tbuckley/cabot-search huggingface dataset:
AB0496 Venous thrombosis is more prevalent in patients with antiphospholipid syndrome (APS) accompanying systemic lupus erythematosus, while livedo reticularis in primary aps
Authors: EN Haładyj, A Wieczorek, M Olesińska
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> APS more often overlaps with other systemic autoimmune diseases like systemic lupus erythematosus (SLE) or rheumatoid arthritis (RA) than occur as a distinct disease. Our purpose was to evaluate what are the differences between patients with primary APS and APS accompanying SLE. <h3>Objectives</h3> The objective of this study was to compare patients with primary APS and APS/SLE group whther we can find find any clinical or laboratory parameters that can distinguish them from each other. <h3>Methods</h3> 112 patients with APS were included to the study, 57 of them with primary APS and 55 with coexisting SLE. These patients were followed at the Department of Connective Tissue Diseases, NIGRiR, Warsaw, Poland. At inclusion a full medical history and physical examination data were recorded. <h3>Results</h3> Both groups were similar in age, gender and duration of disease. Among all the clinical manifestations of APS, venous thrombosis was more frequent in patients with concomitant SLE. Skin involvement was significantly more prevalent in primary APS and it was caused mainly by livedo reticularis presence. From neurological manifestations, the occurrence of epilepsy was comparable, when EEG changes were more frequent in APS/SLE group. From laboratory measures, leucopenia, low complement concentrations and proteinuria were more prevalent in APS/SLE group. No differences were observed in thrombocytopenia and elongation of APTT as well as antiphospholipid antibody profile. <h3>Conclusions</h3> In conclusion, arterial thrombosis is more characteristic for primary APS. We found that livedo reticularis is very characteristic feature for primary APS. Although epilepsy occurrence is comparable in APS and APS/SLE, higher frequency of EEG changes in APS/SLE group suggests that the mechanisms leading to this feature can be distinct between this two conditions. Additionally, it appears to be more reasonable to add thrombocytopenia to clinical criteria of APS, because its frequency does not depend on SLE presence. <h3>Disclosure of Interest</h3> None declared
FRI0557 Does weighted kypho-orthosis (WKO) reduce risk of fall in women with osteoporosis? a preliminary study
Authors: SA Raeissadat, SM Rayegani, L Sedighipour, M Hashemi, MH Bahrami
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> It was suggested that posture training support with spinal orthosis including weighted kyphorthosis can improve balance in patients with osteoporosis. <h3>Objectives</h3> The aim of the present study was to determine the effects of weighted kyphorthosis on improving dynamic balance tests and consequently reducing risk of fall in women with osteoporosis. <h3>Methods</h3> In this Randomized controlled clinical trial, twenty three patients with osteoporosis were included. The patients were assigned into two groups: 1) control group who received 4-week home-based daily exercise program and 2) intervention group (weighted kyphorthosis) who performed exercises and wore weighted kyphorthosis for one hour twice a day. Patients were assessed using computerized balance tests by Balance Master (NeuroCom) (Limits of Stability, Step Quick Turn, Sit to Stand and Walk across tests) before and 4 weeks after start of treatment. <h3>Results</h3> Speed in walk across test was improved significantly in both groups compared to baseline from (77.6±25 cm/s to 91.57±30 cm/s and from 72.60±20cm/s to 88.73±18 cm/s)in case and control groups respectively. Improvement in right turn time in step quick turn, end point excursion and mean of excursion parameters of Limits of Stability was more significant in orthosis group in comparison with control group (P&lt;0.05). <h3>Conclusions</h3> Applying WKO together with exercise program improved some computerized balance tests in women with osteoporosis.WKO can be suggested as an effective intervention in postmenopausal women in order to reduce the risk of falling. <h3>Key words</h3> Osteoporosis, balance tests, Weighted KyphOrthoris, posture training support. <h3>Disclosure of Interest</h3> None declared
AB0137 Alterations in microrna expression profiles in primary sjÖgren's syndrome and systemic lupus erythematosus
Authors: G Papp, J-Q Chen, S Pόliska, K Szabό, T Tarr, BL Bálint, P Szodoray, M Zeher
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> MicroRNAs (miRNAs) are single-stranded, endogenous non-coding small RNAs, ranging from 18 to 25 nucleotides in length. MiRNAs are essential in regulating gene expression, cell development, differentiation and function. Dysregulation in miRNAs expression may contribute to the development of autoimmunity. However, a given miRNA may have hundreds of different mRNA targets and a target might be regulated by multiple miRNAs, thus the characterisation of dysregulated miRNA expression profiles could give a better insight into the development of immunological disturbances in autoimmune diseases. <h3>Objectives</h3> The aim of our study was to examine the changes in miRNA expression profiles in patients with primary Sjögren9s syndrome (pSS) and systemic lupus erythematosus (SLE). <h3>Methods</h3> Eight pSS patients, 8 SLE patients and 7 healthy control subjects were enrolled in the investigation. MiRNAs were isolated from peripheral blood mononuclear cells, and expression patterns were determined with Illumina next-generation sequencing technology. Since the immunopathogenesis of pSS and SLE encompasses pronounced B cell hyperactivity along with specific autoantibody production, we paid a special attention on the association between miRNA expression levels and altered peripheral B cell distribution. <h3>Results</h3> In SLE patients 135, while in pSS patients 26 miRNAs showed altered expression. Interestingly, the 25 miRNAs including miR-146a, miR-16 and miR-21, which were over-expressed in pSS patients, were found to be elevated in SLE group, as well. On the contrary, we observed the down-regulation of miR-150–5p, which is a novel and unique finding in pSS. Levels of several miRNAs over-expressed in SLE, were not changed in pSS, such as miR-148a-3p, miR-152, miR-155, miR-223, miR-224, miR-326 and miR-342. Expression levels of miR-223–5p, miR-150–5p, miR-155–5p and miR-342–3p, which miRNAs are potentially linked to B cell functions, showed associations with the B cell proportions within peripheral blood mononuclear cells. <h3>Conclusions</h3> The observed differences in miRNA expression profiles and the better understanding of immune regulatory mechanisms of miRNAs may help to elucidate the pathogenesis of pSS and SLE. <h3>Acknowledgements</h3> This work was supported by the ÚNKP-16–4-III New National Excellence Program of the Ministry of Human Capacities. <h3>Disclosure of Interest</h3> None declared
AB0917 Safetey and efficacy of direct-acting antiviral agents for the treatment of hcv in rheumatic diseases: case series
Authors: MC Ditto, R Talotta, S Parisi, MC Gerardi, A Batticciotto, F Rigamonti, P Sarzi Puttini, E Fusaro, M Antivalle
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Until approximately 5 years ago interferon (IFN) associated with Ribavirina (RBV) was the gold standard of HCV therapy. Literature data shown that IFN could exacerbate symptoms related to the autoimmune diseases, and flares or intolerance are reported. Recent advances in antiviral therapy had completely modified the HCV infection approach. Direct acting antiviral agents (DAAs) are more efficacious, safer and more tolerable of IFN-HCV treatment. <h3>Objectives</h3> To show safety and efficacy of IFN-free HCV eradication in inflammatory arthritis patients with concomitant immunosuppressive treatment. <h3>Methods</h3> We evaluated 5 patients (M:F=4:1; median age 50±13.4), affected by inflammatory arthritis and concomitant HCV infection requiring eradication, treated with cDMARDs or bDMARDs (anti-TNFα) and DAAs. Demographic data and concomitant medications are showed in table 1. <h3>Results</h3> Patient 01 and 02 were affected by psoriatic arthritis with severe cutaneous and articular involvement. The first one was also under Lamivudina treatment because concomitant HBV infection and cirrhosis. Patient 03 was affected by enteropathic arthritis (RCU), treated with AZA 100 mg. Patient 04 was affected by rheumatoid arthritis and latent TBC under Nicizina 300 mg daily treatment. Patient 05 was affected by ankylosing spondylitis. Before starting DAAs he was treated with Telaprevir/Peg-IFN/RBV but during the treatment vulgar psoriasis with diffuse and severe involvement appeared. IFN-therapy was interrupted and DAAs therapy started with no flares or other adverse events. Four patients (01, 02, 04, 05) were under anti-TNF-α treatment and 1 patient (03) was under Azatioprina treatment. During therapy with DAAS any flare of autoimmunity disease or adverse events were observed. HCV-RNA was undetectable after 3 months in 4 patients and after 6 months in 1 patient. <h3>Conclusions</h3> For all of patients IFN-free therapy was efficacious (blood-HCV-RNA undetectable after treatment) and safe (no adverse events). DAAs therapy could be the best choice to HCV eradication in patients affected by autoimmune rheumatic diseases treated with immunosuppressive drugs. <h3>References</h3> Kohtaro Ooka and Joseph K. Lim, Treatment of Hepatitis C in Patients Undergoing Immunosuppressive Drug Therapy, Journal of Clinical and Translational Hepatology 2016 vol. 4 | 206–227. <h3>Disclosure of Interest</h3> None declared
Impact of obesity and overweight in the prognosis of women diagnosed with non metastatic breast cancer in a Mexican cohort.
Authors: José Luis Aguilar, Itzel Anahi Martinez, Cynthia Mayte Villarreal-Garza, Gisell Anaid Lara, Fernando Lara Medina, Alberto Alvarado Miranda, Jaime G. De La Garza, Alejandro Mohar, Abelardo Meneses, Angel Herrera Gomez, Daniela Olvera-Caraza, Martin Granados-Garcia, Oscar Arrieta
Journal: Journal of Clinical Oncology (2012)

1607 Background: Mexico positions right up at the top with U.S. in worldwide rankings of the most obese countries. In addition, breast cancer (BrCa) is the main type of cancer among women in this country. Studies have shown inconsistent results regarding obesity as a prognostic factor for worse outcome. Methods: Our aim is to identify if overweight and obesity confer poor prognosis in non-metastasic BrCa patients (pts). We identified 1799 Hispanic women with newly diagnosed BrCa who attended the National Cancer Institute in Mexico from 2004-2008 and compared clinical and pathological features and overall survival (OS) between pts with a body mass index (BMI) &gt; or ≤ than 25. Results: The median age at diagnosis was 51 years. A BMI&gt;25 was found in 71% of pts. Postmenopausal women comprised 52%, and had a greater proportion of cases with a BMI&gt;25 than premenopausal pts (75% vs. 67%, p&lt;0.0001). Pts with BMI&gt;25 presented with more advanced TNM stages and nodal involvement than their counterparts (73% vs. 67%, p=0.005 and 76% vs. 71%, p=0.017; respectively). Overall prevalence of hormone-receptor (HR), triple-negative (TN) and HER2 positive disease was 62%, 23%, and 27%, respectively. Differences according to receptor status between pre and postmenopausal pts and BMI are shown in table. There was no difference in disease-free survival and OS according to overweight and obesity in the overall population, but when menopausal status was considered, premenopausal pts with BMI&gt;25 had a worse OS compared to pts with BMI&lt;25 (HR 1.6, p=0.037). This difference was not seen in the postmenopausal group. Conclusions: Obesity may influence BrCa outcomes via several hormonal and inflammatory mechanisms. In this study, overweight and obesity confer a poor prognosis in premenopausal patients, possibly related to excess estrogen availability and higher prevalence of TN BrCa. Therefore, overweight and obesity deserve additional attention to assess possible causal relationships that potentially could be modified to improve outcomes in premenopausal patients. [Table: see text]
SAT0342 Transthoracic echocardiography to quantify pulmonary vascular resistance in patients with systemic sclerosis
Authors: S Billet, T Chollet, P Fournier, L Tetu, G Prévot, M Galinier, D Carrié, O Lairez, G Pugnet
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> One of the major causes of systemic sclerosis (SSc)-related death is pulmonary arterial hypertension, which develops in 12–15% of patients with SSc and accounts for 30–40% of deaths. Consequently, monitoring of pulmonary arterial pressure (PAP) and resistances is essential in patients with SSc. Abbas formula performed by transthoracic echocardiography (TTE) was reported as a good tool to quantify pulmonary vascular resistances (PVR). <h3>Objectives</h3> Explore the accuracy of TTE and Abbas formula to quantify PVR in patients with SSc. <h3>Methods</h3> All consecutive patients with SSc, diagnosed according to the 2013 ACR/EULAR criteria, or the LeRoy and Medsger criteria for diffuse or limited subsets classification, had within 24H a Doppler echocardiographic examination and right-heart catheterization were performed. The ratio of peak tricuspid regurgitant velocity (TRV, <i>ms</i>) to the right ventricular outflow tract time-velocity integral (TVI<sub>RVOT</sub>, <i>cm</i>) obtained by Doppler echocardiography (TRV/TVI<sub>RVOT</sub>) was then correlated with invasive PVR measurements using regression analysis. An equation was modeled to calculate PVR in Wood units (WU) using echocardiography, and the results were compared with invasive PVR measurements [1]. <h3>Results</h3> Thirty-three consecutive patients were included, 13 (39.4%) were male and the mean age was 64.6±12.1 years. Most were classified as limited cutaneous SSc (lcSSc; n=29, 87.9%). All patients tested positive for antinuclear antibodies, 18 (21.2%) for anti-scleroderma-70, 7 (54.5%) for anticentromere antibodies and 2 (6.1%) for anti-RNA polymerase III antibodies. Mean and systolic PAP were 31±9 and 53±16 mmHg respectively. There was a good correlation between right ventricle to right atrium gradient pressure assessed by TTE and RHC (R=0.620, P&lt;0.001). RVP assessed by Abbas formula (2.6±1.0 wood units) were well correlated with RVP assessed by RHC (4.8±2.3; R=0.446, P=0.013). <h3>Conclusions</h3> Doppler echocardiography using Abbas formula may provide a reliable, noninvasive method to determine PVR in SSc patients. <h3>References</h3> Abbas AE, Fortuin FD, Schiller NB, Appleton CP, Moreno CA, Lester SJ. A simple method for noninvasive estimation of pulmonary vascular resistance. J Am Coll Cardiol. 2003 Mar 19;41(6):1021–7. <h3>Disclosure of Interest</h3> None declared
A phenotype combining hidradenitis suppurativa with Dowling-Degos disease caused by a founder mutation in <i>PSENEN</i>
Authors: M. Pavlovsky, O. Sarig, M. Eskin-Schwartz, N. Malchin, R. Bochner, J. Mohamad, A. Gat, A. Peled, A. Hafner, E. Sprecher
Journal: British Journal of Dermatology (2017)

Dowling-Degos disease (DDD), featuring reticulate pigmentation, and familial hidradenitis suppurativa (HS) share many clinical features including autosomal dominant inheritance, flexural location and follicular defects. The coexistence of the two disorders was recently found to result from mutations in PSENEN, encoding the γ-secretase subunit protein presenilin enhancer.To investigate PSENEN mutations in a series of four unrelated patients who presented with combined DDD and HS.Mutation and haplotype analysis of PSENEN by polymerase chain reaction, and cellular assays investigating the Notch signalling pathway.Here we report four families of Jewish Ashkenazi origin who presented with clinical features characteristic of both disorders. All patients were found to carry the same, heterozygous mutation in PSENEN (c.168T>G, p.Y56X). Haplotype analysis revealed that the mutation originated from a common ancestor. Genes associated with DDD, as well as HS, have been shown to encode important regulators of Notch signalling. Accordingly, using a reporter assay, we demonstrated decreased Notch activity in a patient's keratinocytes.The present data confirm the genetic basis of the combined DDD-HS phenotype and suggest that Notch signalling may play a central role in the pathogenesis of this rare condition.
FRI0038 Release of peptidylarginine deiminiase 2 from activated neutrophils
Authors: MA Steffensen, D Damgaard, M Bassi, CH Nielsen
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Extracellular citrullination catalyzed by peptidylarginine deiminase (PAD) is thought to play a central role in the pathogenesis of rheumatoid arthritis. Neutrophils are a major reservoir of PAD2 and PAD4. Cellular release of PAD2 and PAD4 is usually considered a consequence of cell death. <h3>Objectives</h3> We aimed to determine if PAD2 can be released from live, activated neutrophils as an active process. <h3>Methods</h3> Whole blood cells were purified from healthy blood were stimulated with phorbol 12-myristate 13-acetate (PMA). To capture PAD2 released from neutrophils and detect it by flow cytometry, we used biotinylated anti-CD15 and anti-PAD2 (mAb DN6) mAbs linked by streptavidin, and a different PE-labelled anti-PAD2 amtibody (mAb DN2). In addition, intracellular PAD2 was quantified by intracellular staining with PE-anti-PAD2. PAD2 released from leukocytes and subcellular fractions of human granulocytes were assessed for content of PAD2 using an in-house luminex-based assay. <h3>Results</h3> On incubation of whole blood cells with PMA, PAD2 was detectable in the supernatants after 30 minutes, and levels increased thereafter in parallel with increasing cell death. However, using PAD2 catch reagent, we found that live neutrophils released PAD2 in the 30 minutes after stimulation. Intracellular staining for PAD2 showed that the content of PAD2 in live neutrophils decreased correspondingly. Upon subcellular fractioning of granulocytes, the majority of PAD2 was found in cytosol and, in 25 times lower quantities, in the fraction containing plasma membranes and secretory vesicles. Sparse amounts of PAD2 were also observed in gelatinase granules. <h3>Conclusions</h3> In conclusion, PAD2 can be released from live, activated neutrophils, which may contribute to extracellular citrullination and thereby play a role in driving inflammatory processes in RA patients with immune responses to citrullinated proteins. <h3>Disclosure of Interest</h3> None declared
AB0862 The sensitivity of the double contour sign in hand joints would be better by the dorsal surface examination
Authors: AM Mahdi, H Rkain, M Erraoui, R Watfeh, S Aktaou, L Tahiri, R Bahiri, F Allali, N Hajjaj-Hassouni
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Objectives</h3> To compare the prevalence of ultrasonographic gout specific sign double contour between the dorsal and palmar surfaces of the hand joints. <h3>Methods</h3> This is a cross-sectional study which includes 15 patients with chronic gout, defined according to the American College of Rheumatology criteria (ACR 1977). Ultrasound (US) examination was performed using a high-frequency linear probe (Toshiba Xario®, frequency (8–14 MHz)) in B and Doppler modes. 560 articular sites were studied at their dorsal and palmar surfaces. We compared the prevalence of the hyperechoic band over the superficial margin of the articular cartilage described as a double contour (DC) between the dorsal and palmar surfaces at each site studied. <h3>Results</h3> The mean age at onset was 54.7±12,6 years, and the median diagnosis duration was 0 (0.3) years). The results of the US examination are summarized in Table 1 <h3>Conclusions</h3> Our study suggests that globally, DC predilect significatily in dorsal than in palmar surfaces of hand joints. These results should be verified on a larger population. <h3>Disclosure of Interest</h3> None declared
SP0186 Cognitive fog: subjective and objective understandings of the symptom of dyscognition
Authors: B Walitt
Journal: Annals of the Rheumatic Diseases (2017)

Dyscognition refers to the complaint that a person9s ability to perform thinking tasks is impaired. This complaint is colloquially known as "brain fog". It is a major symptom of a variety of disorders and associated with considerable work and social disability for those experiencing it. However, attempts to demonstrate objective cognitive impairment in persons reporting "brain fog" have not been straightforward. In this lecture, the symptom of cognitive dysfunction will be described from the patient9s point of view, using fibromyalgia as a disease model. The cognitive tests used to determine objective alterations in cognitive ability will be reviewed, the amount of objective impairment demonstrated in fibromyalgia will be placed into clinical context, and the "disconnect" between what the experience of dyscognition is and the cognitive content measured by modern testing will be discussed. The poor relationship between the magnitude of subjective dyscognition and objective cognitive performance will be examined, including evidence gleaned from neurological imaging studies. In conclusion, the experience of cognitive fog is not well captured by current testing paradigms. Subjective complaint is a poor predictor of objective cognitive performance. The neuronal mechanisms responsible for the experience of cognitive fog may be separate from those required to perform cognitive tasks. <h3>Disclosure of Interest</h3> None declared
Cardiomyopathies
Authors: Eugene Braunwald
Journal: Circulation Research (2017)

The nonischemic cardiomyopathies are a diverse group of cardiac disorders that frequently cause heart failure and death and are now recognized with increasing frequency. There has been substantial progress in the clinical recognition and understanding of the natural history of these conditions. Well-established and new techniques of cardiac imaging are also helpful in this regard. Basic scientists are elucidating the pathogenesis and pathobiology of individual cardiomyopathies. In this compendium, some of the most important advances in this field are reviewed. Scientific opportunities to enhance further collaborative research to accelerate progress are identified.
SpyGlass percutaneous transhepatic cholangioscopy-guided lithotripsy of a large intrahepatic stone
Authors: Tomazo Franzini, Leandro Cardarelli-Leite, Estela Figueira, Flávio Morita, Fernanda Domingos, Francisco Carnevale, Eduardo de Moura
Journal: Endoscopy (2017)

Occasionally, biliary stone management can be really challenging, depending on location, size, number, altered anatomy, and presence of strictures [1]. Although different approaches can be used in this setting, such as endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic biliary drainage (PTBD), and extracorporeal shock wave lithotripsy or surgery [2], more complex cases may require management using a combination of techniques [3] [4] [5].