tbuckley/cabot-search huggingface dataset:
Common Elements in Rare Kidney Diseases: Conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference
Authors: Ségolène Aymé, Detlef Bockenhauer, Simon Day, Olivier Devuyst, Lisa M. Guay-Woodford, Julie R. Ingelfinger, Jon B. Klein, Nine V.A.M. Knoers, Ronald D. Perrone, Julia Roberts, Franz Schaefer, Vicente E. Torres, Michael Cheung, David C. Wheeler, Wolfgang C. Winkelmayer, Aris Angelis, Corinne Antignac, Kyongtae Bae, Carsten Bergmann, Anthony J. Bleyer, Marjolein Bos, Klemens Budde, Katherine Bull, Dominique Chauveau, Avital Cnaan, Martina Cornel, Etienne Cosyns, Jane de la Fosse, Jie Ding, Susie Gear, Timothy H.J. Goodship, Paul Goodyer, Oliver Gross, Nicole Harr, Peter C. Harris, Tess Harris, Julia Höfele, Marie C. Hogan, Ewout Hoorn, Shigeo Horie, Clifford E. Kashtan, Larissa Kerecuk, Robert Kleta, Martin Konrad, Craig B. Langman, Segundo Mariz, Gayle McKerracher, Annet Nieuwenhoven, Dwight Odland, Eric Olinger, Alberto Ortiz, York Pei, Yves Pirson, Brian L. Rayner, Giuseppe Remuzzi, Daniel Renault, Rémi Salomon, Aude Servais, Richard J. Smith, Neveen A. Soliman, Bénédicte Stengel, Marjolein Storm, Roser Torra, William van't Hoff, Rosa Vargas-Poussou, Elizabeth Vroom, Christoph Wanner, Hui-Kim Yap
Journal: Kidney International (2017)

Rare kidney diseases encompass at least 150 different conditions, most of which are inherited. Although individual rare kidney diseases raise specific issues, as a group these rare diseases can have overlapping challenges in diagnosis and treatment. These challenges include small numbers of affected patients, unidentified causes of disease, lack of biomarkers for monitoring disease progression, and need for complex care. To address common clinical and patient issues among rare kidney diseases, the KDIGO Controversies Conference entitled, Common Elements in Rare Kidney Diseases, brought together a panel of multidisciplinary clinical providers and patient advocates to address five central issues for rare kidney diseases. These issues encompassed diagnostic challenges, management of kidney functional decline and progression of chronic kidney disease, challenges in clinical study design, translation of advances in research to clinical care, and provision of practical and integrated patient support. Thus, by a process of consensus, guidance for addressing these challenges was developed and is presented here.
AB0117 CADHERIN-11 MRNA expression is increased in the peripheral blood of patients with active spondylarthropathy: a pilot study
Authors: NI Vlachogiannis, PF Christopoulos, G Bamias, K Fragiadaki, M Tektonidou, PP Sfikakis
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Cadherin-11 is a key regulator of synovial architecture and has a central role in the formation of the rheumatoid pannus. Immunohistochemical studies have shown upregulation of cadherin-11 in the synovium of patients with seronegative spondylarthropathies (SpA), comparable to that in rheumatoid arthritis (RA), as well as in the intestinal tissue of patients with inflammatory bowel disease (IBD). Moreover, cadherin-11 mRNA transcripts have been identified in the peripheral blood of patients with RA and independently associated, among various disease characteristics, with the presence of active inflammation in multiple joints [1]. <h3>Objectives</h3> To test the hypothesis that cadherin-11 mRNA transcripts are increased in the peripheral blood of patients with active SpA and search for possible associations with clinical features. <h3>Methods</h3> Fifteen patients with active SpA (BASDAI&gt;4), aged between 21 and 71 years, 11 men, and 30 age- and gender-matched healthy controls were examined. Peripheral whole blood samples (3 ml) were subjected to cDNA synthesis and cadherin-11 mRNA expression was quantified by real-time PCR. Available cDNA from 33 IBD patients without axial or peripheral active arthritis served as disease control and were studied in parallel. <h3>Results</h3> Cadherin-11 mRNA was detected in the peripheral blood of 9/15 (60%) patients with SpA versus 5/30 (17%) healthy controls and 10/33 (30%) patients with IBD (SpA vs healthy controls p: 0.006, SpA vs IBD p: 0.06). Notably, cadherin-11 was not associated with BASDAI or skin/nail psoriasis present in 10 patients, but was detected with increased frequency among SpA patients with clinically active peripheral arthritis at the time of sampling (7 out of 10, 70%) than the remaining patients (2 out of 5, 40%). Moreover, cadherin-11 positivity associated significantly with increased erythrocyte sedimentation rate in SpA but not in IBD. <h3>Conclusions</h3> Cadherin-11 mRNA is upregulated in the peripheral blood of patients with SpA and may correlate with "spreading" of inflammation in peripheral joints. Since an anti-cadherin-11 mAb is in early clinical development for RA, further studies in patients with inflammatory arthritis are warranted. <h3>References</h3> Sfikakis PP, Christopoulos PF, Vaiopoulos AG, et al. Cadherin-11 mRNA transcripts are frequently found in rheumatoid arthritis peripheral blood and correlate with established polyarthritis. Clin Immunol 2014;155:33–41. doi:10.1016/j.clim.2014.08.008. <h3>Disclosure of Interest</h3> N. Vlachogiannis: None declared, P. Christopoulos: None declared, G. Bamias: None declared, K. Fragiadaki: None declared, M. Tektonidou: None declared, P. Sfikakis Grant/research support from: Educational grant from Janssen
Minimal Residual Disease (MRD) Assessment By Multiparametric Flow Cytometry Is Prognostic for Progression-Free Survival in Phase 1/1b Relapsed/Refractory Acute Myeloid Leukemia (AML) Patients Treated with Idasanutlin MDM2 Antagonist
Authors: Benjamin Lanza, Giovanni Martinelli, Karen W.L. Yee, Lori Jukofsky, Bernhard Reis, Steven Blotner, Mark W. Drummond, Norbert Vey, Karen Seiter, Michael J. Dickinson, Kevin R. Kelly, Margaret Kasner, Michel Theron, Jeffrey M. Venstrom, Steven Middleton, Lin-Chi Chen, Nelson Kinnersley, Gwen Nichols, William E. Pierceall
Journal: Blood (2016)
AB0911 Acute rheumatic fever case series presenting with arthritis in adults
Authors: I Dogan, ON Koyun
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Acute rheumatic fever (ARF) is an inflammatory reaction to group A hemolytic streptococcus that emerging 2–3 weeks after infection; presenting with joint, cardiac, neurological and dermatological symptoms. The incidence is reported to be 0.5–3: 100.000 in developed countries and 20–100: 100.000 in developing countries.<sup>1</sup> ARF is a common disease in Turkey. It is most common between the ages of 6 and 20 years. It is rare in adults and arthritis is common with carditis in adults.<sup>2</sup> <h3>Objectives</h3> Due to the ARF is rarer at adulthood, ARF-associated arthritis at adults has been reported as a case report series. <h3>Methods</h3> Five patients who were admitted to our rheumatology policlinic in a tertiary medical center located in Corum province, with early arthritis between 2014 and 2016 were diagnosed with ARF associated arthritis. <h3>Results</h3> The age of ARF associated arthritis patients ranged from 35 to 56 years (Mean age 45.6 years). Two of these patients were male and three were female. One patient has atrial fibrillation and coronary artery disease in other one as a comorbidity. All of patients had tonsillitis in the last 15 days. Two of the patients had a previous ARF story. The results of the laboratory and the clinical findings are given in table: Acute rheumatic fever associated arthritis was diagnosed according to Jones criteria due to migratory arthritis, carditis findings with Echocardiography, and ASO titer elevation. The ANA, anti-CCP, RF, and Brucella agglutination tests were negative. Non-steroidal anti-inflammatory drugs, oral methyl prednisolone 16 mg per day and benzathidine penicillin G 1.2 million units per month were initiated as treatments. Methyl prednisolone dose was taperde gradually. In one patient using warfarin, depo peniciline i.m. was not given because it could be risky for hematoma. In another patient with coronary artery disease, methylprednisolone 4 mg per day was started because coronary bypass was planned. Symptoms of all patients were improved after treatment. <h3>Conclusions</h3> Acute rheumatic fever associated arthritis is not a common condition in adult patients,and should be kept in mind with poststreptococcal reactive arthritis in arthritis developed after upper respiratory tract infections in adults. <h3>References</h3> Stollerman G.H. Rheumatogenic group A streptococci and the return of rheumatic fever, Adv. Intern. Med. 35 (1990) 1–25. Ben-Dov I. Acute rheumatic fever in adults over the age of 45 years: an analysis of 23 patients together with a review of the literature. Semin Arthritis Rheum. 1980 Nov; 10(2):100–10. <h3>Disclosure of Interest</h3> None declared
Adjuvant chemotherapy in patients with stage I endometrioid or clear cell ovarian cancer in the platinum era: a Surveillance, Epidemiology, and End Results Cohort Study, 2000–2013
Authors: A. Oseledchyk, M.M. Leitao, J. Konner, R.E. O’Cearbhaill, D. Zamarin, Y. Sonoda, G.J. Gardner, K. Long Roche, C.A. Aghajanian, R.N. Grisham, C.L. Brown, A. Snyder, D.S. Chi, R.A. Soslow, N.R. Abu-Rustum, O. Zivanovic
Journal: Annals of Oncology (2017)
OP0028 Low dose IL-2 therapy can recovery TH17/TREG cell balance in patients with ankylosing spondylitis
Authors: D Xu, J Fan, Q Chen, K Qin, X Li, C Gao
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Recent studies have shown that increased number of Th17 cells and decreased number of Treg cells in the peripheral blood contribute to ankylosing spondylitis (AS). However, current therapy for AS, including biological agents, immunosuppressant and glucocorticoid, can not correct the imbalance of Th17 and Regulatory T (Treg) cells in AS patients effectively. It has been reported that low dose IL-2 can selectively expand Treg cells and had a critical effect on homeostatic balance between the Th17 and Treg cells. <h3>Objectives</h3> The study is to explore the effect of low dose IL-2 therapy on the balance of Treg and Th17 cells in patients with AS and observe the efficiency and side effects of the therapy. <h3>Methods</h3> Seventeen patients, who met the 1984 modified New York criteria and had evidence of active inflammatory spondylitis (defined as Bath AS Disease Activity Index (BASDAI) score &gt;4) despite of receiving standard therapy including glucocorticoid, immune-suppressants, biological agents or combination of them, were enrolled. These patients were not only given traditional treatment, but also injected subcutaneously low-dose IL-2 (50 WIU/day for 5 days). Clinical and laboratory indicators were compared before and after IL-2 treatment. The side effects were observed in the course of therapy. <h3>Results</h3> The number of Treg cells significantly increased after the treatment by 1 week (22.58±12.80 vs. 73.46±33.79, p&lt;0.001). At the same time, there was a significantly decrease in the ratio of Th17/Treg cells (0.67±0.70 vs. 0.32±0.33, p=0.068). Besides, Th17 cells were also increased (12.83±9.24 vs. 19.26±13.24, p=0.054). Clinical manifestations were improved after the combination treatment of IL-2 and traditional drugs, especially BASDAI was decreased (4.57±0.61 vs. 1.98±0.83, P&lt;0.001). No obvious adverse reactions were observed. <h3>Conclusions</h3> Low dose IL-2 therapy can restore and maintain the balance of Th17 and Treg cells in the active patients with AS. Manifestation improved after the combination therapy. The therapy is safe. Further research is needed to investigate long term benefits of low-dose IL-2 therapy. <h3>References</h3> Wright P, Utriainen L, Milling S. Dendritic cells and regulatory T cells in spondyloarthritis. Curr Opin Rheumatol. 2013; 25(4):440–7. Zambrano-Zaragoza JF, Agraz-Cibrian JM, González-Reyes C, et al. Ankylosing spondylitis: from cells to genes. Int J Inflam. 2013; 2013:501653. Klatzmann D, Abbas AK. The promise of low-dose interleukin-2 therapy for autoimmune and inflammatory diseases. Nat Rev Immunol. 2015;15(5):283–94. He J, Zhang X, Wei Y, et al. Low-dose interleukin-2 treatment selectively modulates CD4(+) T cell subsets in patients with systemic lupus erythematosus. Nat Med. 2016;22(9):991–3. <h3>Disclosure of Interest</h3> None declared
SP0170 Assessing rheumatology skills
Authors: C Haines
Journal: Annals of the Rheumatic Diseases (2017)

Medical Education is making great advances in understanding the power of assessment. Modern learners seek frequent, faster feedback on their learning to study more effectively. It is now common for examinations to be statistically monitored in order to show that acceptable levels of reliability have been reached. But, a test is only as good as its questions. Clinicians are not often trained in how to create valid and reliable test questions. This session will describe some basic principles for creating valid and reliable tests for the knowledge, skills and attitudes which are required by practising rheumatologists. The speaker is currently the Eular Educationalist, who advises Eular on how to continually enhance assessment processes for the online courses and the new trainee examination. As a result of the session participants will be better able to: Describe how face validity and reliability apply to Eular assessments Describe the differences between formative assessment for learning and summative assessment for grading Identify improvements which they could make to test questions Identify improvements which they could make to tests <h3>Disclosure of Interest</h3> None declared
THU0079 Evaluation of synovitis in the foot and the ankle not included in the 28-joint disease activity score using ultrasonography and histological findings in the patients with rheumatoid arthritis
Authors: A Abe, H Ishikawa
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> In the treatment of rheumatoid arthritis (RA), the early diagnosis and early medical treatment via tight control have become increasingly important with the advent of biological therapy. Ultrasonography (US) for the affected joints enables the evaluation of synovial hypertrophy, effusion and bone erosion in real time. US is a reliable method that can detect more erosive sites than radiography. US is now utilized widely and is a reliable tool of rheumatologists for diagnosing RA and evaluating the disease activity. <h3>Objectives</h3> This study was conducted to clarify the relationship between the systemic disease activity, local disease activity using US and a synovial histopathological evaluation. <h3>Methods</h3> Between March 2011 and September 2015, 668 joints were surgically treated on the whole, and synovial biopsies were performed at the time of surgery. Among them, a total of 118 toes and 21 ankles, which are not included in the 28-joint disease activity score (DAS28), were investigated. Just before surgery, the US probe was placed on the dorsal aspect of the ankle and/or the toes to evaluate the activity of local synovitis. The maximum grade of power Doppler (PD) signal was determined, ranging from 0 to 3. The serum C reactive protein (CRP), matrix metalloproteinase-3 (MMP-3) and DAS28 values were also examined just before surgery. A histopathological examination of the gathered synovium at the surgical site was performed using the Rooney score (RS). Biological disease-modifying antirheumatic drugs were used in 45 cases, namely infliximab in 7 cases, etanercept in 16, adalimumab in 7, tocilizumab in 6, abatacept in 4, certolizumab pegol in 1, and golimumab in 4. <h3>Results</h3> The PD score was grade 0 in 82 cases, 1 in 32 cases, 2 in 20 cases and 3 in 5 cases. The total RS and its item scores except for "proliferating blood vessels" correlated well with the PD signal intensity. The systemic disease activity, as indicated by DAS28, CRP and MMP-3, had no significant correlation with the local PD signal intensity. However, the DAS28, CRP and MMP-3 values and each RS item score, except for "proliferating blood vessels", were significantly lower in the PD grade 0 group than in the PD grades 1, 2 and 3 groups. <h3>Conclusions</h3> No PD signal intensity in the ankle and foot indicates systemically well-controlled disease activity. US is an excellent tool for determining local synovitis as well as the systemic disease activity in patients with RA. <h3>Disclosure of Interest</h3> None declared
SAT0664 Is there an early ultrasonographic pattern in salivary glands in both primary and secondary sjogren syndrome?
Authors: VC Iorgoveanu, D Mazilu, I Saulescu, V Vlad, V Bojinca, L Groseanu, D Opris, A Balanescu, C Constantinescu, D Predeteanu, R Ionescu
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Sjogren Syndrome (SS) affects mainly exocrine glands. Ultrasonography (US) demonstrates specificity and sensibility in major salivary glands (SG) evaluation. Recent data confirm US might be used as primary evaluation technique for its ability to show structural alterations of parenchyma [1]. <h3>Objectives</h3> To assess the gray scale (GS) parenchymal inhomogeneity of major SG in patients with established primary and secondary SS and correlate with clinical and biological data. <h3>Methods</h3> Consecutive patients with SS were recruited and SG US was performed. Inhomogeneity of glandular parenchyma was quantified binary on each gland. ESSDAI and ESSPRI scores were calculated. Statistics was performed with SPSS. <h3>Results</h3> Twenty one (42.85% primary SS, 90.47% female) consecutive patients were included. Mean age was 53.66+/-12.99 years and disease duration 5.33+/-3.74 years. Antibody SSA/SSB presence was found in 85.7% (18/21). ESSDAI mean was 8.67+/-8.9 (0–29), ESSPRI 10.13+/-5.59 (0–20). There were no differences regarding ESSDAI and ESSPRI in the two groups (primary and secondary SS). Right parotid gland showed alterations in 71.4% patients (77% with primary SS, 66% with secondary SS). Frequently inhomogeneity was found in all major SG (33%, 22% left and right submandibular, 77%, 44.4% left and right parotid glands) in primary SS. Both submandibular glands were symmetrically involved (p&lt;0.02). Duration of disease was negatively correlated to inhomogeneity of right parotid gland (p&lt;0.02). <h3>Conclusions</h3> Inhomogeneity in major SG in GS US was found in the majority of patients with primary and secondary SS. The symmetrical involvement of submandibular glands was significant. The inhomogeneity appears in the early period of diagnosis. No major differences were found between two groups. <h3>References</h3> Damjanov N, Milic V, Nieto-González JC, Janta I, Naredo E. Multiobserver Reliability of Ultrasound Assessment of Salivary Glands in Patients with Established Primary Sjögren Syndrome. J Rheumatol. 2016 Oct;43(10):1858–1863. <h3>Disclosure of Interest</h3> None declared
Circular RNA_LARP4 inhibits cell proliferation and invasion of gastric cancer by sponging miR-424-5p and regulating LATS1 expression
Authors: Jing Zhang, Hui Liu, Lidan Hou, Ge Wang, Rui Zhang, Yanxia Huang, Xiaoyu Chen, Jinshui Zhu
Journal: Molecular Cancer (2017)

Non-coding RNAs (ncRNAs) have been shown to regulate gene expression involved in tumor progression of multiple malignancies. Our previous studies indicated that large tumor suppressor kinase 1 (LATS1), a core part of Hippo signaling pathway, functions as a tumor suppressor in gastric cancer (GC). But, the underlying molecular mechanisms by which ncRNAs modulate LATS1 expression in GC remain undetermined. The correlation of LATS1 and has-miR-424-5p (miR-424) expression with clinicopathological characteristics and prognosis of GC patients was analyzed by TCGA RNA-sequencing data. A novel circular RNA_LARP4 (circLARP4) was identified to sponge miR-424 by circRNA expression profile and bioinformatic analysis. The binding site between miR-424 and LATS1 or circLARP4 was verified using dual luciferase assay and RNA immunoprecipitation (RIP) assay. The expression and localization of circLARP4 in GC tissues were investigated by fluorescence in situ hybridization (FISH). MTT, colony formation, Transwell and EdU assays were performed to assess the effects of miR-424 or circLARP4 on cell proliferation and invasion. Increased miR-424 expression or decreased LATS1 expression was associated with pathological stage and unfavorable prognosis of GC patients. Ectopic expression of miR-424 promoted proliferation and invasion of GC cells by targeting LATS1 gene. Furthermore, circLARP4 was mainly localized in the cytoplasm and inhibited biological behaviors of GC cells by sponging miR-424. The expression of circLARP4 was downregulated in GC tissues and represented an independent prognostic factor for overall survival of GC patients. circLARP4 may act as a novel tumor suppressive factor and a potential biomarker in GC.
FRI0575 Safety of denosumab in a monocentric cohort of kidney transplant recipients
Authors: S Doddoli, P Lafforgue, T Pham
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> The safety of Denosumab, a fully human monoclonal antibody against RANKL, which was developed for treatment of osteoporosis and prevention of fractures, remains unclear in kidney transplanted patients. <h3>Objectives</h3> Our aim was to assess its clinical and biological tolerance in this specific population. <h3>Methods</h3> <i>Design:</i> Prospective observational monocentric cohort. <i>Inclusion criteria:</i> kidney transplant recipient who received at least one subcutaneous injections of 60 mg denosumab; age ≥18 years. <i>Safety assessment:</i> the following variables were collected every 6 months: infection, reaction at the injection site, plasmatic parameters of renal function and mineral metabolism (estimated glomerular filtration rate, serum creatinine, calcium, 1–25 [OH] vitamin D, PTH). <h3>Results</h3> Patients were recruited from April 2014 to September 2015. All patients received immunosuppression therapy including prednisolone ≥5 mg/d. The main baseline characteristics of the 37 kidney transplant recipients were the following [mean]: male: 41%, age: 60.5 years, BMI: 24,1, transplantation duration: 7.1 years, osteopenic patients: 36%, osteoporosis patients: 64%, total lumbar spine T-score: -2.04 SD, total hip T-score: -2.7 SD, T-score femoral neck: 0.676 g/cm<sup>2</sup>, serum creatinine: 132.8 mmol/L, calcium: 2.33 mmol/L, 1–25 [OH] vitamin D: 93.5 nmol/L, PTH 95: ng/l. All patients were prescribed vitamin D and calcium supplementation. During the mean 12-month follow-up period, there were no unexpected adverse event [AE] or severe adverse event, no graft failure and no deaths. No patient experienced fracture. Only one patient presented an infectious AE with recurrent cutaneous abscess. Renal function remained stable with no difference in serum creatinine between baseline and 12 months for the majority of the kidney transplant recipients. However, 9 recipients experienced a decrease in renal function with a mean increased in serum creatinine of 32.5micromol/L between baseline and 12 months. Serum calcium was stable, no hypocalcaemia was observed. Among patients with normal baseline PTH, two presented hyperparathyroidism during the follow-up period. Among the 11 patients with baseline hyperparathyroidism, 7 had an increased PTH level between baseline and 12 months. None were initiated on cinacalcet. <h3>Conclusions</h3> Our results suggest that denosumab is safe in kidney transplant recipients. We did not observe an increase in the infection rates, nor hypocalcemia. However, several patients experienced a decrease in their renal function or an increased hyperparathyroidism. <h3>Disclosure of Interest</h3> None declared
CD4 T cell–restricted IL-2 signaling defect in a patient with a novel IFNGR1 deficiency
Authors: Aaruni Khanolkar, Dawn A. Kirschmann, Edward A. Caparelli, Jeffrey D. Wilks, Jillian M. Cerullo, Jenna R.E. Bergerson, Lawrence J. Jennings, Ramsay L. Fuleihan
Journal: Journal of Allergy and Clinical Immunology (2017)