tbuckley/cabot-search huggingface dataset:
AB0615 Persistence of human papillomavirus in the cervix of women with systemic sclerosis
Authors: C Vacchi, M Colaci, G Cassone, C Esposito, F Lumetti, D Giuggioli, C Ferri
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Persistent infection by high-risk oncogenic Human Papillomavirus (HPV) is the main cause of the development of dysplastic or malignant lesions of the cervix. Furthermore, a few life habits, such as smoking, sexual habits and hormonal contraception, are known risk factors for cervical HPV infection. In addition, increased frequency of persistent HPV infection or high-grade intra-epithelial lesions rates was anecdotally described in patients affected by immune-mediated diseases, such as systemic lupus erythematosus, rheumatoid arthritis and systemic sclerosis (SSc), in comparison with the general population. <h3>Objectives</h3> To determine the prevalence of persistent HPV infection in an SSc patients series and its possible correlation with the disease clinical features. <h3>Methods</h3> The study retrospectively evaluated 52 consecutive female SSc patients (age 56.7±11.2SD years, disease duration 12.1±7.4SD years), classified according to the ACR/EULAR 2013 criteria, referring to our university-based Rheumatology Unit. Detection of HPV DNA and viral genotyping in cervical swabs were carried out. Moreover, abnormal Papanicolau test smears were classified using the Bethesda system. <h3>Results</h3> Fourteen(26.9%) patients presented a cervical swab positive for HPV infection,including12 infected by high risk or probable-high risk HPV types. Six (11.5%) patients presented multiple infection (≥2 HPV types), including one case with high-grade intra-epithelial lesion. Only tabagism was significantly correlated to HPV infection; namely,smoking habit was observed in 41.6% of SSc patients with and in 21.7% of those without HPV infection, respectively; p=0.006); moreoverimmunesuppressive therapies, namely mofetil mycophenolate, cyclophosphamide or rituximab, tended to be associated withHPV infection (presence/absence 21.4 vs 21.7%; p=0.055). More interestingly, amongSSc patients over 50, HPV infection was found in 9/38 (23.7%) individuals, a frequency markedly higher than that expected in age-matched general population from the same geographical area (5%). <h3>Conclusions</h3> Persistent HPV infection was observed in over a quarter of SSc patients, notably in women over 50. The HPV positivity was not related to SSc clinical features, while a significant association with tabagism and immunesuppressive therapies was evidenced. Considering the possible clinico-prognostic implication on the overall disease outcome, routinely gynaecological screening of SSc female patients ishighly recommendable. <h3>Disclosure of Interest</h3> None declared
THU0484 Ro60 expression decreases with age in peripheral blood mononuclear cells of children and adolescents and correlates with tlr7 stimulation in pdcs of prepubertal children
Authors: A Radziszewska, K Webb, H Peckham, Y Ioannou
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Auto-antibodies to the RNA binding protein Ro60 are present in patients with autoimmune disorders such as systemic lupus erythematosus (SLE). In addition to its established role as an auto-antigen, Ro60 has been found to bind Alu RNA retroelements whereby it may target Alu retroelement RNA for degradation suggesting a novel putative function of this auto-antigen. If Ro60 modulates the amount of cellular RNA then one may hypothesise an association with toll-like receptor (TLR) 7 stimulation threshold. <h3>Objectives</h3> To measure the physiological levels of intracellular Ro60 protein in healthy children and adolescents and investigate a possible link between Ro60 protein expression and interferon-α (IFN-α) production after TLR7 stimulation. <h3>Methods</h3> Peripheral blood mononuclear cells (PBMCs) were isolated from blood from 48 healthy children and adolescents (age range 6.7–17.9 years old). Cell lysates from thawed PBMC samples were tested for Ro60 expression by Western blot. PBMCs were also stimulated for 20 hours with TLR7/8 agonist R848, at 1ug/ml in the presence of brefeldin A, and plasmacytoid dendritic cell (pDC) IFN-α expression was measured using flow cytometry. Statistical tests to measure correlation between IFN-α expression in (pDCs) and PBMC Ro60 expression were performed using SPSS. <h3>Results</h3> Ro60 expression in PBMCs correlated negatively with age (Spearman9s rho = -0.317, p=0.032). When participants were divided into two groups based on their self-reported puberty status, Ro60 expression was higher in the pre-pubertal group (p=0.02). There was, however, no difference in Ro60 expression between males and females (p=0.44) or when sexes were stratified according to pubertal status. pDC IFN-α production after TLR 7 stimulation, did not correlate with ex vivo PBMC Ro60 expression overall (Spearman9s rho =0.159, p=0.302) however a moderate positive correlation was observed in pre-pubertal samples only (Spearman9s rho =0.554, p=0.021). <h3>Conclusions</h3> Ro60 expression decreases with age in healthy young people. These findings need to be confirmed in a larger cohort and further studies are necessary to investigate the link between Ro60 expression and TLR7 signalling across different age groups as well as in patients with SLE. <h3>Disclosure of Interest</h3> None declared
NLRP3 inflammasome driven liver injury and fibrosis: Roles of IL‐17 and TNF in mice
Authors: Alexander Wree, Matthew D. McGeough, Maria Eugenia Inzaugarat, Akiko Eguchi, Susanne Schuster, Casey D. Johnson, Carla A. Peña, Lukas J. Geisler, Bettina G. Papouchado, Hal M. Hoffman, Ariel E. Feldstein
Journal: Hepatology (2017)

The NLRP3 inflammasome, a caspase-1 activation platform, plays a key role in the modulation of liver inflammation and fibrosis. Here, we tested the hypothesis that interleukin 17 (IL-17) and tumor necrosis factor (TNF) are key cytokines involved in amplifying and perpetuating the liver damage and fibrosis resulting from NLRP3 activation. To address this hypothesis, gain-of-function Nlrp3
AB0649 Systemic sclerosis in argentina: evaluation of a single center cohort and comparison with international series
Authors: M Garcia Carrasco, LO Roa Prerez, R Gomez, S Leal, F Paniego, N Perez, GG Nasswetter, D Diana
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Systemic sclerosis (SS) is an autoimmune disease with generalized vascular dysfunction and a myriad of clinical and laboratory manifestations. Spreading of thickening of the skin, serological characteristics and the pattern of compromise of the internal organs help to classify them as diffuse scleroderma, localized sclerosis and systemic sinus scleroderma. Reports of Latin American cohorts published to date are scarce. <h3>Objectives</h3> To describe the clinical and serological data for a single center cohort and to compare those with national and international cohorts. <h3>Methods</h3> Descriptive, observational, cross-sectional study. We analyzed our SS database. Patients were evaluated since 01/15 to 05/2016 and fulfilled SS classification criteria (ACR 1980/ACR-EULAR 2013).Patients were classified according to Le Roy criteria in Limited Systemic Sclerosis (lSS), Diffuse Systemic Sclerosis (dSS), Systemic Sclerosis sine scleroderma (SsnS) and pre-systemic sclerosis (pSS). Organic compromise was defined as follows: Gastrointestinal (GI): esophagic dismotility by manometry or esophagitis by endoscopy; Lung: Pulmonary hypertension (PH): PSAP&gt;25 mmHg by right heart catheterization; Intersticial Lung Disease (ILD): compatible HRCT or &lt;70% predicted FVC or&lt;80% predicted DLCO; Heart: Left ventricle dysfunction without PH or arterial hypertension, pericardial effusion; Digital ulcers and pitting scars; History of sclerodermic renal crisis (RSC); calcinosis. Anti-Scl-70 was determined by ELISA and anti-Centromere (ACA) by IIF. <h3>Results</h3> 123 patients were included.74% with lSS,24% dSS and 2% SsnS; age at diagnosis (years): 49.7 (18–79 DS: 12.27) and 48.7 (27–79 DS: 13.01) in lSS and dSS respectively. Raynaud9s phenomenon previous to diagnosis (years) 7,7 (0–54 DS: 12.81) in lSS and 4.03 (0–22 DS: 5.5) in dSS. 49.5% lSS were ACA positive vs 17% in dSS as well also 41,4% had Scl-70 positive (p&lt;0.05). Lung:58.6% of dSS presented ILD and 3,4% RSC, according to published reports. No statistically significant differences were found about the presence of calcinosis, digital tip ulcers or PH between dSS and lSS. GI: was studied in only 39/123 patients with 84,6% affected among those. <h3>Conclusions</h3> Our data showed similar results to the local and european cohorts. We found fewer calcinosis and digital tip ulcers in both lSS and dSS than international cohorts. A lower prevalence of PH was reported in lSS respect to other series, and it might be atributed to the method wich it was measured. (right heart catheterism). <h3>Disclosure of Interest</h3> None declared
AB0389 The immunogenicity of branded and biosimilar infliximab in rheumatoid arthritis according to th9-related responses
Authors: R Talotta, A Berzi, A Doria, A Batticciotto, MC Ditto, D Trabattoni, P Sarzi Puttini
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> There are many evidences that Th9 lymphocytes take part to the pathogenesis of rheumatoid arthritis (RA), however it is unclear whether these cells are implicated in the immunogenicity of biologic agents. <h3>Objectives</h3> We aimed to evaluate the immunogenicity of branded and biosimilar infliximab by detecting changes in Th9 percentages following an <i>"in vitro"</i> stimulation test. <h3>Methods</h3> PBMCs from 55 consecutive RA outpatients (15 drug-free, 20 successfully treated with branded infliximab and 20 failing branded infliximab) and from 10 healthy controls were collected. PBMCs were cultured with/without 50 μg/mL infliximab originator (Remicade®) or 50 μg/mL infliximab biosimilar (Remsima®), 50 μg/mL Human IgG1kappa and 50 μg/mL recombinant Human IgG Fc for 18 hours. Th9 lymphocytes were identified by means of flow cytometry as PU.1+, IRF4+, IL9+ CD4+ cells. Furthermore, the markers CCR7 and CD45RA were used to distinguish naive from memory IL-9-producer cells. <h3>Results</h3> In unstimulated condition, RA patients showed the highest percentages of Th9 lymphocytes. Following stimulation with branded infliximab, the percentage of PU.1+, IRF4+ Th9 cells, CCR7+, CD45RA- central memory and CCR7-, CD45RA- effector memory IL-9 producer cells significantly increased in the group of infliximab non responder RA patients. On the contrary, no significant variation was observed after biosimilar exposure. Figures 1,2,3,4 resume the results. <h3>Conclusions</h3> According to our results, Th9 cells seem to be involved in the immune response against the epitopes of branded but not biosimilar infliximab and this condition could rely on the recall and the stimulation of both central and effector memory cells. Further studies are indeed needed to confirm these data. <h3>Disclosure of Interest</h3> None declared
AB1016 ANTI-DFS70, a tool in usual clinical practice: a case series
Authors: D Ybáñez-García, E Valls-Pascual, C Vergara-Dangond, M Aguilar-Zamora, L Montolio-Chiva, Ά Martínez-Ferrer, JM Lόpez-Ortega, JJ Alegre-Sancho
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> The presence of anti-nuclear antibodies (ANA) has been considered a characteristic of systemic autoimmune diseases (SAD). Patients are frequently referred for study because they have ANA and are followed because of the possibility to develop SAD. Approximately, 20% of healthy individuals with ANA detected by indirect immunofluorescence (IFI), especially at low titers, have a dense, fine speckled pattern (DFS) that frequently corresponds to the presence of anti-DFS70 antibodies. The importance of this antibody is due to its low prevalence in subjects with ASD (&lt;1%) compared to its presence in 33.1% of healthy subjects with ANA. <h3>Objectives</h3> To describe the usefulness of Anti-DFS70 in a series of patients presenting ANA. <h3>Methods</h3> We collected prospectively throughout the year 2016 all the patients referred to a tertiary hospital for ANA study and in whom the presence of anti-DFS70 antibodies was confirmed. All patients underwent a thorough medical history, physical examination, and relevant follow-up tests were performed according to the clinical presentation. The IFI was performed in a Menarini Zenit-Up/GSight system, as well as ANA screening in Hep-2000 (Fluorescent IgG ANA-Ro Test System-immunoconcepts) and the detection of anti-DFS70 antibodies by immunoblot (ANA + DFS70 Dot Blot-Alphadia). <h3>Results</h3> We collected in a period of 12 months a total of 7 patients with anti-DFS70 antibodies. Most of them (6/7) were referred because of non-specific symptoms such as arthralgia, fatigue, thrush, edema, ... and the presence of ANA. The findings are detailed in Table 1. <h3>Conclusions</h3> Anti-DFS70 is a valuable biomarker, with a very low prevalence in SAD, which gives it a role as a negative predictive marker of developing SAD when it is appears alone. Its detection in serum with a dense fine speckled pattern ANA (IFI) should be part of the protocol of the immunology laboratory. It is a cost-effective determination, as demonstrated in a recent study, by avoiding the costs associated with the follow-up of these patients. In our case, its finding allowed us to reassure the patient and avoid the accomplishment of further complementary tests, as well as an unnecessary monitoring. <h3>Disclosure of Interest</h3> None declared
SP0096 EULAR/PRES standardised procedures for musculoskeletal ultrasound (MSUS) imaging in pediatric rheumatology
Authors: P Collado
Journal: Annals of the Rheumatic Diseases (2017)

Musculoskeletal ultrasound (MSUS) is a readily available and suitable imaging technique to asses the immature skeleton of paediatrics in different musculoskeletal rheumatic diseases (MSRD). Besides the potential operator –dependent feature of this technique, the age-related variation of normal sonoanatomy due to the child9s growth makes more difficult acquisition, interpretation and comparison of images than in adults. Hence, to use MSUS as a valid technique for diagnosis of MSRD, it is needed to develop specifically evidence-based recommendations for paediatrics. Additionally, the variability in background and experience of ultrasonographers in different countries requires an international multidisciplinary contribution for an optimal standardization of MSUS performance in paediatric MSRD. Reflecting the perceived need for developing recommendations on the standardization of procedures for performing MSUS examination in MSRD, a collaborative international EULAR/PReS task force was convened. In the talk, it is going to summarize the original proposed specific aims and update on work for each aim previously mentioned. A systematic literature search was performed in Medline and Embase from databases inception to 1st June 2016 as the first step. One hundred and eighty-eight articles were finally included after reviewing 6059 records identified. The scanning for the knee and the ankle joint was the most common reported, whereas the paediatric wrist was uncommon. <h3>Disclosure of Interest</h3> None declared
FRI0342 Distribution of vasculitides in eastern mediterranean: results of a prospective cohort
Authors: O Karadag, A Erden, ED Batu, E Bilgin, AO Uyaroglu, E Seyhoglu, EC Bolek, E Firat, HE Sonmez, SZ Arici, L Kilic, A Akdogan, SA Bilgen, S Kiraz, I Ertenli, S Ozen
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> The ethnic and geographical differences have been commented on, mainly from European data. We lack large data sets from the eastern part of Europe. <h3>Objectives</h3> This study is aimed to present the distribution of vasculitides in the Pediatric and Adult Vasculitis patient groups. <h3>Methods</h3> Hacettepe University is one of the main referral centers in the capital of Turkey, serving approximately 470,000 adult and pediatric patients/a year. Hacettepe University Vasculitis Centre (HUVAC) was established to organize a prospective cohort in 2014. All patients starting from October 2014 have been recorded to the database. Additionally electronic patient recording database between October 2014-December 2016 was searched for the patients having any of the 10th revision of the International Statistical Classification of Diseases (ICD)-10 code for the particular vasculitis. The study group of Hacettepe University Vasculitis Centre (HUVAC) re-evaluated the ascertained patients9 hospital records to review their diagnosis according to the 2012 revised Chapel Hill nomenclature criteria. <h3>Results</h3> A total of 1196 patients had been newly followed in this period. 271 (22.7%) of them were pediatric patients and 31.8% of them were newly diagnosed. The leading vasculitis among adult patients was Behcet9s Disease whereas in pediatric patients it was HSP/IgA Vasculitis (Table). Granulomatous polyangiitis was the most common small vessel vasculitis in adults. Takayasu arteritis was more frequent than giant cell arteritis among the adult patients. There was a female predominance in patients with large vessel vasculitis. During prospective follow up, 22 (1.9%) patients deceased; 7 due to the primary disease, 6 due to infections, 7 due to cardio and cerebrovascular diseases. <h3>Conclusions</h3> Behcet9s Disease is the most frequent vasculitis in Turkey and more than half of the patients had organ/system involvements. GPA is the most common small vessel vasculitis whereas Microscopic polyangiitis is very rare. Among children Kawasaki was less and Behçet9s disease markedly more when compared to Western European figures. Even though this study is a single-center one, the number of annual admitted patients of our hospital along with more than 2 years-prospective recruitment, reflects the true burden of vasculitides in Turkey, at the cross-roads between Europe and Middle East. <h3>Disclosure of Interest</h3> None declared
AB0869 Stress fractures, a retrospective study of 325 fractures in 227 patients in rheumatology practice between 2000 and 2015
Authors: C Nourisson, J-J Dubost, B Pereira, A Tournadre, S Malochet-Guinamand, M Soubrier
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Stress fractures are common in both young and active patients (fatigue fracture) as well as in elderly osteoporotic patients (insufficiency fractures). <h3>Objectives</h3> The aim of this study was to describe characteristics of stress fractures treated in rheumatology at the Clermont-Ferrand University Hospital, to compare them according to their anatomic location (pelvis, lower limb or feet/ankle) and their diaphyseal or metaphyseal/epiphyseal location. <h3>Results</h3> Between 01/01/2000 and 12/31/2015, 325 fractures were identified in 227 patients divided in 176 women (including 116 postmenopausal women) and 51 men. Population averaged age was 65.2 years [15–99 years]. 142 (43.7%) fractures occurred at pelvis, 93 (28.6%) at lower limbs and 87 (26.8%) at feet and ankles. The fracture was spontaneous in 63.6% of cases. History of pain was recorded in 92.3% with more frequently (90.3%) mechanical pain. Diagnostic delay was on average 70 days. In 51 patients (22.5%) an orthopedic factor could have promoted stress fracture. 54 patients (23.8%) had a history of chronic inflammatory rheumatism, 28 (12.3%) of cancer, and 20 (8.8%) had chronic renal failure. Among the possible iatrogenic factors, 51 patients (23.4%) received oral corticosteroid therapy, 20 (9.2%) methotrexate, 59 (27%) proton pump inhibitor and 17 (7.8%) serotonin reuptake inhibitor. 49 patients (22.5%) already had vitamin-calcium supplementation, 37 (17%) received biphosphonate therapy. 56.6% were osteoporotic and 29.5% were osteopenic. First-line medical imaging was in 87% conventional radiography, positive in 37%. 106 patients (46.7%) performed bone scan with a sensibility of 99%. MRI sensibility (n=65, 28.7%) was 88% and CT-scan sensibility (n=47, 20.7%) 65.2%. Compared to other sites, pelvic fractures were more frequent (p&lt;0.001) and occurred more frequently in postmenopausal women (p=0.03). These patients were older (p&lt;0.001) and had more fracture history (p=0.006). Hip bone mineral density (p=0.03) was lower, and osteoporosis prevalence higher (p=0.004). Pelvic fractures occurred more frequently after fall (p&lt;0.001), lower limb fractures spontaneously (p=0.03) and those of the feet-ankles group after an unusual activity (p&lt;0.001). Patients from metaphyseal-epiphyseal fracture group were older (p=0.05) than those from the diaphyseal fractures group and delay in completing complementary examinations was longer (p=0.04). <h3>Conclusions</h3> In conclusion, pelvic stress fractures are more frequent in a rheumatology department and suggest insufficiency fracture. Stress fracture should be more often suspected when mechanical pain of the pelvis and lower limbs occurs, even spontaneously. Regardless of the site, an assessment of fracture risk factors and bone mineral density evaluation seems necessary. <h3>Disclosure of Interest</h3> None declared
Recombinant ADAMTS-13: first-in-human pharmacokinetics and safety in congenital thrombotic thrombocytopenic purpura
Authors: Marie Scully, Paul Knöbl, Karim Kentouche, Lawrence Rice, Jerzy Windyga, Reinhard Schneppenheim, Johanna A. Kremer Hovinga, Michiko Kajiwara, Yoshihiro Fujimura, Caterina Maggiore, Jennifer Doralt, Christopher Hibbard, Leah Martell, Bruce Ewenstein
Journal: Blood (2017)