tbuckley/cabot-search huggingface dataset:
AB0546 Clinicopathological characteristics of sjÖgren's syndrome in the presence or absence of objective sicca symptoms
Authors: Y Suzuki, H Fujii, K Yamada, M Kawano
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Sjögren9s syndrome (SS) is generally diagnosed on the basis of objective criteria, including xerophthalmia, xerostomia, autoantibodies, and labial salivary gland biopsy. Patients without objective sicca symptoms (non-sicca SS) require a biopsy. For such patients, we should evaluate pretest probability using parameters other than sicca symptoms before performing an invasive biopsy. To assess pretest probability, data on clinicopathological characteristics of non-sicca SS are needed. <h3>Objectives</h3> This study aimed to analyze the clinicopathological features of non-sicca SS. Epidemiological data, antibody profiles, organ involvement, and labial salivary gland biopsy results in non-sicca SS patients were compared with those in SS patients with objective sicca symptoms (sicca SS). <h3>Methods</h3> We selected 103 patients with primary SS who met Japanese or American College of Rheumatology criteria; those whose results exceeded the focus score by 1 underwent salivary gland biopsy. Objective xerophthalmia was evaluated with the Schirmer9s test, and objective xerostomia with the Saxon9s test. Seventeen patients were excluded because neither test was performed. Sicca SS was defined as a positive Schirmer9s and/or Saxon9s test result. Clinical and laboratory data were compared in 70 sicca SS and 16 non-sicca SS patients. <h3>Results</h3> Non-sicca SS patients were younger at diagnosis (45.9±14.8 vs. 61.4±15.1 years, p&lt;0.001), had a shorter disease duration (1.1±1.5 vs. 6.9±8.9 years, p&lt;0.001), and had a higher rate of positive anti-SS-A/Ro antibody (100 vs. 74.3%, p=0.023), and a lower rate of positive anti-centromere antibody (6.3 vs. 44.3%, p=0.005). Subjective xerophthalmia and xerostomia rates were similar between the groups, but fewer non-sicca SS patients had sicca symptoms as chief complaints (18.8 vs. 58.6%, p=0.004). There were no significant differences in focus score, leukocyte and lymphocyte counts, serum IgG levels, and positive rheumatoid factor and antinuclear antibody levels. The maximum European League Against Rheumatism Sjögren9s Syndrome Disease Activity Index (ESSDAI) score during follow-up showed no significant difference (3.34±4.27 in non-sicca SS vs. 3.83±4.68 in sicca SS, p=0.30). However, more non-sicca SS patients had ESSDAI scores ≧ 1 (100 vs. 71.4%, p=0.015), a positive correlation with the biological domain of the ESSDAI (87.5 vs 58.6%, p=0.03), and articular symptoms (37.5 vs 8.6%, p=0.003). <h3>Conclusions</h3> Non-sicca SS patients were younger, had shorter disease duration, and a higher rate of positive correlation with the biological and articular domains of the ESSDAI. Moreover, all non-sicca SS patients had ESSDAI scores ≥1. When we diagnose SS patients without objective sicca symptoms, we should assess age, disease duration, and extraglandular organ involvement before performing labial salivary gland biopsy. <h3>Disclosure of Interest</h3> None declared
Targeting bone morphogenic protein receptor 2 (BMPR2) signalling to treat pulmonary arterial hypertension
Authors: Lewis J. Rubin
Journal: European Respiratory Journal (2017)

Since the demonstration nearly 20 years ago that mutations of bone morphogenic protein receptor 2 (BMPR2) are associated with heritable pulmonary arterial hypertension [1–4], investigators have postulated that altered BMPR2 signalling, whether inherited or acquired, may be a novel therapeutic target for pulmonary arterial hypertension (PAH) of all aetiologies. Enhancing BMPR2 activity in animal models of pulmonary hypertension attenuates the severity of pulmonary vascular growth and proliferation [5, 6], although these models have not been predictive of responses in human disease. In the current issue of the European Respiratory Journal, Spiekerkoetter et al. [7] report that BMPR2 mRNA expression is attenuated in PAH and that FK506 (tacrolimus), a BMPR2 activator, produced no meaningful effects in a small, single-centre 16-week clinical trial in subjects with PAH due to a variety of aetiologies. While the authors identified several "FK506 responders" in a post hoc analysis, they found no difference in BMPR2 expression between "responders" and "non-responders", nor did they observe any change in serologic biomarkers linked to increased BMPR2 signalling. BMPR signalling may be a therapeutic target for PAH, but further studies are needed <http://ow.ly/KokP30efxbf>
FRI0535 Strong influence of vitamin d status on bone mineral density and bone turnover markers during weight restoration in patients with anorexia nervosa
Authors: A Giollo, L Idolazzi, C Caimmi, A Fassio, F Bertoldo, R Dalle Grave, S Calugi, PV Bazzani, O Viapiana, M Rossini, D Gatti
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Anoressia nervosa (AN) is associated with an increased risk of low bone mineral density (BMD) and fractures as a consequence of an inadequate bone mass peak in adolescence and bone loss in young adulthood. Moreover, recently we have showed that vitamin D (25-OH-D) deficiency is widespread in untreated patients with AN, and there is a strong positive relationship between vitamin D status and BMD in AN. However, if vitamin D status could affect the efficacy of weight restoration in improving bone health in patients with AN is currently unknown. <h3>Objectives</h3> Our aim was to investigate the potential role of vitamin D status in determining the efficacy on bone mineral density (BMD) of weight restoration in AN. <h3>Methods</h3> Bone mineral density assessed by dual-energy x-ray absorptiometry (DXA), vitamin D, N-propeptide of type I collagen (P1NP), C-terminal telopeptide of type I collagen (CTX), intact parathyroid hormone (PTH) were evaluated before and after a 20-weeks intensive weight restoration therapy in patients with anorexia nervosa and secondary amenorrhoea for at least 6-months. The subjects were not receiving medications known to affect bone metabolism. <h3>Results</h3> Ninety-one female patients aged 13–45 years old were evaluated, baseline weight 39.4±5.6 kg and BMI 15.1±1.6 kg/m<sup>2</sup>. Weight and BMI were significantly increased in all patients after treatment. The mean BMD values were significantly increased only at the spine (1.0±3.6%, p=0.009). A positive trend was demonstrated between post-treatment 25-OH-D and BMD changes at the spine (p=0.032). However, only the patients with post-treatment 25-OH-D ≥30 ng/ml showed significantly higher increases in BMD at the spine (2.5% vs 0.5% respectively for 25-OH-D ≥30 ng/ml and 25-OH-D &lt;30 ng/ml, p&lt;0.03; Figure 1). Both P1NP and PTH increased, whereas a significant decrease was found in 25-OH-D and CTX (p&lt;0.05). Post-treatment CTX levels were inversely correlated with spine BMD. A positive relationship was found between changes in weight and P1NP (R2 =0.27). <h3>Conclusions</h3> In anorexia nervosa, a hypovitaminosis D status counteracts the efficacy of the weight restoration treatment because of an increase in bone resorption mediated by a secondary hyperparathyroidism. Our study strongly support the use of vitamin D supplements for bone health in anorexia nervosa. <h3>Disclosure of Interest</h3> None declared
AB0182 In vitro characterization of dermal fibroblasts from systemic sclerosis patients
Authors: R Brizzolara, P Montagna, S Soldano, AC Trombetta, B Ruaro, A Sulli, S Scabini, E Stratta, V Smith, M Cutolo
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Systemic sclerosis (SSc) is characterized by progressive fibrosis of the skin and/or internal organs. Skin fibrosis is mainly due to the excessive production of extracellular matrix (ECM) proteins from dermal activated fibroblasts. The high amounts of transforming growth factor-beta (TGFbeta) production induce fibroblast proliferation and their transition into profibrotic myofibroblasts. Consequently, the increased presence of alpha-smooth muscle actin (alpha-SMA)-positive myofibroblasts in affected tissues is associated with fibrotic tissue remodelling, that characterize SSc [1–4]. <h3>Objectives</h3> To characterize dermal fibroblasts from SSc patients by evaluating the gene expression of specific phenotypic and profibrotic markers: fibrillar collagen type I (COL I), fibronectin (FN), fibroblast-specific protein 1 (S100A4), TGFbeta and alpha-SMA (marker of myofibroblasts). <h3>Methods</h3> Human dermal fibroblasts were obtained by skin biopsy from affected areas of 6 active lcSSc patients after written informed consent and approval of medical ethics committee. SSc patients fulfilled the new EULAR/ACR criteria for SSc and they were treated only with various vasodilators (no severe clinical SSc complications were present at the time of skin sampling). Human SSc dermal fibroblasts were cultured up to 80% of confluence and at the third to fifth subpassages were used for experiments. Total RNA was extracted and quantitative real-time PCR (qRT-PCR) was performed to evaluate the gene expression of relevant fibroblast markers: COL I, FN, S100A4, TGFbeta and alpha-SMA. <h3>Results</h3> In human SSc fibroblasts the gene expression of the relevant markers COL I, FN and S100A4 resulted of 10<sup>7</sup> RNA copies for each gene. Interestingly, in the same tested fibroblasts, the profibrotic alpha-SMA gene expression (marker of myofibroblasts) was similarly found highly expressed, with a result of 10<sup>7</sup> gene copies. On the contrary, TGFbeta showed a much lower gene expression (10<sup>3</sup> copies) compared to the other investigated genes. <h3>Conclusions</h3> In human SSc cultured dermal fibroblasts, the gene expression of ECM proteins (COL I and FN), was found associated with a relevant gene expression of S100A4 and alpha-SMA, confirming their myofibroblast phenotype, as highly differentiated. Interestingly, TGFbeta gene appears to be less expressed. It could be concluded that myofibroblasts from the SSc skin mainly undergo the effects of the tissutal TGFbeta, since it seems that they do not contribute to its further production once differentiated. <h3>References</h3> Cutolo M. et al. J Rheumatol 2015;42:3. Brunasso A.M. et al. F1000Research 2016;5:723. Hinz B et al. Am J Pathol 2012;180:1340–55. Dumoitier N. et al. Arthritis Rheumatol 2016 [Epub ahead of print]. <h3>Disclosure of Interest</h3> None declared
Abstract 72: Impact of Extracorporeal Membrane Oxygenation Support on Clinical Outcome of Pediatric Patients With Acute Cardiopulmonary Failure: A Single-center Experience
Authors: Jiunn-Jye Sheu, Sarah Chua, Hon-Kan Yip
Journal: Circulation (2013)

Background: Conventional therapy against acute pediatric cardiopulmonary failure (APCPF) caused by a variety of disease entities remains unsatisfactory with extremely high morbidity and mortality. For refractory APCPF, extracorporeal membrane oxygenation (ECMO) is one of the last resorts. Materials and Methods: In this study, the in-hospital outcomes of pediatric patients with refractory APCPF receiving ECMO support were reviewed. Results: Between August 2006 and May 2011, a single-center cohort study was performed in pediatric patients who required ECMO support due to cardiogenic shock or severe hypoxemia. A total of 22 patients with mean age of 7.0 ± 6.3 years received ECMO (male = 11; female = 11). The indications included acute fulminant myocarditis (AFM) (n = 6), congenital diaphragmatic hernia (CDH) (n = 3), acute respiratory distress syndrome (ARDS) (n = 6), enterovirus 71 (n = 3), viral sepsis (n = 2), refractory ventricular fibrillation due to long QT syndrome (n = 1), and pulmonary edema with brain herniation (n = 1). Eighteen patients received veno-arterial (VA) mode ECMO, while another four patients undertook the veno-venous (VV) mode. The duration of ECMO use and hospitalization were 6.1 ± 3.1 and 24.4 ± 19.4 days, respectively. The survival rate in patients with AFM was 100% (n = 6). Successful ECMO weaning with uneventful discharge from hospital was noted in 14 (63.6%) patients, whereas in-hospital mortality despite successful ECMO weaning occurred in 5 patients (22.7%). Failure in ECMO weaning and in-hospital death was noted in 3 patients (13.6%). Conclusions: ECMO resuscitation is an effective strategy in the clinical setting of APCPF. Key words: Acute cardiopulmonary failure, clinical outcome, extracorporeal membrane oxygenation, pediatrics
THU0697 Feasibility and reliability of the sparcc sacroiliac joint structural score for children with spondyloarthritis
Authors: NA Chauvin, WP Maksymowych, RG Lambert, JL Jaremko, DM Biko, J Paschke, TG Brandon, PF Weiss
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Clinical trials in juvenile spondyloarthritis (JSpA) and axial disease are lacking. To assess the effectiveness of medications, we need measures to evaluate structural progression in the pediatric sacroiliac joint (SIJ). <h3>Objectives</h3> To evaluate the reliability of the SPARCC sacroiliac joint structural score (SSS) in children with suspected or confirmed JSpA. <h3>Methods</h3> The SSS assesses a spectrum of structural lesions of the SIJ on MRI including fat metaplasia, erosion, backfill, and ankylosis on 5 consecutive slices through the cartilaginous part of the joint. These components are scored 0–20 (backfill and ankylosis) or 0–40 (fat metaplasia, erosion). We developed a pediatric training module that included a detailed description of each SSS component plus sclerosis (0–40), scoring methodology, and numerous examples. After reviewing the module, 6 readers (mix of adult and pediatric radiologists and rheumatologists) scored 30 studies (Exercise 1). All readers then reviewed a second training module based on DICOM images and scored an additional 29 studies (Exercise 2). Inter-observer reliability was assessed using intraclass correlation (ICC). <h3>Results</h3> The SSS had face validity and was feasible to score in the 59 pediatric cases. 35 (59%) were male and median age was 15 years (IQR 12–16). The ICCs for the SSS components from both calibration exercises are shown in the Table. In the 1st exercise, fat metaplasia and sclerosis had good reliability (≥0.4) while reliability for erosion, backfill, and ankylosis were low. During the first exercise, 31 (17%), 131 (73%), 56 (31%), 100 (56%), and 21 (12%) of the 180 ratings from 6 readers had a score&gt;0 for fat metaplasia, erosion, backfill, sclerosis, and ankylosis, respectively. In the second exercise, the ICC for erosion, backfill, and sclerosis improved and had good reliability. Of the 174 ratings from 6 readers, 91 (52%), 18 (10%), and 49 (28%) of scores were &gt;0 for erosion, backfill, or sclerosis in exercise 2, respectively. Reliability for fat metaplasia and ankylosis were not calculated due to low frequency of lesions (8 (5%) and 3 (2%), respectively). <h3>Conclusions</h3> The SSS was feasible to score and had acceptable reliability for pediatric SIJ MRI evaluation. ICC improved with additional calibration exercises based on DICOM, even for readers with limited experience. <h3>Disclosure of Interest</h3> None declared
Comparison of two fluoroscopic images to ensure efficient scope insertion for biliary intervention in patients with Roux-en-Y hepaticojejunostomy
Authors: Koichiro Tsutsumi, Hironari Kato, Ken Hirao, Sho Mizukawa, Shinichiro Muro, Yutaka Akimoto, Daisuke Uchida, Kazuyuki Matsumoto, Takeshi Tomoda, Shigeru Horiguchi, Shuntaro Yabe, Hiroyuki Seki, Yasuhiro Noma, Naoki Yamamoto, Ryo Harada, Tsuneyoshi Ogawa, Hiroyuki Okada
Journal: Endoscopy (2017)

Abstract Background and study aims No standard procedure for endoscopic retrograde cholangiopancreatography is available for patients with Roux-en-Y hepaticojejunostomy (RYHJ) with side-to-end hepaticojejunostomy. We therefore explored methods of efficient scope insertion at a hepaticojejunostomy site. Patients and methods Patients with suspected biliary disease were prospectively enrolled. Based on two fluoroscopic images obtained on scope insertion into each lumen of a two-pronged Roux-en-Y anastomosis, we selected the lumen in which the distal end of the scope progressed toward the patient’s liver or head. The accuracy of this method for selecting the correct lumen leading to the hepaticojejunostomy site was investigated. Results Of the 33 included patients, successful insertion to the hepaticojejunostomy site was achieved in 32 (97 %), 26 (81 %) of whom had undergone the imaging method. The accuracy of the method was 88 % (23/26). The time required for insertion between the anastomotic site and the hepaticojejunostomy site was shorter when the lumen selection had been correct (13 minutes [7 – 30] (n = 23) vs. 18 minutes [8 – 28] (n = 9); P = 0.95). Conclusion This method based on two fluoroscopic images was useful for achieving efficient scope insertion in patients with RYHJ. Trial registered at University Hospital Medical Information Network Clinical Trials Registry (UMIN000014183).
AB0805 Panlar consensus. recommendations for the management of osteoarthritis of the hand, hip and knee. short title: pan-american league of associations for rheumatology (PANLAR) osteoarthritis (OA) study group
Authors: MJ Quintero, H Riera, R Espinosa, R Souto, C Rossi, JF Molina, J Salas, F Radrigan, G Reyes, L Urioste, S Papasidero, A Garcia, I Iraheta, E Nieto, R Arape, O Castaneda, J Angulo, I Coimbra, LM Franco, C Acosta, V Liendo, R Chacόn-Díaz, M Vera, A Reginato, CV Caballero, O Rillo
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> This consensus derives from a previous study of Demographic and Clinical Characteristics by the PANLAR OA study group, reporting significant differences in handling these patients and in which it was evident the need of reaching an agreement in the management of hand, hip and knee OA in Latin America (2) <h3>Objectives</h3> The aim is to update the recommendations for the treatment of hand, hip and knee OA by agreeing on key propositions relating to the management of hand, hip and knee OA,identifying and critically appraising research evidence for the effectiveness of the treatments and by generating recommendations based on the available evidence and expert opinion. <h3>Methods</h3> Recommendations were developed by a group of 40 specialists made up of rheumatologists and members of other medical disciplines. A systematic review of articles, meta-analyses and guidelines for the management of hand, hip and knee OA published from 2008 and January 2014 was done. The level of evidence and strength of recommendation were classified according to the Jadad scale (3).The level of agreement was established through a Delphi technique. <h3>Results</h3> Both "strong" and "conditional" recommendations are given for management of hand, hip and knee OA and non-pharmacological, pharmacological and surgical modalities of treatment are presented according to the different levels of agreement. <h3>Conclusions</h3> These recommendations are based on the consensus of clinical experts from a wide range of disciplines considering the available evidence, while balancing the benefits and risks of non-pharmacological, pharmacological and surgical treatment modalities. It is hoped that these recommendations will be utilized by healthcare providers involved in the management of patients with hand, hip and knee OA. <h3>References</h3> Reginato, Anthony M. et al. Osteoarthritis in Latin America: Study of Demographic and Clinical Characteristics in 3040 Patients. JCR: Journal of Clinical Rheumatology 21.8(2015):391–397. Jadad, A.R.; Moore R.A.; Carroll D.; Jenkinson C.; Reynolds D.J.M.; Gavaghan D.J.; McQuay H.J.(1996). Assessing the quality of reports of randomized clinical trials: Is blinding necessary? Controlled Clinical Trials 17:1–12. doi: 10.1016/0197-2456(95)00134-4. PMID 8721797. <h3>Disclosure of Interest</h3> None declared
AB0174 Lymphocyte subsets t, b and nk cels in systemic sclerosis
Authors: L Schneider, V Hax, N Marcondes, RM Xavier, R Chakr
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Systemic sclerosis (SSc) is a rare multisystem disease with underlying immune mechanisms, whose pathogenesis remains unclear. Few previous reports have evaluated lymphocyte subpopulations in SSc and your results are conflicting. <h3>Objectives</h3> The present study aimed to analyze the lymphocyte subsets in SSc patients in comparison to healthy individuals. <h3>Methods</h3> Peripheral blood (PB) samples to analyze lymphocyte subsets were obtained from a non-random convenience sample of 20 SSc patients. Twenty healthy individuals recruited from the blood bank were used as sex and age-matched controls. Blood samples were analyzed by flow cytometry for total T cells, CD4+ and CD8+ T cells subsets, CD19+ B cells and total NK cells. Statistical analyses were performed using the IBM Statistical Package for Social Sciences (SPSS 18.0). Data are expressed as mean ± SD and median and range. Non-parametric Mann–Whitney U test was used for analyses of the flow cytometry. A probability <i>p</i>&lt;0.05 was considered statistically significant. <h3>Results</h3> The mean (SD) age of SSc patients was 57.9 (14.2) years, 95% were female and 31.6% presented diffuse cutaneous SSc (dcSSc). Patients presented a lower mean total lymphocyte count compared to healthy controls (23.7% vs. 29.6%, <i>p</i>=0.026) (Table 1.). No statistically significant differences were found in the percentages or the absolute numbers of T, B or NK cells. <h3>Conclusions</h3> Our data support previous reports indicating that depletion of lymphocyte in the PB of SSc patients. However, we found no significant difference in relation to lymphocyte subtypes, which differs from the literature data. <h3>References</h3> T and NK Cell Phenotypic Abnormalities in Systemic Sclerosis: a Cohort Study and a Comprehensive Literature Review. Almeida et al, 2015. Liu M, Wu W, Sun X, Yang J, Xu J, Fu W, Li M. New insights into CD4+ T cell abnormalities in systemic sclerosis. Cytokine &amp; Growth Factor Reviews, 2016; 28:31–36. Gambichler T, Tigges C, Burkert B, Höxtermann S, Altmeyer P, Kreuter A. Absolute count of T and B lymphocyte subsets is decreased in systemic sclerosis. Eur J Med Res 2010; 15:44–46. <h3>Disclosure of Interest</h3> None declared