tbuckley/cabot-search huggingface dataset:
SAT0262 7 Cases of Chinese Juvenile Relapsing Polychondritis and Systemic Review
Authors: D.F. Lin, W.Q. Yang, P.P. Zhang, Q. Lv, O. Jin
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Relapsing polychondritis (RP) is a rare systemic autoimmune disease characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. Juvenile RP (&lt;18 years) mentioned more severe than adult as previous reports. <h3>Objectives</h3> Our work is to study the clinical and prognostic features of Juvenile Relapsing polychondritis (RP). <h3>Methods</h3> 7 RP cases from 1994–2013 in our hospital were reviewed and compared with 51 international juvenile cases and 158 Chinese adult case series reported from 1985–2013 including clinical features, systemic involvement, differential diagnosis and prognosis. <h3>Results</h3> 1)Average age at the onset was 15.1 years old, the mean age at first symptoms was 15.1 months and female-male ratio was 0.4:1, 6 of 7 had been misdiagnosed. 2)The incidences of arthritis (100%: 53%), nasal chondritis (1%: 53%), laryngotracheal syndromes (86%: 68%), auricular chondritis (86%: 67%), cardiovascular involvement (14%: 10%) and autoimmune diseases (14%: 5%) were higher than in Chinese adult except skin (20%: 46%). 3) Our juvenile RP patients had more severe in respect toocular inflammation (57%: 40–47%), arthritis (100%, 71–90%), cardiovascular (14%: 3–10%) and skin involvement (20%:10–11%) than Caucasian juvenile RP. <h3>Conclusions</h3> Our juvenile RP series were more severe than Chinese adult RP as the same as in Caucasians and has its unique characteristics in spite of sharing most clinical features with case series in literature. <h3>Disclosure of Interest</h3> None declared
Identification of New Recurrent Lesions and Clinical Subsets by Genome-Wide DNA Profiling in Chronic Lymphocytic Leukemia with 17p Deletion.
Authors: Francesco Forconi, Andrea Rinaldi, Ivo Kwee, Elisa Sozzi, Davide Rossi, Francesca Toraldo, Clara Deambrogi, Emanuele Zucca, Daniela Marconi, Riccardo Bomben, Valter Gattei, Francesco Lauria, Gianluca Gaidano, Francesco Bertoni
Journal: Blood (2007)
AB0406 Clinical Experience from Abatacept Use in Systemic Lupus Erythematosus: Effectiveness and Safety: Table 1.
Authors: B. Serrano-Benavente, N. Bello, J.C. Nieto-González, J.G. Ovalles-Bonilla, I. Janta, R. González-Benítez, C. Mata, D. Hernández-Flόrez, F.J. Lόpez-Longo, E. Naredo, C.M. González-Fernández, I. Monteagudo
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Biologicals therapies have been used for the treatment of Systemic Lupus Erythematosus (SLE) but only Belimumab has been approved by the European Medicines Agency. Abatacept (ABA), a T-cell co-stimulation blocker, has been proposed as an effective treatment for SLE due to its mechanism of action. Three randomised clinical trials have been performed with ABA in patients with SLE. Although any of these trials met the primary outcome of efficacy, it is still considered as an "off-label" option when none of the other conventional therapies have worked. <h3>Objectives</h3> To assess effectiveness and safety of ABA in patients diagnosed of SLE in a Rheumatology service from a tertiary care hospital in Madrid, Spain. <h3>Methods</h3> We performed an observational retrospective study in patients with SLE (according to the 1997 American College of Rheumatology classification criteria) who received treatment with ABA. We recorded demographic, clinical and laboratory parameters at the beginning of treatment, and after 6 and 12 months of treatment. We also recorded adverse events. <h3>Results</h3> We included 8 patients, all of them women. The median age was 45.13 years (range 30–67 years) and the median time from SLE diagnosis to ABA treatment was 12.12 years (range 3–21 years). Three patients had an overlap disease with Rheumatoid Arthritis. The most frequent symptoms were arthritis (n=5), cutaneous (n=3) and neurological symptoms (n=5), specifically organic brain syndrome (n=4) and lupus headache (n=3). None of the patients had renal disease. In all patients ABA was use after anti-TNFα or Rituximab treatment. One patient discontinued ABA after 2 months due to inefficacy, so she was excluded from statistical analysis. Table 1 shows clinical and laboratory parameters at the beginning of treatment and after 6 and 12 months of treatment. After 6 months of treatment, disease activity according to SLEDAI decreased. One patient persisted with organic cerebral syndrome. After 12 months of treatment neurological symptoms showed a substantial improvement in every patient that presented them at the beginning. Four patients showed significant improvement of arthritis. Two patients experienced serious infections that required hospitalization. <h3>Conclusions</h3> ABA was effective in our SLE patients. Clinical response was evident at 6 months and persisted at 12 months. ABA may be a therapeutic option in SLE patients with inadequate response to conventional therapies, specially if arthritis or neurological symptoms are present. <h3>References</h3> Aringer M. Expert Opin Drug Saf. 2015;14:243–51. Ikeda K. Clin Dev Immunol. 2013;2013:697525. <h3>Disclosure of Interest</h3> None declared
AML Cells Have Increased Mitochondrial Mass but Less Reserve in Their Respiratory Chain Complexes Leading to Heightened Sensitivity to Inhibition of Mitochondrial Protein Translation,
Authors: Shrivani Sriskanthadevan, Skrtic Marko, Bozhena Livak, Yulia Jitkova, Rose Hurren, Marcela Gronda, Rob Laister, Peter Mullen, Linda Penn, Mark D. Minden, Aaron D Schimmer
Journal: Blood (2011)
We agree and we disagree, which is exactly what most people do most of the time
Authors: Bert H. Hodges, Dominic J. Packer
Journal: Behavioral and Brain Sciences (2016)

Abstract Humans are continually diverging and converging with respect to each other. Research across many domains suggests that differentiation and integration are aspects of a more complex set of dynamics, and are not step-wise but interdependent and continuous. Research on conformity in particular reveals that divergence and dissent are forms of cooperation, reflecting concerns for both individual and group integrity.
The Thrombospondin-1 N700S Polymorphism Does Not Alter von Willebrand Factor Multimer Size in Patients Suffering an Acute Myocardial Infarction.
Authors: Jeffrey Zwicker, Flora Peyvandi, Roberta Palla, Rosanna Lombardi, Maria Teresa Canciani, Kenneth Bauer, Jack Lawler, P.M. Mannucci
Journal: Blood (2005)
Pentostatin, Cyclophosphamide, and Rituximab (PCR) Has Comparable Activity but Appears To Be Better Tolerated Than Fludarabine, Cyclophosphamide, and Rituximab (FCR) in Patients with Previously Treated Chronic Lymphocytic Leukemia.
Authors: Nicole Lamanna, Matt Kalaycio, Peter Maslak, Mark L. Heaney, Renier Brentjens, Joseph Jurcic, Emily Biederman, Alison N. Gencarelli, Katherine S. Panageas, David A. Scheinberg, Mark A. Weiss
Journal: Blood (2005)
FRI0249 Risk Factors for Development of Pulmonary Arterial Hypertension in Australian Scleroderma Patients
Authors: K. Morrisroe, M. Huq, W. Stevens, C. Rabusa, S. Proudman, M. Nikpour
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Pulmonary arterial hypertension (PAH) is the leading cause of mortality in patients with scleroderma (SSc). <h3>Objectives</h3> To determine the incidence, prevalence and risk factors for PAH development in a large Australian SSc cohort. <h3>Methods</h3> PAH was diagnosed on right heart catheterization (mPAP &gt;25 and PAWP &lt;15 mmHg at rest). Patients with PH secondary to interstitial lung disease (ILD; defined as abnormal HRCT scan and FVC&lt;60%) were excluded. Summary statistics, chi-square tests, univariate and multivariable logistic regression along with post-estimation diagnostics were used to determine the associations of different combinations of risk factors with PAH. <h3>Results</h3> Among 1579 SSc patients, 8.4% (132 patients) were diagnosed with PAH over a mean (±SD) follow-up of 3.2 (±2.5) years. The incidence of PAH in this cohort was 0.7% per annum. Of these, 68.9% had limited disease subtype (lcSSc). In multivariable regression analysis, the presence of anti-centromere antibody (ACA) (OR 1.6, 95%CI 1.1–2.5, p=0.03), oesphageal stricture (OR 2.0, 95%CI 1.2–3.3, p=0.006), calcinosis (OR 1.9, 95%CI 1.2–2.9, p=0.003), sicca symptoms (OR 1.6, 95%CI 1.1–2.5, p=0.03), mild ILD (OR 2.3, 95%CI 1.5–3.7, p&lt;0.001) and digital ulcers (OR 1.6, 95%CI 1.0–2.4, p=0.03) were predictive of PAH. This model had an area under the curve of 0.7 and concordance of 91.8%. Risk factors for PAH were were analyzed by disease subtype and are presented in Table 1 and 2. <h3>Conclusions</h3> The incidence and prevalence of PAH in this cohort are 0.7% per annum and 8.4%, respectively. The clinical-serologic risk factors for PAH differ based on disease subtype. In both subtypes, mild ILD is associated with PAH, suggesting the possibility of a common pathogenic mechanisms underlying both of these disease manifestations. This model identifies a subset of patients at an appreciably higher risk of developing PAH, who should be screened and would in future, benefit from preventative therapies. <h3>Acknowledgement</h3> This work was supported by Scleroderma Australia, Arthritis Australia, Actelion Australia, Bayer, CSL Biotherapies, GlaxoSmithKline Australia, Pfizer and BMS. Dr Morrisroe is a recipient of an NHMRC and RACP Shields Fellowship. Dr Nikpour holds a University of Melbourne Faculty of Medicine Dentistry and Health Science David Bickart Clinician Researcher Fellowship and is a recipient of an NHMRC Fellowship (APP1071735). <h3>Disclosure of Interest</h3> None declared
The effect of different durations of remission on damage accrual: results from a prospective monocentric cohort of Caucasian patients
Authors: Margherita Zen, Luca Iaccarino, Mariele Gatto, Silvano Bettio, Francesca Saccon, Anna Ghirardello, Leonardo Punzi, Andrea Doria
Journal: Annals of the Rheumatic Diseases (2016)

To identify the shortest duration of remission associated with improved outcomes in systemic lupus erythematosus (SLE).We studied 293 Caucasian patients with SLE during 7-year follow-up. Disease activity was assessed by SLE Disease Activity Index 2000 and damage by Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). We defined three remission levels: complete, clinical off-corticosteroids, clinical on-corticosteroids (prednisone 1-5 mg/day). The effect of different durations of remission (1, 2, 3, 4 and ≥5 consecutive years) on damage was evaluated by multivariate logistic regression analysis.Among patients achieving 1-year (27 patients), 2-year (47 patients), 3-year (45 patients), 4-year (26 patients) remission, damage was similar irrespective of the level of remission achieved, whereas, among patients achieving ≥5-year remission (113 patients), damage was higher in those in clinical remission on-corticosteroids (p<0.001).In multivariate analysis, ≥2 consecutive year remission was protective against damage (OR (95% CI)): 2 years 0.228 (0.061 to 0.850); 3 years 0.116 (0.031 to 0.436); 4 years 0.118 (0.027 to 0.519) and ≥5 years 0.044 (0.012 to 0.159). Predictors of damage were cumulative prednisone dose ≥180 mg/month (3.136 (1.276 to 7.707)), antiphospholipid antibody syndrome (5.517 (2.092 to 14.546)), vasculitis (3.107 (1.030 to 9.307)) and number of flare/year (8.769 (1.692 to 45.449)).Two consecutive years is the shortest duration of remission associated with a decrease in damage progression in Caucasian patients with SLE.
Value of Early Evaluation with FDG-PET for Follicular Lymphoma.
Authors: Remy R. Gressin, Jean Philippe J.P. Vuilliez, Lysiane L. Molina, Brigitte B. Pegourie, Stephane S. Courby, Frederic F. Garban, Claude Eric C.E. Bulabois, Frederique F. Orsini, Emmanuelle E. Nicolas, Caroline C. Makowski, Jean Jacques J.J. Sotto, Jean Yves J.Y. Cahn
Journal: Blood (2005)
Expression of cyclooxygenase-2 (COX-2) and peroxisome proliferator-activated receptor gamma (PPARγ) in skin tumors
Authors: Stefanie Meyer, Markus Ameres, Michael Landthaler, Frauke Bataille, Matthias Woenckhaus, Albrecht Reichle, Arndt Hartmann, Peter Johannes Wild
Journal: Cancer Research (2007)

AACR Annual Meeting-- Apr 14-18, 2007; Los Angeles, CA

175 

Background: To investigate whether protein expression of cyclooxygenase-2 (COX-2) and peroxisome proliferator-activated receptor gamma (PPARγ) is associated with clinico-pathologic characteristics in benign and malignant skin tumors.

Methods: Two tissue microarrays (TMAs) were used to analyze COX-2, PPARγ, TP53, and the Ki67 labeling index immunohistochemically. TMA-1 (n=323) consisted of malignant melanomas, nevi, squamous cell carcinomas, basal cell carcinomas, Kaposi sarcomas, histiocytomas, capillary hemangiomas, and sebaceous adenomas. TMA-2 consisted of 88 malignant melanomas with follow-up data, 101 melanoma metastases and 161 benign nevi. Cytoplasmic COX-2 and nuclear PPARγ expression was scored semiquantitatively (0-3+).

Results: Using TMA-1, COX-2 and PPARγ expression of any intensity was detected in 82% (152/186) and 33% (69/212) of any analyzable skin neoplasm, respectively. As regards TMA-2, COX-2 immunoreactivity significantly increased from benign nevi (51%) to primary melanomas (86%) to melanoma metastases (91%; P <0.001). In case of primary melanomas, positive COX-2 staining was associated with advanced Clark levels ( P =0.004) and shorter recurrence free survival ( P =0.0271). COX-2 expression was not associated with overall survival. Accordingly, PPARγ immunoreactivity was significantly increasing from benign nevi (0%) to malignant melanomas (22%) and melanoma metastases (33%; P <0.001). However, PPARγ expression in melanomas was not associated with any of the clinico-pathologic characteristics, including overall survival and tumor recurrence.

Conclusions: Expression of the enzyme COX-2 and the transcription factor PPARγ is a common phenomenon during the metastatic process of malignant melanoma, representing possible targets for an anti-inflammatory and anti-proliferative therapy.
OP0106 Change in Bone Mineral Density with High-Dose Prednisone in Patients with Rheumatoid Arthritis: Table 1.
Authors: L. Rasch, L. van Tuyl, M. Kremer, I. Bultink, M. Boers, W. Lems
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> Recently, we showed that treatment with COBRA-light therapy including prednisone with initially 30 mg/day, was as effective as the original COBRA scheme, with initially 60 mg/day [1,2], in the treatment of rheumatoid arthritis (RA). Since high-dose glucocorticoids are associated with bone loss and vertebral and nonvertebral fractures, we investigated the differences in bone mineral density (BMD) after one year of treatment in both arms. <h3>Objectives</h3> To compare 1-year changes in BMD (lumbar spine [L1-L4], total hip, and femoral neck) between the treatment groups. <h3>Methods</h3> An open-label, randomised controlled, non-inferiority trial of patients with active, newly diagnosed RA following a treat-to-target protocol. <h3>Results</h3> BMD data were determined in 144 out of 164 included RA patients, all randomized to either COBRA (n=71) or COBRA-light (n=73) therapy. Overall bone loss was very limited, and no significant difference in change in BMD between COBRA and COBRA-light was found at any site (<i>Table 1</i>). However, in secondary analyses, COBRA-light showed a significant decrease in BMD in the lumbar spine and total hip after 52 weeks, whereas the femoral neck and the COBRA group did not. <h3>Conclusions</h3> During the trial, overall bone loss was very limited and not significantly different between the treatments. These findings strengthen the hypothesis that positive effects associated with the large reduction in disease activity as a result of combination therapy and tight control treatment counteract the negative effects of (high-dose) prednisone on bone. <h3>References</h3> Den Uyl D, et al. Ann Rheum Dis 2014; Ter Wee MM, et al. Ann Rheum Dis 2015 <h3>Disclosure of Interest</h3> L. Rasch: None declared, L. van Tuyl: None declared, M. Kremer: None declared, I. Bultink: None declared, M. Boers: None declared, W. Lems Grant/research support from: This research was performed within the framework of project T1–106 of the Dutch Top Institute Pharma, and was additionally funded by an unrestricted grant from Pfizer.
SP0178 Transition Care Programmes: A European Perspective
Authors: K. Minden
Journal: Annals of the Rheumatic Diseases (2016)

The special health care needs of adolescents and young adults with chronic diseases and the inadequate state of services for them have been on the agenda for almost 30 years. But still too many young people, including those with inflmmatory rheumatic diseases, do not receive the care they expect and need. In fact, approximately half of young people are not satisfied with the experienced mode of transition. One in two wishes a better preparation for adult health care. For one third the transfer to adult care comes too early, unplanned or abruptly. Up to half of young people with rheumatic diseases do not make a successful transfer to adult rheumatology and are therefore at particular risk of unfavorable outcome. In response to this, there have been a number of guidelines and activities across Europe seeking to reduce the risks associated with moving to adult services and to implement developmentally focused adolescent care as normal part of a health-care plan. This lecture will describe European initiatives, which aim at improving health care transition services, such as specific and generic transition care programmes or checklists, education programmes, recommended frameworks and pathways to implement and assess transition programmes. <h3>Disclosure of Interest</h3> K. Minden Grant/research support from: Pfizer, Abbvie
Sustained Superior Long-Term Outcomes of Imatinib Therapy In Japanese Patients with Newly Diagnosed Chronic Myelogenous Leukemia In Chronic Phase: Sub-Analysis According to the Mean Daily Dose of Imatinib and the Plasma Trough Levels In JALSG CML202 Study After 66 Months Follow-up.
Authors: Chiaki Nakaseko, Kazunori Ohnishi, Jin Takeuchi, Shin Fujisawa, Tadashi Nagai, Shigeki Ohtake, Hirohito Yamazaki, Tetsuzo Tauchi, Kiyotoshi Imai, Naoki Mori, Fumiharu Yagasaki, Hideki Akiyama, Yasuhiro Maeda, Noriko Usui, Yoshio Saburi, Takashi Ishida, Hiroshi Kosugi, Koichi Miyamura, Yasushi Miyazaki, Hiroshi Handa, Akira Yokota, Kenji Matsumoto, Katsumichi Fujimaki, Toshimitsu Matsui, Hitoshi Kiyoi, Shuichi Miyawaki, Ryuzo Ohno, Tomoki Naoe
Journal: Blood (2010)
SP0045 Assessing Hand Osteoarthritis in Clinical Care and Research
Authors: C.H.E. Van Den Ende
Journal: Annals of the Rheumatic Diseases (2016)

Assessment of hand osteoarthritis (OA) is a challenge due to the involvement of multiple joints in the disease process and the complex interplay between thumb, fingers, wrists and hands. Symptoms of hand OA, commonly addressed by non-pharmacological interventions are pain, stiffness, reduced joint mobility, instability and reduced muscle function. Treatment of hand OA focuses on the relief of symptoms, improvement of dexterity, and the improvement of hand activities. In clinical research standardization of assessment is necessary to estimate the clinical burden of the disease and to evaluate the efficacy of (novel) treatment modalities. The OMERACT (Outcome Measures in Rheumatology) identified four core domains for clinical research in OA: pain, physical function and patient global assessment for symptom modifying trials and, in addition, imaging for structure modifying trials. Recently, a working group of the OMERACT proposed a preliminary set of core (sub) domains with preliminary instruments.<sup>1</sup> In clinical practice assessment of hand OA guides setting of treatment goals and the treatment plan. In addition, structured assessment of OA can be used to monitor progress and to evaluate treatment results. In general, structured assessment in clinical practice comprises a combination of self-reported, objective and performance-based measures. The choice for a specific measurement instrument should be based on the purpose of assessment (for instance to provide information about possible targets for treatment) and on the psychometric qualities of measurement instruments. An overview of measures to assess hand OA in clinical practice and research will be presented and discussed. <h3>References</h3> Kloppenburg et al, J of Rheumatol 2015;42:2190–7 <h3>Disclosure of Interest</h3> None declared
SAT0201 Dermatomyositis and Pregnancy: Assessment of Disease Activity and Pregnancy Outcomes Complicated by Maternal Dermatomyositis
Authors: G.D. Miller, E. Moore, A. Valenzuela, L. Chung, V. Werth
Journal: Annals of the Rheumatic Diseases (2016)

<h3>Background</h3> The effect of pregnancy on Dermatomyositis (DM) disease activity and on maternal and fetal outcomes in patients with DM is unclear<sup>1,2</sup>. <h3>Objectives</h3> Evaluate disease activity and pregnancy outcomes complicated by maternal dermatomyositis. <h3>Methods</h3> A total of 10 patients with 21 pregnancies from a cohort of 438 DM patients of child-bearing potential, diagnosed between 2006 and 2015 from two centers, were eligible for interview with a specific questionnaire regarding pregnancy and fetal outcomes. <h3>Results</h3> Weakness, rash and arthritis were the most common clinical symptoms that improved during pregnancy in affected patients (Figure 1). In 16 out of 18 pregnancies, patients experienced weakness. Weakness improved during 13 pregnancies (81.3%), stayed the same during 2 pregnancies (12.5%), and worsened during one (6.3%). All 10 patients experienced cutaneous rash during all 18 pregnancies carried to live birth. Cutaneous rash improved during 13 pregnancies (72.2%), remain unchanged during 3 (16.7%), and worsened during 2 (11.1%). Five patients experienced arthralgias or arthritis in a total of 11 pregnancies. Symptoms improved during 10 of these pregnancies (91%) and worsened during one (9%).There were no cases of interstitial lung disease, cancer or antiphospholipid antibody syndrome among the 10 patients studied. One patient suffered from pre-eclampsia while diagnosed with DM. 3 total pregnancy losses: two early miscarriages (&lt;10 weeks gestation) one therapeutic abortion for an ectopic pregnancy. 3 neonates out of 18 live births went to the NICU after delivery – reasons for transfer: meconium aspiration; apnea and pre-term birth. <h3>Conclusions</h3> Pregnancy does not appear to carry a worse prognosis for DM patients and all but one of our patients experienced improvement in clinical symptoms during pregnancy. <h3>References</h3> Iago PF et al. Pregnancy in adult-onset idiopathic inflammatory myopathy: Report from a cohort of myositis patients from a single center. Semin Arthritis Rheum, 2014. 44 (2):p.234–240. Oddis, C.V., et al., Incidence of polymyositis-dermatomyositis: a 20-year study of hospital diagnosed cases in Allegheny County, PA 1963–1982. J Rheumatol, 1990. 17(10): p. 1329–34. <h3>Disclosure of Interest</h3> None declared