is an association between the types of rheumatic diseases and ABO blood groups. <h3>Methods</h3> The study included the patients, followed up at the Immunology-Rheumatology clinic between <sup>January 2010</sup> and December 2017 for diagnosis of rheumatic disease, who had an ABO Rh blood data. Age, gender, type of rheumatic disease, ABO Rh blood groups were recorded. <h3>Results</h3> A total of 1551 patients were included in the study, such as 392 AS patients, 297 RA patients, 752 SLE patients and 110 pSS. The patients were assessed for blood types, 40.2% had O type, 26% patients had A type, 26.8% had B type, and 7.0% had AB type. AS, RA, SLE and pSS were more common in the patients with O blood type. The blood type where all the diseases are observed the least commonly was AB. There was no significant difference in the distribution of blood types in rheumatic diseases. <h3>Conclusions</h3> In our study, we thought that there was lack association between types of different rheumatic diseases and ABO blood groups. <h3>Reference</h3> [1] Cildag S, Kara Y, Senturk T. ABO blood groups and rheumatic diseases. European journal of rheumatology20174;(4):250–253. <h3>Acknowledgements</h3> no <h3>Disclosure of Interest</h3> None declared
Reference equations for pulmonary diffusing capacity of carbon monoxide and nitric oxide in adult Caucasians
Authors: Mathias Munkholm, Jacob Louis Marott, Lars Bjerre-Kristensen, Flemming Madsen, Ole Find Pedersen, Peter Lange, Børge G. Nordestgaard, Jann Mortensen
Journal: European Respiratory Journal (2018)

The aim of this study was to determine reference equations for the combined measurement of diffusing capacity of the lung for carbon monoxide (CO) and nitric oxide (NO) ( D LCONO ). In addition, we wanted to appeal for consensus regarding methodology of the measurement including calculation of diffusing capacity of the alveolo-capillary membrane ( D m ) and pulmonary capillary volume ( V c ). D LCONO was measured in 282 healthy individuals aged 18–97 years using the single-breath technique and a breath-hold time of 5 s (true apnoea period). The following values were used: 1) specific conductance of nitric oxide (θ NO )=4.5 mL NO ·mL blood −1 ·min −1 ·mmHg −1 ; 2) ratio of diffusing capacity of the membrane for NO and CO ( D mNO / D mCO )=1.97; and 3) 1/red cell CO conductance (1/θ CO )=(1.30+0.0041·mean capillary oxygen pressure)·(14.6/Hb concentration in g·dL −1 ). Reference equations were established for the outcomes of D LCONO , including D LCO and D LNO and the calculated values D m and V c . Independent variables were age, sex, height and age squared. By providing new reference equations and by appealing for consensus regarding the methodology, we hope to provide a basis for future studies and clinical use of this novel and interesting method.
Congenital macrothrombocytopenia with focal myelofibrosis due to mutations in human G6b-B is rescued in humanized mice
Authors: Inga Hofmann, Mitchell J. Geer, Timo Vögtle, Andrew Crispin, Dean R. Campagna, Alastair Barr, Monica L. Calicchio, Silke Heising, Johanna P. van Geffen, Marijke J. E. Kuijpers, Johan W. M. Heemskerk, Johannes A. Eble, Klaus Schmitz-Abe, Esther A. Obeng, Michael Douglas, Kathleen Freson, Corinne Pondarré, Rémi Favier, Gavin E. Jarvis, Kyriacos Markianos, Ernest Turro, Willem H. Ouwehand, Alexandra Mazharian, Mark D. Fleming, Yotis A. Senis
Journal: Blood (2018)
Abstract 14699: Akt1-mediated Skeletal Muscle Growth Induces Exosome-mediated Secretion of Cardio-protective microRNAs
Authors: Yasuhiro Izumiya, Yoshiro Onoue, Yuichi Kimura, Toshifumi Ishida, Satoru Yamamura, Shinsuke Hanatani, Satoshi Araki, Seiji Hokimoto
Journal: Circulation (2016)

Introduction:  Muscle wasting is frequently observed in patients with heart failure, and is an independent risk factor in these patients. Resistance exercise exerts beneficial effects for these patients, however the mechanisms are largely unknown. It has been proposed that skeletal muscle secretes factors that influence the behavior of remote cells. Recent evidence indicates that circulating miRNAs are involved in cardiovascular diseases.   Hypothesis:  We assessed the hypothesis that cardio-protective miRNAs are secreted from growing skeletal muscle.   Methods:  To identify muscle-derived miRNAs, we utilized skeletal muscle-specific, conditional transgenic mice, that can induce the growth of functional muscle by switching Akt1 signaling on in muscle fibers (Akt1 TG mice). Two weeks after Akt1 activation in skeletal muscle, we isolated miRNA from gastrocnemius muscle in Akt1 TG and control mice (n=4 in each group). We also isolated exosomal miRNA from serum in each mouse. The miRNA expression was comprehensively analyzed by miRNA PCR array. Adenovirus-mediated Akt1 overexpression experiments were performed in L6 myotubes. Secreted miRNA in culture media was evaluated by quantitative real-time PCR.   Results:  Activation of Akt1 signaling in myofibers led to an increase in skeletal muscle mass, assessed by gastrocnemius muscle weight at 14 days after transgene induction (171.5 vs. 241.3 mg; p   Conclusions:  Akt1-mediated skeletal muscle growth induces exosome-mediated secretion of miRNAs that might be involved in intra-tissue communication between skeletal muscle and cardiovascular tissue. Stimulators of Akt1 signaling in skeletal muscle, such as resistance training, could attenuate cardiovascular diseases through secretion of muscle-derived cardio-protective miRNAs.
Analysis of Fecal Primary Bile Acids Detects Increased Stool Weight and Colonic Transit in Patients With Chronic Functional Diarrhea
Authors: Priya Vijayvargiya, Michael Camilleri, Victor Chedid, Paula Carlson, Irene Busciglio, Duane Burton, Leslie J. Donato
Journal: Clinical Gastroenterology and Hepatology (2018)
AB0372 Left atrial function in rheumatoid arthritis patients
Authors: M. Fawzy, S. bahoom, None Ashour, M. sheik
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Rheumatoid arthritis (RA) is a common autoimmune systemic inflammatory disease affecting approximately 1% of the worldwide population. The interaction of genetic and environmental factors results in a cascade of immune reactions, which ultimately lead to the development of synovitis, joint damage, and structural bone damage.<sup>1</sup> The importance of the left atrium in cardiovascular performance has long been acknowledged. Quantitative assessment of left atrial (LA) function is laborious, requiring invasive pressure-volume loops and thus precluding its routine clinical use. In recent years, novel postprocessing imaging methodologies have emerged, providing a complementary approach for the assessment of the left atrium. Atrial strain and strain rate obtained using either Doppler tissue imaging or two-dimensional speckle-tracking echocardiography have proved to be feasible and reproducible techniques to evaluate LA mechanics.<sup>2</sup> <h3>Objectives</h3> To screen cardiac affection in rheumatoid arthritis patients To assess subclinical echocardiography affection in RA patients <h3>Methods</h3> 30 healthy control, and 45 RA patient subjected to full clinical assessment, DAS 28 ESR score, full laboratory evaluation, conventional and tissue Doppler imaging (TDI) and strain (S) and strain rate (SR) analysis by two-dimensional speckle tracking of the left atrium. <h3>Results</h3> we found statistically significant difference in 2 Left atrial PEF, 2 Left atrial EI, 2 Left atrial TEF, TDI mitral lateral anuulus e', TDI mitral lateral annulus S, Average SR E 1/s between patients and controls, and negative correlation between TDI lateral e, TDI lateral s, and Strain rate e and rheumatoid factor, There was negative correlation between 2LA PEF, 2LA EI, and 2LA TEF. Rheumatoid factor is correlated to strain rate e, and negatively correlated with left atrial passive emptying <h3>Conclusions</h3> RA had alteration in left LV longitudinal myocardial function, left atrial expansion volume can be a predictor of AF in RA. RA patient had more left atrial stiffness. Our study concluded cardiac affection is more in seropositive RA patients. <h3>References</h3> [1] Gibofsky A. Overview of epidemiology, pathophysiology, and diagnosis of rheumatoid arthritis. Am J Manag Care2012;18(13):S295–302. [2] Maria J Vieira, Rogério Teixeira, LinoGonçalves, et al. Left Atrial Mechanics: Echocardiographic Assessment and Clinical Implications2014;27(5):463–478. <h3>Disclosure of Interest</h3> None declared