tbuckley/cabot-search huggingface dataset:
OP0219 Update on Malignancies in Children with Juvenile Idiopathic Arthritis in The German Biker Registry: Table 1.
Authors: G. Horneff, A. Klein, A. Hospach, P. Oommen, I. Foeldvari, J. Kuemmerle-Deschner, I. Feddersen, K. Minden
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> While TNFα-inhibitors improved outcome of JIA markedly, concerns have been raised about an increased risk for malignancies especially lymphoma, probably already due to the disease itself. <h3>Methods</h3> The BIKER data base was used to identify cases of suspected and confirmed malignancies <h3>Results</h3> Until 2015, 3695 pts treated with MTX and /or TNFα inhibitors were prospectively followed with 13198 observation years (OY). 12 cases of suspected malignancies, including 7 lymphoid neoplasms, have been reported. 11 pts had received MTX, 2 CSA, single patients received Sulfasalazine, Azathioprine or Leflunomide. 9 pts received Etanercept (ETA), 2 Adalimumab, and 1 case consecutively ETA, Infliximab and Abatacept. A case of myelodysplasia, with spontaneous recovery and a cervical dysplasia were labelled as suspected. Confirmed cases included 2 Hodgkin9s lymphoma, 1 non-Hodgkin9s lymphoma, 2 ALL and 2 lymphoproliferative disorders, who recovered after discontinuation of immunosuppressive therapy. Singular confirmed cases were thyroid carcinoma, yolk sac carcinoma, cervical dysplasia and anaplastic ependymoma. The total rate of malignancies observed in the registry (0.91/1000Y) exceeds the rate expected according to data of the German cancer registry (0.16/1000Y, p&lt;0.001). The calculated risk-ratio in the MTX cohort (n=3: 1 lymphoma, 2 acute lymphatic leukaemias) was significantly increased for both all malignancies (RR 4.5; 95% CI1.5–14.0) and for haemato-lymphatic malignancies (RR 10.1; 95% CI 3.2–31.2). The risk ratio in the ETA cohort also was significantly increased (RR for all malignancies 8.1; 95% CI 4.2–15.5; RR for leukaemia/lymphoma 10.4 (3.9–27.7). No difference was found between the incidence rate of malignancies within the ETA cohort compared to the MTX cohort (p=0.39). Rates did not markedly differ if suspected malignancies (cervical dysplasia, myelodysplasia) were excluded. 6 of the 7 haemato-lymphatic malignancies occurred in ps &lt;15 y of age. There was a higher rate within MTX (0.93/1000Y; 95%CI 0.30–2.89) than with ETA (0.56/1000Y; 95%CI 0.18–1.74). 4 of 5 solid tumours occurred after the age of 15, all in the ETA cohort. One patient never exposed to biologics but to MTX died of ALL, all other recovered. <h3>Conclusions</h3> Malignancies were more frequent than expected. Whether the increased risk is through the rheumatic disease or through treatment remains unclear. Treatment with ETA seems not to further increase the malignancy risk. Long-term observation of JIA patients treated with TNFα inhibitors into adulthood remains an important challenge. <h3>Disclosure of Interest</h3> None declared
Mycophenolate Mofetil (MMF) with or without Tracolimus (FK506) as a Second Line Treatment for Steroid-Resistant Acute Graft-Versus-Host Disease. The Experience of Saint Louis Hospital.
Authors: Gustavo Fisher, Vanderson Rocha, Moema dos Santos, Agnes Devergie, Marie Robin, Regis P. de Latour, Patricia Ribaud, Christele Ferry, Eliane Gluckman, Gerard Socie
Journal: Blood (2006)
Genetic Abnormalities and Patterns of Antigenic Expression in Multiple Myeloma.
Authors: G. Mateo, M. Castellanos, A. Rasillo, N. C. Gutierrez, M. A. Montalban, M. L. Martin, J. M. Hernandez, M. C. Lopez-Berges, L. Monteiano, Joan Blade, M. V. Mateos, A. Sureda, J. Rubia, J. Diaz-Mediavilla, J. J. Lahuerta, A. Orfao, J.F. San Miguel
Journal: Blood (2004)
Copy Number Abnormalities Of The Interferon Regulatory Factor-4 (IRF4) Gene Are Associated With IRF4/MUM1 Expression In Peripheral T-Cell Lymphomas
Authors: Rebecca L Boddicker, Ryan A Knudson, Rhett P Ketterling, Law E Mark, Xiaoming Xing, James R Cerhan, Yanhong Wu, Julie M Cunningham, Stephen M Ansell, Andrew L. Feldman
Journal: Blood (2013)
Equilibrium and Non-Equilibrium Molecular Dynamics Simulations Provide Potential Mechanisms for the Loss of Ligand Binding to αIIbβ3 Mutants Affecting the Ligand-Induced Metal Binding Site (LIMBS).
Authors: Marta Murcia, Marketa Jirouskova, Jihong Li, Barry S. Coller, Filizola Marta
Journal: Blood (2006)
Reassessment of Cardiac Function and Implantable Cardioverter-Defibrillator Use Among Medicare Patients With Low Ejection Fraction After Myocardial Infarction
Authors: Sean D. Pokorney, Amy L. Miller, Anita Y. Chen, Laine Thomas, Gregg C. Fonarow, James A. de Lemos, Sana M. Al-Khatib, Eric J. Velazquez, Eric D. Peterson, Tracy Y. Wang
Journal: Circulation (2016)

Guidelines recommend that patients with low ejection fraction (EF) after myocardial infarction (MI) have their EF reassessed 40 days after MI for implantable cardioverter-defibrillator (ICD) candidacy. This study examines rates of EF reassessment and their association with 1-year ICD implantation in post-MI patients with low EF.We examined rates of postdischarge EF reassessment and ICD implantation among 10 289 Medicare-insured patients ≥65 years of age with an EF≤35% during the index MI admission from January 2007 through September 2010 in ACTION Registry-GWTG (Acute Coronary Treatment and Intervention Outcomes Network Registry-Get With The Guidelines). Multivariable Cox models tested the association between time-dependent EF reassessment and 1-year ICD implantation, stratified by revascularization status during the index MI admission.Among patients with EF ≤35% during the index MI admission, 66.8% (95% confidence interval [CI], 65.9-67.8) had EF reassessment within the next year. Revascularized patients were more likely to have EF reassessment (76.9% [95% CI, 75.8-78.0)] versus 53.7% [95% CI, 52.2-55.2]; P<0.001) and had shorter times to EF reassessment (median, 67 versus 84 days; P<0.001) than nonrevascularized patients. Among patients with EF reassessment, only 11% received an ICD within 1 year. Reassessment of EF was associated with a higher likelihood of ICD implantation for both revascularized (unadjusted, 12.1% versus 2.4%, P<0.001; adjusted hazard ratio, 10.6, 95% CI, 7.7-14.8) and nonrevascularized (unadjusted, 10.0% versus 1.7%, P<0.001; adjusted hazard ratio, 6.1, 95% CI, 4.1-9.2) patients.In US practice, EF reassessments are commonly performed among patients with MI with an initially reduced EF. Although 1-year EF reassessment is associated with increased likelihood of ICD implantation, 1-year ICD implantation rates remain very low even among patients with EF reassessment, regardless of revascularization status.
SAT0322 Prevalence and Risk Factors for Cognitive Impairment in Systemic Lupus Erythematosus
Authors: O. Vera-Lastra, M. Castrejon, J. Sepulveda-Delgado, M.D.P. Cuz-Dominguez, G. Medina, L.J. Jara
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Neuropsychiatric disorders have a high morbidity being cognitive impairment (CI) one of the most frequent syndromes in systemic lupus erythematosus (SLE) which lead to a poor quality of life <h3>Objectives</h3> To determine the prevalence and risk factors of CI in patients with systemic lupus erythematosus <h3>Methods</h3> Twenty seven patients were selected from a total of 150 patients with SLE. CI was evaluated in all patients by the test of Barcelona (for qualitative evaluation of cognitive state: language, reading, writing, visual recognition, memory and abstraction) and the Montreal cognitive assessment (COCA). The following risk factors were evaluated: SLEDAI, obesity/overweight, systemic arterial hypertension, dyslipidemia, pulses of methylprednisolone, SLIC/ACR, use of benzodiazepine, cyclophosphamide and chloroquine. Statistical analysis: Fisher test, T-test Student and logistical regression. <h3>Results</h3> There were 27 SLE patients (25 women and 2 men), mean age 42±14 years) mean disease evolution (10±8.6 years), length of education (11.7±4.2 years). With the test of Barcelona we found that 100% of patients had involvement in at least one item, being the most affected areas: attention in 33%, memory in 52%, analysis synthesis and abstraction in 44.4%. With respect to the MOCA a prevalence of 55.6% (15 of 27) of CI was found. Of these patients 12 of 15 had mild CI and 3 of 15 dementia. We only found that the use of chloroquine conferred protection against cognitive impairment (OR 0.236, CI 0.120–0.692, p&lt;0.003). The other risk factors were not significant: dyslipidemia (OR 8.5, CI 0.508–143, p= NS), SLIC/ACR (OR 6.3, CI 0.64–109.7), obesity/overweight (OR 0.407, CI 0.02–5-2, systemic arterial hypertension (OR 0.782, CI 0.031–754), pulses of methylprednisolone (1. 85, CI 0.64- 18.119), use of cyclophosphamide (OR 0. 327, CI 0.115–7.056), among others. <h3>Conclusions</h3> We found a high prevalence of cognitive impairment being the use of chloroquine a protective factor. It is important to recognize these patients in order to offer them a medical alternative with a psychological rehabilitation. <h3>Disclosure of Interest</h3> None declared
Mutations in Driver Genes and Changes in Clonal Dynamics Are Associated with Shorter Time to Treatment in MBL Cases
Authors: Santiago Barrio, Juhi Ojha, Charla Secreto, Kari G. Chaffee, klaus Martin Kortum, Sachin Pathangey, Susan L. Slager, Rafael Fonseca, Neil E. Kay, Tait D. Shanafelt, Esteban Braggio
Journal: Blood (2015)
THU0538 Decreasing Uric Acid Level Is Associated with Improving Glomerular Filtration Rate in Chronic Kidney Disease Patients With/without Gout
Authors: T.-S. Lin, C.-Y. Huang, S.-C. Hsieh
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Hyperuricemia was a risk factor for hypertension, metabolic syndrome, type 2 diabetes mellitus, cardiovascular disease, and chronic kidney disease. Uric acid lowering therapy was indicated when hyperuricemic patients with established diagnosis of gout. Clinical trials have demonstrated that xanthine oxidase inhibitor decreased the progression of chronic kidney disease in gout patients. Uric acid lowering therapy was not suggested in asymptomatic hyperuricemic patients due to lack of evidence. <h3>Objectives</h3> To evaluate the association between the change of uric acid level and renal function in chronic kidney disease patients with/without gout. <h3>Methods</h3> This is a retrospective cohort study. The data source was the electronic records of National Taiwan University Hospital and its branch Hospital during 2011 to 2015. Patients were included if they have chronic kidney disease stage 2, 3, and 4 according to K-DOQI staging and receive follow-up for more than 6 months. Multiple variables linear regression models were applied for evaluating the association between the change of uric acid level and eGFR. The variables including age, gender, hypertension, type 2 diabetes mellitus, dyslipidemia, gout, and initial uric acid level were analyzed. We performed a subgroup analysis of the patients with decreasing uric acid level. <h3>Results</h3> Total 272 patients were included. There were 154 (56.6%) females and 118 (43.4%) males. The mean age was 57.9±14.7 (SD) years. The mean uric acid level was 6.5±1.9 (SD) mg/dL. The mean duration of follow-up was 128.7 ±55.3 (SD) weeks. 52 (19%) patients had the diagnosis of gout. In multivariable linear regression models, the change of eGFR was affected by the change of uric acid level (β= -0.249, <i>P</i> &lt;0.001). In the subgroup analysis of the patients with decreasing uric acid level, the change of eGFR was also affected by the change of uric acid level (β= -0.214, <i>P</i> =0.011). <h3>Conclusions</h3> The decreasing uric acid level is associated with increasing eGFR in chronic kidney disease patients even after adjusting the status of gout. The causal relationship between the uric acid level and renal function should be interpreted carefully. <h3>References</h3> Richette P, Perez-Ruiz F, Doherty M, et al. Improving cardiovascular and renal outcomes in gout: what should we target? Nature Reviews Rheumatology 2014;10(11):654–61. <h3>Disclosure of Interest</h3> None declared
No Outcome Differences Were Observed Between Supra-Diaphragmatic and Infra-Diaphragmatic Lesions in Limited-Stage Diffuse Large B Cell Lymphoma Treated with R-CHOP Therapy, Whereas the Presence of Gastro-Intestinal Lesions Was Associated with a Favorable Prognosis
Authors: Yuki Nakajima, Naoto Tomita, Megumi Itabashi, Kazuho Miyashita, Reina Watanabe, Takuya Miyazaki, Takayoshi Tachibana, Hirotaka Takasaki, Rika Kawasaki, Masatsugu Tanaka, Chizuko Hashimoto, Etsuko Yamazaki, Jun Taguchi, Katsumichi Fujimaki, Rika Sakai, Hiroyuki Fujita, Shin Fujisawa, Hiroshi Harano, Shigeki Motomura, Yoshiaki Ishigatsubo
Journal: Blood (2014)
SP0123 gout: A Variable Inflammatory Disease
Authors: Md R. Terkeltaub
Journal: Annals of the Rheumatic Diseases (2016)

In patients with tissue deposits of urate crystals, acute flares of gout develop more frequently after certain dietary triggers and in early uric acid lowering therapy (ULT). However, most acute flares of gout develop and recur variably and unpredictably. New gout inflammation biomarkers, and novel, rational, lab discovery-based therapeutics targeted to them, would advance care. Elevation of serum urate, though a biomarker for long-term prognosis in gout, has no clear role in assessing the inflammatory state in gout. Moreover, the only serum biomarker of inflammation in both acute and intercritical gout appears to be elevation of IL-8. Increasing the number of options for anti-inflammatory treatment for gout also is a central unmet need. Gouty inflammation is driven by innate immune responses to urate crystals, with "first signal" priming of the NLRP3 inflammasome in macrophage lineage cells by C5a, NF-kB transcription factor activation and a variety toll-like receptor 2 and 4 (TLR2, 4) ligands, and coupled "second signals" via NLRP3 inflammasome activation driving secretion of mature IL-1beta. Sources of variability in the capacity of urate crystals to cause inflammation will be reviewed in this talk. They include not simply exogenous dietary factors, but also constitutive "master" regulation of host inflammatory responses to urate crystals. I will discuss, in depth, AMP-activated protein kinase (AMPK), a druggable master regulator of urate crystal-induced inflammation and mediator of comorbidity. AMPK is one of several biosensors of changes in nutrition and cellular energy processes (eg, glycolysis, oxidative phosphorylation) that are emerging as therapy targets for controlling adaptive and innate immune inflammatory responses, exemplified by suppression of SLE using metformin. Last, I will summarize how metabolic regulation of innate inflammation is directly relevant to gout, since both hyperuricemia and clinical expression of gouty arthritis are influenced by highly prevalent metabolic co-morbidities and dietary factors, such as the linked co-morbidities obesity, metabolic syndrome and type II diabetes, and related caloric excesses. <h3>Disclosure of Interest</h3> R. Terkeltaub Md Consultant for: Sobi, AZ, REVIVE, Relburn
Circulating Angiogenic Cytokines Are Elevated in Patients with Smoldering Myeloma; Implications Into Disease Biology
Authors: Maria Gkotzamanidou, Evangelos Terpos, Athanasios Papatheodorou, Evangelos Eleutherakis-Papaiakovou, Meletios Athanasios Dimopoulos, Efstathios Kastritis
Journal: Blood (2011)
Effect of Nilotinib (NIL) on Molecular Response in Chronic Myelogenous Leukemia - Chronic Phase (CML-CP) Patients (pts) with a Suboptimal Molecular Response to Imatinib (IM)–ENABL Study Update
Authors: Sikander Ailawadhi, Carole B. Miller, Anand P. Jillella, Nebu Koshy, Brian Tudor, Luke P. Akard, Solveig G. Ericson, Felice P. Lin, Jerald P. Radich, Daniel J. DeAngelo
Journal: Blood (2011)
AB0487 Antiphospholipid Syndrome and Epilepsy: Where Do We Stand?
Authors: M.A. Noureldine, G. Harifi, A.A. Haydar, A. Berjawi, M. Nader, R. Elnawar, A. Sweid, J. Al Saleh, M.A. Khamashta, I. Uthman
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Epilepsy and seizures are commonly reported among the neurological manifestations of Antiphospholipid Syndrome (APS). <h3>Objectives</h3> To discuss the epidemiological, pathophysiological, laboratory, clinical, radiological, and therapeutic aspects of epilepsy/seizures in APS/ antiphospholipid antibody (aPL)-positive patients. <h3>Methods</h3> We searched the literature through Google Scholar and PubMed for all publications reporting on the epidemiology, pathogenesis, laboratory, imaging and associated clinical findings, and management of epilepsy/seizures in APS/aPL-positive patients. The following keywords were used: antiphospholipid, Hughes syndrome, anticardiolipin, lupus anticoagulant, anti-β2-glycoprotein I, partial, complex, generalized, and idiopathic epilepsy, seizure, and convulsion. All reports published between 1971 and 2015 were included. <h3>Results</h3> Temporal lobe epilepsy is particularly prominent in many APS cases. The prevalence of epilepsy/seizures in APS patients is approximately 8%, which is nearly 10 times the prevalence of epilepsy in the general population (0.5–1%). The pathogenesis is complex and may involve micro-thrombosis and/or immune-mediated neuronal damage. The association of seizures with aPL is more significant in the presence of SLE. Seizure patients with positive aPL tend to develop thrombocytopenia and livedo racemosa more frequently than those without aPL. The presence of SLE and the presence of neurological symptoms significantly correlate with the presence of white matter changes on MRI. In contrast, the correlation between aPL positivity and the presence of white matter changes is very weak. However, MRI can be normal in more than 30–40% of patients. New MRI techniques can better elucidate the pathology of brain damage in neuro-APS. The theurapeutic management of epileptic APS patients relies on anti-epileptic treatment and anticoagulant agents when there is evidence of a thrombotic event. The anti-thrombotic benefit of hydroxychloroquine and statins is supported by several studies. <h3>Conclusions</h3> MRI remains the imaging modality of choice. aPL testing is recommended in young patients presenting with atypical seizures and multiple hyper-intensity lesions on brain MRI in the absence of other possible conditions. In the absence of consensual recommendations, the decision of lifelong anticoagulation in epileptic APS patients is discussed on a case-by-case basis. <h3>Disclosure of Interest</h3> None declared
Pattern of metastasis and outcome in patients with colorectal cancer
Authors: G.G. Cardellino, M. Bonotto, K. Rihawi, L. Gerratana, M. Casagrande, L. Ferrari, E. Ongaro, D. Iacono, S.K. Garattini, V. Fanotto, E. Iaiza, P. Ermacora, M. Giovannoni, N. Pella, F. Puglisi, G. Aprile, G. Fasola
Journal: Annals of Oncology (2015)
Convergence of TGFb and IL-1 signaling pathways: Inhibition of SMAD3 transcriptional activity by TAK1: Inhibition of SMAD3 transcriptional activity by TAK1.
Authors: A.T.J. Wierenga, G.F.J.D. Benus, Jan-Jacob Schuringa, Edo Vellenga, B.J.L. Eggen
Journal: Blood (2003)
SP0023 Monogenic Lupus and Protein Kinase C Delta Deficiency
Authors: A. Belot
Journal: Annals of the Rheumatic Diseases (2016)

Next generation sequencing (NGS) represents a revolution in the field of molecular medicine, and offers a new approach to deciphering the pathogenesis of complex diseases. Paediatric-onset SLE (pSLE) is considered as a paradigm of early-onset autoimmunity and is a very rare and more severe disease than its adult-onset counterpart. These differences are possibly associated with a greater contribution of genetic aetiological factors. Others and we have identified the deficiency of Protein Kinase C-delta (PKC-delta) as a new cause of monogenic autoimmunity. To date, six patients in four kindred have been reported. The phenotype is broad and ranges from lymphoproliferative disease to lupus-like autoimmunity. In mouse, PKC-delta deficiency is associated to systemic autoimmunity and is B cell autonomous. We have shown that BCR- or Thapsigargin-dependent apoptosis is impaired in patient9s B cells. PKC-delta is crucial in regulating B cell negative selection. PKC-delta is widely expressed and mutations may impact on additional immune cells. One out of our three patients has developed late-onset papillomatosis suggestive for NK cell deficiency but the underlying mechanisms remain unclear. As described in other apoptosis deficiencies, PKC-delta deficiency may occur in a context of somatic mutation. As part of an exome sequencing panel project on SLE-related genes, we have recently screened 130 additional patients with early-onset SLE but we did not identified any homozygous or heterozygous variants suggesting <i>PRKCD</i> mutations remain extremely rare in juvenile SLE. <h3>Disclosure of Interest</h3> None declared
PWE-062 Fructose Intolerance in Pelvic Radiation Disease; Is It More Common than We Realised?
Authors: R Stacey, N Langasco, H Ludlow, J Turner, J Green
Journal: Gut (2016)

<h3>Introduction</h3> Guidelines have recently been published regarding the management of Pelvic Radiation Disease (PRD) and late effects of cancer care.<sup>1</sup> The guidelines have been shown in peer reviewed studies to be effective in the management of PRD.<sup>2</sup> The guidelines include a number of investigations for certain symptoms and include carbohydrate tolerance tests for substrates such as fructose. That said there are currently no published case series or studies evaluating the prevalence and diagnostic yield of fructose breath testing in PRD. We have recently set up a dedicated late effects of cancer care and pelvic radiation disease clinic in Cardiff and audited the results of our fructose tolerance breath tests. Patients with diarrhoea, bloating or abdominal pain were investigated with fructose breath testing as per these guidelines. <h3>Methods</h3> We audited the results for all fructose breath tests in 2014 in patients with suspected PRD via a database search and correlation with clinical notes. Total of 82 patients were identified as having a fructose breath tests in the 1 year period) 20 of which were being investigated for suspected PRD. (10 Male, 10 female, median age 65.5) <h3>Results</h3> 45% of fructose breath tests in patients with suspected PRD were positive (n = 9) All of these patients were referred with either diarrhoea, bloating or abdominal pain. Of these patients with positive tests, 44% were found to have a dual pathology (n = 4) with other diagnoses including small intestinal bacterial overgrowth, lactose intolerance and bile acid diarrhoea. <h3>Conclusion</h3> Fructose intolerance is common in patients with diarrhoea, bloating and abdominal pain in PRD. The diagnostic yield of testing is high and should be considered as a differential diagnosis in these patients. It is important to recognise that dual pathology frequently occurs and that an algorithmic approach is useful in these patients. <h3>References</h3> 1 Andreyev HJN, Muls AC, Norton C, Ralph C, Watson L, Shaw C, <i>et al</i>. Guidance: The practical management of the gastrointestinal symptoms of pelvic radiation disease. Frontline Gastroenterology [Internet]. BMJ Publishing Group Ltd; 2014 Jun 17;flgastro–2014–100468. Available from: http://fg.bmj.com/content/early/2014/06/17/flgastro-2014-100468.abstract 2 Andreyev HJN, Benton BE, Lalji A, Norton PC, Kabir M, GaugeGage PH, <i>et al</i>. ArticlesAlgorithm-based management of patients with gastrointestinal symptoms in patients after pelvic radiation treatment (ORBIT): a randomised controlled trial. Lancet. Elsevier Ltd; 2013 Sep 20;1–9. <h3>Disclosure of Interest</h3> None Declared