tbuckley/cabot-search huggingface dataset:
PS1474 NON‐HODGKIN LYMPHOMA IN PATIENTS WITH MYELOPROLIFERATIVE NEOPLASMS
Authors: K. Yoshinaga, M. Shiseki, M. Ryuzaki, Y. Iizuka, A. Watanabe, N. Tanaka, M. Ishiyama, A. Shinohara, H. Kazama, S. Hagiwara, N. Mori, J. Tanaka
Journal: HemaSphere (2019)

Background: Development of aggressive B‐cell lymphomas during JAK1/2 inhibitor therapy in patients with myeloproliferative neoplasms (MPN) were reported in 2018 European Haematology Association Meeting in Stockholm. It's important to evaluate the incidence of lymphomas in patients with ruxolitinib. Aims: We conducted a retrospective study to evaluate the incidence and clinical characteristics of lymphomas in patients with MPN. Methods: We reviewed laboratory data and clinical record of the patients with MPN diagnosed until Dec 2017 in Tokyo Women's Medical University Hospital. Results: Among 207 patients with MPN, including 129 essential thrombocytosis (ET), 68 polycythemia vera (PV), and 10 primary myelofibrosis (PMF), three patients (1.4%) developed an aggressive non‐Hodgkin Lymphoma (NHL) (Table). Among 207 patients with MPN 15 patients (7.2%) received ruxolitinib. Two of three patients developing NHL had a history of ruxolitinib treatment. There was significant difference in the incidence of NHL occurrence between MPN patients with ruxolitinib treatment (2/15, 13.3%) and those without ruxolitinib treatment (1/192, 0.5%) (Fisher's exact test, P = 0.014). Initial symptoms of lymphoma were extra‐nodal mass (cutaneous or colon mass) in two patients with ruxolitinib treatment. Times from starting ruxolitinib treatment to the diagnosis of lymphoma in these two patients were 2 and 16 months, respectively. They did not achieve complete response by anti‐lymphoma chemotherapy, and died from AML. In contrast, the DLBCL patient without ruxolitinib achieved complete response and is still alive. Summary/Conclusion: The occurrence of lymphoma was more common in patients with ruxolitinib than in the non‐administered group. Porpaczy et al reported that pre‐existing B‐cell clones in myelofibrosis developed to B‐cell lymphoma after ruxolitinib treatment. In the present cases, lymphoma occurred within relatively short period after starting ruxolitinib treatment, suggesting that there were pre‐existing lymphoma clones before ruxolitinib treatment. Extra‐nodal site involvements were seen in present cases similar to the previous reports, suggesting biological difference of lymphoma complicated with MPN patients. Unlike previous report, one T‐cell lymphoma case was included in the present study. It remains unclear whether there is difference in clinical features and mechanisms between B‐cell lymphoma and T‐cell lymphoma development in MPN patients with ruxolintinib treatment. It may be underestimated to the occurrence of lymphoma if biopsy may not perform depending on the condition of patient. We need to careful watch and actively examine the tumor in MPN. image
AB0095 A REGULATORY ROLE OF ANTXR1 IN RANKL-INDUCED OSTEOCLAST DIFFERENTIATION AND FUNCTION
Authors: Chang-Hoon Lee, Ju-Young Kim, Chong Hyuk Chung, Yoon-Jung Choi, Wan-Hee Yoo, Myeung-Su Lee
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Anthrax toxin receptor 1 (ANTXR1) has been known to have relationship with extracellular transmembrane protein deeply associated with the process of bone formation and exert important role in angiogenesis. However, there have been no reports to prove the effects of ANTXR1 on bone metabolism mediated by two types of bone cells, osteoclasts and osteoblasts. The aim of this study is to reveal the role of ANTXR1 in the differentiation and function of osteoclasts and osteoblasts. <h3>Objectives:</h3> The aim of this study is to reveal the role of ANTXR1 in the differentiation and function of osteoclasts and osteoblasts. <h3>Methods:</h3> To determine the effect of ANTXR1 on osteoclastogenesis or osteoblast differentiation, we examined TRAP staining, F-actin staining and Pit assay, or ALP and Alizarin Red-mineralization staining, respectively. The mechanism of ANTXR1 by transfection of retrovirus or siRNA analyzed using real-time PCR and western blot analysis. Also, the effect of ANTXR1 on osteoclast-mediated angiogenesis of endothelial cells assessed by in vitro vascular tubule formation assay of human umbilical vein endothelial cells (HUVECs). <h3>Results:</h3> Through performing gain- and loss-of-function studies, we found that ANTXR1 positively regulated receptor activator of nuclear factor kappa B ligand (RANKL)-induced osteoclast differentiation and bone resorption with no effects on osteoblast differentiation. During ANTXR1-mediated regulation of osteoclastogenesis, phosphorylation of early signal transducers, c-jun N-terminal kinase (JNK), Akt, and inhibitor of kappa B (IκB) was affected, which in turn alteration of mRNA and protein levels of c-Fos and nuclear factor of activated T cells cytoplasmic 1 (NFATc1). In addition, genetic manipulation of ANTXR1 in bone marrow macrophages (BMMs) modulated the capillary-like tube formation by HUVECs via two kinds of angiogenic factors, matrix metalloproteinase-9 (MMP-9) and vascular endothelial growth factor-A (VEGF-A). These results explained the important role of ANTXR1 in osteoclast differentiation and functional activity, as well as, osteoclast-mediated angiogenesis of endothelial cells. <h3>Conclusion:</h3> Taken together, it was proposed that ANTXR1 might be a promising candidate for gene therapy related with bone metabolic diseases and further, have potential to be served as an important biomarker in the research fields of bone metastasis associated with vascularization. <h3>Disclosure of Interests:</h3> None declared
Does surgery of lymphatic malformations lead to an increase in superficial lymphangiectasia? A retrospective study of 43 patients
Authors: A. Schreiber, V. Soupre, N. Kadlub, E. Galliani, A Picard, B. Chrétien‐Marquet, S. Pannier, S. Guéro, N. Khen‐Dunlop, S. Hadj‐Rabia, P. Delanoe, C. Bodemer, O. Boccara
Journal: British Journal of Dermatology (2019)
THU0472 FIBROMYALGIA SYNDROME IN WEST AFRICA: ACR 1990 IS NOT SENSITIVE FOR THE UNDER-DIAGNOSED AND WIDELY MISUNDERSTOOD DISORDER
Authors: Richard Akintayo, Bola Ahmed, Kayode Olafimihan, Foluke Akintayo
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Like most poorly understood rheumatic conditions, Fibromyalgia is thought to be rare in West Africa. Many clinicians rely on the various ACR classification criteria to make a diagnosis of Fibromyalgia despite the unknown performance of these criteria among patients of sub-Saharan African origin. <h3>Objectives</h3> To describe the characteristics of fibromyalgia in Nigerian patients and determine the sensitivities of four ACR criteria sets. <h3>Methods</h3> Consecutive patients diagnosed with fibromyalgia for the first time by a rheumatologist were evaluated using ACR 1990, ACR 2010, 2011 modification of ACR 2010 (ACR 2011) and ACR 2016 classification criteria for fibromyalgia. Sleep quality was assessed with the Pittsburgh Sleep Quality Index (PSQI), fatigue with the Fatigue Severity Scale (FSS) and severity of fibromyalgia with the Revised Fibromyalgia Impact Questionnaire (FIQR). The overall characteristics and the beliefs of these patients regarding their disease were analysed. <h3>Results</h3> Of the one hundred and fourteen (114) patients, ninety six (96) were females; male-to-female ratio is 1:5.3. The median duration of symptoms before diagnosis is 54 months (3 to 273 months) and the mean age is 44.6±15.6 years. Mild, moderate, and severe fibromyalgia were found in 32(28.1%), 53(46.5%) and 29(25.4%) patients respectively. Sensitivities of ACR 1990, ACR 2010, ACR 2011, andACR 2016 were 38.5%, 68.2%, 76.7% and 76.7%, respectively. Poor sleep was found in 83 (72.8%) patients. Patients in functional classes I, II, and III were 71 (62.3%), 19 (16.7%) and 24 (21.1%), respectively. There was none in class IV. There was positive history of widespread pain in at least one first degree relative of 56 (49.1%) patients. Twenty-one (18.4%) patients had changed or quit their job due to the disease and there was significant association between job loss and number of tender points as well as disease severity. The Mean FFS score is 4.48±1.3 and 73 (64.0%) patients believed their problem is a spiritual attack while 43 (37.7%) had done one form of ritual or the other in search of cure. <h3>Conclusion</h3> Fibromyalgia syndrome exists in West Africa. It is poorly understood among the sufferers and ACR 1990 is not sensitive as a diagnostic tool for the condition. <h3>References</h3> [1] Bidari A, Ghavidel Parsa B, Ghalehbaghi B. Challenges in fibromyalgia diagnosis: from meaning of symptoms to fibromyalgia labeling. The Korean journal of pain. 2018 Jul;31(3):147-54. <h3>Disclosure of Interests</h3> None declared
Abstract 21417: Fragmented QRS Complex as a Predictor of Exercise-Related Sudden Cardiac Death
Authors: Tomi M. Toukola
Journal: Circulation (2017)

Objective: Our aim was to identify electrocardiographic (ECG) changes related to exercise-induced sudden cardiac death (SCD). Methods and Results: The Finnish Study of Genotype and Phenotype Characteristics of SCD (FinGesture) includes 3,989 consecutive sudden cardiac deaths in northern Finland between 1998 and 2012. Previously recorded ECGs were acquired manually from archives of Oulu University Hospital. A total of 647 SCD had a previously recorded ECG. In 276 of these cases the death was witnessed, and the activity at the time of death could be divided into either rest or physical exercise; in 40 (14%) cases SCD was exercise-related (PE group) and in 236 (86%) cases death took place at rest (R group). A total of 371 (57%) deaths were unwitnessed or the cases did not meet the criteria of either exercise or rest. Fragmented QRS (fQRS) complex in at least 2 consecutive leads within anterior leads (V1-V3) was more common in the exercise-group compared to rest-group (17/40, 43% vs. 51/236, 22%, p=0.005). Pathologic Q wave in anterior leads was somewhat more common in the PE group (9/40, 23% vs. 26/236, 11%; p=0.044). QRS duration, QTc interval or the prevalence of inverted T-waves did not differ significantly between the 2 groups. Conclusions: Fragmented QRS complex in the anterior leads is associated with an increased risk of SCD during physical exercise.
Abstract 23241: Effects of Candesartan and Metoprolol on Myocardial Mass,Edema, and Fibrosis During Anthracycline Treatment in Early Breast Cancer: APRADA-CMR substudy
Authors: Siri Lagethon Heck, Geeta Gulati, Pavel Hoffmann, Florian von Knobelsdorff-Brenkenhoff, Tryggve H Storås, Anne H Ree, Berit Gravdehaug, Helge Røsjø, Kjetil Steine, Jürgen Geisler, Jeanette Schulz-Menger, Torbjørn Omland
Journal: Circulation (2016)

Background: Anthracycline treatment may cause myocardial damage with myocyte death and expansion of the myocardial extracellular volume (ECV) fraction by edema and fibrosis. We tested the hypothese...
An xQTL map integrates the genetic architecture of the human brain's transcriptome and epigenome
Authors: Bernard Ng, Charles C White, Hans-Ulrich Klein, Solveig K Sieberts, Cristin McCabe, Ellis Patrick, Jishu Xu, Lei Yu, Chris Gaiteri, David A Bennett, Sara Mostafavi, Philip L De Jager
Journal: Nature Neuroscience (2017)
THU0315 SEVERITY AND RESPONSE TO INDUCTION THERAPY IN NEW AND RELAPSING ANCA ASSOCIATED VASCULITIS PATIENTS – REAL WORLD PRACTICE DATA
Authors: Peter Rutherford, Dieter Götte
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> ANCA-associated vasculitis (AAV) presents clinically with variable severity of disease and current clinical guidelines give varying advice on initial treatment to induce remission. Achieving remission quickly is important to gain disease control and avoid cumulative organ damage from treatment related adverse events. <h3>Objectives</h3> This study examined real world practice of AAV treatment in Europe to understand the AAV severity spectrum and the response to therapy over 12 months. <h3>Methods</h3> 929 incident and 268 relapsing EU AAV patients receiving care from 399 physicians were studied. Patients were referred between 2014-17 and data collected retrospectively at baseline and 1, 3, 6 and 12 months following induction therapy. Birmingham Vasculitis Activity Score (BVAS) was collected in only 12% of patients and severity was defined as mild (localized disease with no systemic symptoms), moderate (Mod, systemic disease with lung and/or kidney involvement) or systemic (rapidly progressive systemic disease with lung and/or kidney involvement). <h3>Results</h3> AAV disease severity varied in both incident (mild 12.2%, moderate 54.3%, severe 33.6%) and relapsing (corresponding values 9.7, 64.6 and 25.7) patients at start of treatment. Comorbidity at time of induction therapy was more common in severe patients (71.8% of patients having at least one comorbidity) compared to mild patients (55.1%). Since BVAS was not measured routinely, clinical response was categorized as full (no vasculitis activity and GC taper on track), partial (reduction in vasculitis activity and major organ damage arrested) and no response (no improvement in vasculitis). Clinical response is presented below (% patients) for combination of incident and relapsing patients demonstrating that response varied with many patients having slow and/or incomplete response. Response varied by severity of the disease when induction therapy commenced. <h3>Conclusion</h3> Incident and relapsing AAV patients have variable disease severity at the time of induction therapy. Response to induction therapy is with few exceptions better in patients with milder AAV but overall many patients are slow to respond or have only a partial response to current induction therapy. <h3>Acknowledgement</h3> This work was supported by Vifor Pharma <h3>Disclosure of Interests</h3> Peter Rutherford Employee of: Vifor Pharma, Dieter Götte Employee of: Vifor Pharma
FRI0141 EFFICACY OF JAK-INHIBITORS VERSUS BIOLOGIC DMARDS ON QUALITY OF LIFE IN RHEUMATOID ARTHRITIS :A META ANALYSIS OF RANDOMIZED CONTROLLED TRIALS
Authors: Mamadaly Boudhabhay, Thomas Barnetche, Pascale Vergne-Salle
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Recent studies comparing JAK-inhibitors (Jak-i) and adalimumab seem to show a better efficacy of JAK-i on patient-reported outcomes in rheumatoid arthritis (RA.) <h3>Objectives:</h3> As there is no study comparing directly the JAK-i with other biologic DMARDs (bDMARDs), we performed a meta-analysis and compared the effect size of JAK-iand bDMARD versus synthetic DMARDs (sDMARDs) on quality of life. <h3>Methods:</h3> We performed a systematic review of the literature until May 2018 using database including : MEDLINE (via PUBMED), EMBASE and abstracts from the ACR and EULAR congresses 2015-2017. We selected all randomized controlled trials (RCT) comparing quality of life (evaluated by SF36) in patient with rheumatoid arthritis treated with bDMARD or JAK-i versussDMARDs. We performed meta-analysis comparing the effect size of JAK-i (versus sDMARDs) and bDMARD (versus sDMARDs) on PCS SF36 and MCS SF36 at 12 weeks. Statistical analysis determined in each study effect size. Pooled ES were computed by meta-analysis. Data were analyzed using the inverse variance approach. <h3>Results:</h3> The literature search identified 240 articles plus one found by manuel search and no congress abstract. Finally, 44 articles met the inclusion criteria. JAK-i and bDMARD showed higherlevel of quality of life than conventional therapies: For the SF36 PCS at 12 weeks:JAK–i:+4,82 IC95% [3,88, 5.77] and bDMARDs : +3,99 IC95% [2,81, 5,18] For the SF36 MCS a 12 weeks: JAK–i: +3,42 IC95% [2.24, 4,60] and bDMARD: 2,99 IC95% [2.02, 3.96] The range of the confidence intervals seems similar between JAK-I and bDMARDs. <b>SF36 PCS at 12 weeks:</b> Results for JAK-i: Results for bDMARDs: <b>SF36 MCS at 12 weeks:</b> -Results for JAK-i: -Results for bDMARDs: <h3>Conclusion:</h3> In this meta-analysis, JAK-i and bDMARD showed better SF36 PCS and MCS at 12 weeks in comparison with sDMARDs. Although there is no significant difference, there seems to be a trend toward a better efficacy of JAK-I compared to bDMARD on quality of life. The range of the confidence intervals seems similar between JAK-I and bDMARDs, suggesting a similar efficiency on the components of the SF36. More head-to-head studies are needed to draw definitive conclusions on potential efficacy differences between JAK-inhibitor and bDMARDs in RA. <h3> Disclosure of Interests: </h3> Mamadaly Boudhabhay: None declared, Thomas Barnetche Consultant for: Roche SAS, Pascale Vergne-Salle: None declared
PB2021 GASTRIC MUCOSA‐ASSOCIATED LYMPHOID TISSUE LYMPHOMA: A MONOCENTRIC EXPERIENCE
Authors: W. Ben Kridis, S. Falleh, S. Charfi, S. Khmiri, N. Toumi, T. Boudawara, J. Daoud, A. Khanfir
Journal: HemaSphere (2019)

Background: Mucosa‐associated lymphoid tissue (MALT) lymphoma accounts for 7‐8% of B‐cell lymphoma. Tumor proliferation consists of type B lymphomatous cells of low grade malignancy. They are considered indolent and are part of the marginal zone. They constitute the predominant histological type of non‐Hodgkin's malignant lymphoma of the stomach. Transformation into high‐grade lymphoma is rare. Aims: The aim of this study is to describe the clinical characteristics and the therapeutic results of MALT lymphoma. Methods: We reviewed retrospectively the records of 18 patients presenting with gastric MALT lymphoma treated between 1993 and 2018. Patients with low‐grade lymphoma in failure after helicobacter eradication had exclusive gastric external radiotherapy. Chemotherapy were indicated in case of high grade lymphoma transformation. Radiotherapy doses range between 30 and 40 Gy (2 Gy per fraction, five fractions per week). Results: Median age was 52 years old. All tumours were IE stage. Helicobacter pylori was positive in 15 cases. Three patients had other cancers (gastric adenocarcinoma, breast cancer, mantle lymphoma). All patients received antibiotic therapy (triple therapy in 12 cases and concomitant treatment in 7 cases) with responding residual disease in 6 cases and probable minimal residual disease in 9 cases. Second line antibiotherapy was pylera in 3 cases and levofloxacin with amoxicillin in 2 cases. Seven patients with low‐grade MALT lymphoma had radiotherapy. Four patients with high‐grade transformed lymphoma received chemotherapy. Seventeen patients are in complete remission after treatment achievement. Five and 10‐ year disease‐free survival are 100%. Summary/Conclusion: Eradication of Helicobacter pylori is the mainstay of treatment of gastric MALT lymphoma. The levocine‐amoxicillin combination with proton pump inhibitor seems to be effective as a second‐line treatment in case of non availability of the pylera. External irradiation is an effective and well‐tolerated treatment modality in case of resistance to helicobacter eradication. Immuno‐chemotherapy is indicated for diffuse lymphoma (stage III and IV) or in case of transformation into large cell lymphoma.
THU0723-HPR WHAT DO SJÖGREN’S SYNDROME PATIENTS THINK ABOUT RESEARCH?
Authors: Lucia Martin-Gutierrez, Coziana Ciurtin, Elizabeth Jury
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Despite recent progress investigating the pathogenesis of Sjögren's syndrome (SS), there are currently no therapies able to influence disease progression. Patients with SS are usually treated symptomatically with variable success, therefore refining the research agenda according to patients' priorities could provide a valuable insight into the most appropriate research questions. <h3>Objectives:</h3> The aim of this study was to explore patients' ideas and suggestions related to research in SS. <h3>Methods:</h3> A questionnaire to investigate SS patient experience of diagnosis and ideas about research priorities in SS was designed. Over a 5 month period, patients attending outpatient clinics were invited to fill in the questionnaire. 27 patients completed our anonymous survey. <h3>Results:</h3> The most important issue for patients with SS after they had been diagnosed was the lack of knowledge about this disease (25%), the diagnosis delay they had experienced (26%) and the persistence of symptoms of dryness (26%). Patients were interested in finding out what causes this disease (81%) and felt that our research should find a cure (92%) and better treatments (75%). The main two research priorities selected by patients were improved understanding of the disease (89%) and attracting funding for supporting research in to SS (89%). In addition, patients wanted to have a voice and contribute to raising awareness of SS and to participate in patient and public involvement and engagement (PPIE) events. The majority of patients interviewed were willing to take part in research by donating blood samples and/or filling in questionnaires (96%). 58% of patients who completed the survey would either definitely or probably take part in discussion groups helping researchers to design future studies in SS. <h3>Conclusion:</h3> This survey highlighted patients' perception of the need for more meaningful research into the causes of SS, as their priorities were centred around finding a cure or better treatments for Sjögren's Syndrome. The survey also identified patients' lack of knowledge about their condition as well as their desire to help with shaping future research ideas and support funding for research. The results of this survey will be incorporated in our future PPIE events aiming at shaping our research strategy in SS. <h3>Acknowledgement:</h3> British&nbsp;Sjögren's Syndrome&nbsp;Association and&nbsp;Versus&nbsp;Arthritis <h3>Disclosure of Interests:</h3> None declared