tbuckley/cabot-search huggingface dataset:
Assessment of Diagnostic Strategy for Early Recognition of Bullous and Nonbullous Variants of Pemphigoid
Authors: Joost M. Meijer, Gilles F. H. Diercks, Emma W. G. de Lang, Hendri H. Pas, Marcel F. Jonkman
Journal: JAMA Dermatology (2019)

A substantial number of patients with bullous pemphigoid do not develop skin blisters and may not have received the correct diagnosis. Diagnostic criteria and an optimal diagnostic strategy are needed for early recognition and trials.To assess the minimal requirements for diagnosis of bullous and nonbullous forms of pemphigoid and to evaluate the optimal diagnostic strategy.This paired, multivariable, diagnostic accuracy study analyzed data from 1125 consecutive patients with suspected pemphigoid who were referred to the Groningen Center for Blistering Diseases from secondary and tertiary care hospitals throughout the Netherlands. Eligible participants were patients with paired data on at least (1) a skin biopsy specimen for the direct immunofluorescence (DIF) microscopy test; (2) indirect immunofluorescence on a human salt-split skin substrate (IIF SSS) test; and (3) 1 or more routine immunoserologic tests administered between January 1, 2002, and May 1, 2015. Samples were taken from patients at the time of first diagnosis, before introduction of immunosuppressive therapy, and within an inclusion window of a maximum of 4 weeks. Data analysis was conducted from October 1, 2015, to December 1, 2017.Pairwise DIF, IIF SSS, IIF on monkey esophagus, BP180 and BP230 enzyme-linked immunosorbent assays, and immunoblot for BP180 and BP230 tests were performed. The results were reported in accordance with 2015 version of the Standards for Reporting Diagnostic Accuracy.Of the 1125 patients analyzed, 653 (58.0%) were women and 472 (42.0%) were men, with a mean (SD) age of 63.2 (19.9) years. In total, 343 participants received a pemphigoid diagnosis, with 782 controls. Of the 343 patients, 74 (21.6%, or 1 in 5) presented with nonbullous pemphigoid. The DIF microscopy was the most sensitive diagnostic test (88.3% [n = 303]; 95% CI, 84.5%-91.3%), whereas IIF SSS was less sensitive (77.0% [n = 263]; 95% CI, 72.2%-81.1%) but was highly specific (99.9%; 95% CI, 99.3%-100%) and complemented most cases with negative DIF findings. Results of the BP180 NC16A enzyme-linked immunosorbent assay did not add diagnostic value for initial diagnosis in multivariable logistic regression analysis of combined tests. These findings lead to the proposed minimal criteria for diagnosing pemphigoid: (1) pruritus and/or predominant cutaneous blisters, (2) linear IgG and/or C3c deposits (in an n-serrated pattern) by DIF on a skin biopsy specimen, and (3) positive epidermal side staining of IgG by IIF SSS on a serum sample; this proposal extends bullous pemphigoid with the unrecognized nonbullous form.Both DIF and IIF SSS tests should be performed for diagnosis of the bullous and nonbullous variants of pemphigoid, and the BP180 NC16A enzyme-linked immunosorbent assay is recommended as an add-on test for disease activity monitoring.
SP0017 Bits and bytes: fitting medical information – picaso the platform for improved personal, coordinated care
Authors: J. Richter
Journal: Annals of the Rheumatic Diseases (2018)

<h3></h3> Coordination of care plans between healthcare sectors and efficient management of patients with co-morbidities is of large demand. Rheumatoid arthritis (RA) patients are at increased risk of cardiovascular diseases. Different stakeholders are potentially involved in the EULAR recommended management processes. Optimised orchestration of accumulated information is of major importance to ensure data quality, meaningful management processes and cost effectiveness. A newly developed information and communications technology platform within the Horizon2020-funded PICASO-project (www.picaso-project.eu) will support a continuum of care from hospitals and outpatient clinics to the home. The PICASO platform will be developed and trialled with patients and clinicians. First experiences will be reported. The platform will become available for RA-patients in routine care but also for wider applicability in Rheumatology and other chronic diseases. <h3>Acknowledgement</h3> <i>This project received funding from the European Union’s Horizon 2020 research and innovation programme under grant agreement No 6 89 209</i> <h3>Disclosure of Interest</h3> None declared
AB1358 Infectious antibodies repertoire in rheumatoid arthritis
Authors: A. Schmiedl, S. Reuter, A. Lerner, S. Brunner, A. Schwarting, T. Matthias
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> The incidence of infectious diseases in the RA ADAPTHERA study by ELISA antibody screening and the differences in infectious distribution in active or low active disease patients was explored. <h3>Methods</h3> Sera from 88 naive RA patients out of the ADAPTHERA study cohort, disease duration &lt;6 months, were tested for antibody titers against: Herpes simplex virus 1 and 2 (HSV1 +2, IgG and IgM) Helicobacter pylori (HP, IgA and IgG), Cytomegalovirus (CMV, IgG and IgM), Toxoplasma gondii (Toxo, IgG), Adenovirus (IgG and IgM), Epstein Barr virus (EBV, IgG and IgM), and Parvovirus B19 (P-B19, IgG) were determined by using NovaLisa from NovaTec Immundiagnostica GmbH, GERMANY. Borrelia (IgG and IgM) titers were determined by <i>AESKULISA</i> and confirmed by Western blot (<i>AESKUBLOTS</i>) by AESKU.DIAGNOSTICS GmbH and Co. KG, GERMANY. <h3>Results</h3> 82% RA patients were found to be positive for HSV1 +2 IgG (2% IgM positive), 8% for Adenovirus (IgA), 77% (IgG), and 1% (IgM). 99% for EBV-IgG (no IgM positive). 53% (IgG), and 26% (IgM) for CMV. 38% for HP-IgG and 15% for IgA and 79% for P-B19-IgG (3% IgM). 6% for Borrelia-IgM and 14% for IgG. A slightly increase was found for EBV sera positivity (99% IgG), compared to the normal population. <h3>Conclusions</h3> Limited evidence exists regarding the impact of the disease activity on the susceptibility for infections, possibly due to the close association of RA disease activity and therapy dependent dosage of immunosuppressive treatment. Still, some infections may present memory contact, presenting an epiphenomenon. On the contrary, they might play an active role in RA pathophysiology. <h3>Disclosure of Interest</h3> None declared
Clinical Outcomes and Molecular Responses in Children with Langerhans Cell Histiocytosis Treated with MAPK Pathway Inhibitors
Authors: Olive S. Eckstein, Daniel Zinn, Carolyn Fein Levy, Michael M. Henry, Patrick Campbell, Michael B. Jordan, Ashish R Kumar, Carlos Rodriguez-Galindo, Nobuko Hijiya, Oussama Abla, Kenneth L. McClain, Carl E. Allen
Journal: Blood (2018)
Genomic Characterization Provides Diagnostic Clarity in Cases with Overlapping Bone Marrow Fibrosis and Monocytosis
Authors: Andrew T Kuykendall, Chetasi Talati, Ling Zhang, Najla Al Ali, Kendra L. Sweet, David A Sallman, Jeffrey E. Lancet, Alan F. List, Eric Padron, Rami S. Komrokji
Journal: Blood (2018)
SAT0279 Comparison of retention rates between tumour necrosis factor-Α inhibitors in ankylosing spondylitis patients: data from the korean college of rheumatology biologics registry
Authors: E.H. Park, S. Oh, H.-A. Kim, Y.-B. Park, K. Shin
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Drug persistence of tumour necrosis factor-a inhibitors (TNFi) tends to be higher in patients with ankylosing spondylitis (AS) than rheumatoid arthritis but there are few studies of Asian AS patients in the literature. <h3>Objectives</h3> To investigate drug retention rates of various TNFi used in Korean AS patients. <h3>Methods</h3> Subjects were AS patients enrolled in the Korean College of Rheumatology Biologics registry<sup>KOBIO, Dec 2012</sup> ~). All approved and commonly prescribed TNFi were included in the analysis. Discontinuation was defined as switching or stopping the biologic agent. Kaplan-Meier curve and Cox proportional hazard model were used for further analysis. Reason of TNFi discontinuation was also assessed. Univariate and multivariate analyses were used to identify possible predictors of discontinuation. <h3>Results</h3> Data of total of 1005 AS patients were analysed (median follow-up period: 14 months). The mean age of patients was 40.7, and 77.4% were males. The mean disease duration was 7.1 years, HLA-B27 were positive in 82.4%, and 33.2% of patients had lesion(s) of syndesmophytes. Seventy-six percent of patients were first-time biologic users. Discontinuation of TNFi occurred in 24.2% (switching in 9.6%) of patients during follow-up. The drug survival function estimate showed that the adjusted hazard ratio (HR) of golimumab (compared with etancercept) was 0.441 (95% CI 0.277–0.703, p<i>&lt;</i>0.001). The reason of discontinuation was inefficacy (32.6%), adverse events (23.6%), clinical improvement (11.2%), and others (32.6%). A multivariate analysis indicated predictors of discontinuation to be shorter disease duration (HR 0.973, p<i>=</i>0.044), and negative HLA-B27 (HR 1.623, p<i>=</i>0.0093). <h3>Conclusions</h3> Our study demonstrates that few AS patients switched to other TNFi during their course of treatment. The drug retention rate of golimumab was higher compared with other agents prescribed in Korean AS patients. <h3>Disclosure of Interest</h3> None declared
GDF15 and Erythroferrone Mark Erythropoietic Response to ACE-011 (Sotatercept) in Thalassemia
Authors: Maciej W Garbowski, Olivier Hermine, Maria Domenica Cappellini, Raffaella Origa, Gian Luca Forni, Ersi Voskaridou, Frédéric Galactéros, Ali Taher, Jean Benoît Arlet, Jean-Antoine Ribeil, Giovanna Graziadei, Chantal Brouzes, Michaela Semeraro, Patricia Evans, Abderrahmane Laadem, Dimana Miteva, Jun Zou, Tatiana Zinger, Martin Schwickart, Victoria Sung, John Porter
Journal: Blood (2018)
A multicentre, randomized, split face and/or neck comparison of 308‐nm excimer laser and 0·1% tacrolimus ointment for stable vitiligo plus intramuscular slow‐releasing betamethasone for active vitiligo
Authors: Y. Wu, Y. Sun, L. Qiu, H. Wang, Y. Zhang, K. Chen, H. Li, H. Gu, J. Zhang, X.‐H. Gao
Journal: British Journal of Dermatology (2019)

Linked Comment:Silpa‐Archa et al. Br J Dermatol 2019; 181:21–22.
SAT0494 Clinical and electrophysiological diagnosis of symmetrical polyneuropathy in systemic sclerosis: study from a single tertiary centre in malaysia
Authors: T. Balaikerisnan, K.J. Goh, L.P. Ramanaidu, J. Raja
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Peripheral neuropathy (PN) in systemic sclerosis (SSc) is an under recognised non-lethal burden with its prevalence between 0.01% to 28%<sup>1</sup>. Previous studies have been limited by small sample size, variable diagnostic criteria and different populations studied. <h3>Objectives</h3> The aim of this study is to determine the prevalence of symmetrical PN in SSc patients and to identify the associated factors that can predispose to PN in SSc. <h3>Methods</h3> 59 SSc patients from University Malaya Medical Centre participated in this cross-sectional study. Clinical symptoms/signs of PN were assessed using modified Total Neuropathy Score (TNS). Nerve conduction studies (NCS) were carried out on the upper and lower limbs. Diagnosis of symmetrical polyneuropathy was defined as combined TNS score ≥2 and abnormal NCS parameters in at least 2 nerves including the sural<sup>2</sup>. Focal neuropathy was defined as abnormal NCS of a nerve other than the sural nerve (radial, median, ulnar, common peroneal). <h3>Results</h3> Majority were females (54, 91.5%) and had limited cutaneous SSc (44,74.6%). Mean age was 55.7 (SD ±13.1) years while mean duration of disease (non-Raynaud's disease onset) was 8.74 years (SD ±8.09) (range 1 to 44 years). Out of 59 patients, 38 (64.4%) had TNS≥2. On NCS, 17 (31.5%) and 12 (22.2%) had findings of symmetrical polyneuropathy and focal neuropathy respectively. A total of 14 (23.7%) SSc patients were diagnosed to have symmetrical polyneuropathy. Lower haemoglobin level was significantly associated with symmetrical polyneuropathy (p=0.047) after adjustment for potential confounding variables. Serum vitamin B12 was normal in all subjects with symmetrical polyneuropathy. No correlation was seen in SSc related comorbidities (diabetes mellitus and kidney disease), serum fasting blood sugar, creatinine and MCV, as well as disease markers such as skin fibrosis (MRSS skin score), specific organ manifestations, Raynaud's or vasculopathy, SSc specific auto-antibodies and treatment received. <h3>Conclusions</h3> The prevalence of PN in a Malaysian SSc cohort is similar to other studies (23.7%). Lower haemoglobin level was significantly associated with symmetrical polyneuropathy in SSc. <h3>References</h3> [1] Paik JJ, et al. Symptomatic and Electrodiagnostic Features of Peripheral Neuropathy in Scleroderma. Arthritis Care Res (Hoboken)2016;6(8):1150–57. [2] England JD, et al. Distal symmetrical polyneuropathy: a definition for clinical research. A report of the American Academy of Neurology, the American Association of Electrodiagnostic Medicine, and the American Academy of Physical Medicine and Rehabilitation. Arch Phys Med Rehabil2005Jan;86(1):167–74. <h3>Disclosure of Interest</h3> None declared
OP0333 Survival in anca associated vasculitides: a retrospective multicentric analysis in northern italy
Authors: M. Felicetti, S. Monti, M. Bond, A. Berti, R. Padoan, G. Paolazzi, G. Brunori, L. Quartuccio, R. Caporali, F. Schiavon
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Patients affected by ANCA associated vasculitides (AAV) show lower survival than general population, even if the mortality decreased significantly in the last decade. <h3>Objectives</h3> Aim of our study is to analyse the early mortality (within 6 months) and the long-term survival in a multicentric Italian cohort of AAV patients. <h3>Methods</h3> we identified all patients affected by AAV, diagnosed from 1995 until 2017, followed routinely in four vasculitis referral centres in Norther Italy. We enrolled patients with diagnosis of granulomatosis with polyangiitis (GPA) and micro polyangiitis (MPA), fulfilling EMA algorithm or <sup><sup>CHCC 2012</sup></sup> definitions, with complete survival data at last follow up. The analysis focused on early mortality, long-term survival and their predictors. <h3>Results</h3> We enrolled 200 AAV patients (F/M 110/90, Caucasian 98%) with a median age at diagnosis of 54.6±15.2 years. 157 (78.5%) were affected by GPA and 43 (21.5%) by MPA. Data about ANCA antibodies were available in 181 patients and 157 (87%) resulted ANCA positive: 100 c-ANCA/PR3, 56 p-ANCA/MPO and one with double specificity PR3-MPO-ANCA. During the follow up period [53<sup><sup>28–100</sup></sup> months], we registered 21 (10.5%) deaths, 6 (28.5% of all mortality) within 6 months after diagnosis: 9 patients died due to infectious complications, 1 due to hepatic cancer, 1 due to end stage heart failure, 1 due to massive cholestasis and 9 due to unknown causes. Early mortality was significantly associated with a higher frequency of alveolar haemorrhage (p=0.01; OR 11.1 IC95% 2.1–60.1) and respiratory failure (p&lt;0.001, OR 28.3 95% CI: 4.7 to 170.6). The long-term survival, analysed with Kaplan-Maier method, did not show significant differences between GPA and MPA patients, while a significant poorer survival was observed in p-ANCA/MPO patients than c-ANCA-PR3 and ANCA negative patients (Log rank test: p=0.04). At univariate analysis of baseline data, deceased patients resulted older at disease onset (p=0.001) with more comorbidities (p&lt;0.001) and presented at diagnosis a higher frequency of respiratory failure (p=0.002, OR 7.1 IC95% 2.2–22.2) and renal insufficiency (p=0.003, OR 4.7 95% CI: 1.6 to 13.7). No significant differences were noted in term of infections/year, relapses/year and cancer development. <h3>Conclusions</h3> In this large cohort of Italian patients we confirm a higher short and long-term survival in AAV patient than reported in literature. Nevertheless, up to one third of deaths occurred within 6 months after diagnosis and infection diseases resulted the most frequent cause of death. Moreover, our data confirm the prognostic importance of ANCA pattern and the poor outcome of patient with severe lung and renal involvement. <h3>Disclosure of Interest</h3> None declared
AB1134 Determining factors of severity in hemophilic arthropathy
Authors: C. Aguilera Cros, L. Méndez Díaz, M. Arcila Durán, A. Ruíz Román, M.J. Valenzuela Porcel, N. Garrido Puñal, J.P. Sanchez Serrano, R. Nuñez Vazquez, J. Povedano Gómez
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> In patients with haemophilia, the development of inhibitors to factor VIII/IX (Haemophilia A and B, HA/HB), prevents adequate replacement therapy and results in increased risk of serious bleeding episodes, poor control of joint bleeding, and progressive, debilitating joint disease. <h3>Objectives</h3> To describe, according to the Arnold-Hilgartner scale (AHRS), the radiological findings in a cohort of hemophilic arthropathy (HArth) patients and to analyse the relationship that may exist between the degree of joint involvement and HA/HB, severity, age and presence or absence of inhibitor. <h3>Methods</h3> This is a retrospective study, carried out in the Haemophilia Unit of our hospital, in patients with HArth (2007–2017). Severity of haemophilia: percentage of coagulation factor (CF) activity, moderate (1%–5%) and severe &lt;1%. The AHRS includes 5 levels. For the study of the association of the type and degree of haemophilia; and the presence of inhibitor, with the level in the AHRS, a linear model was used. The significance in this regard of the linear contrasts of interest was studied using F tests, defining the first species error at 0.05. <h3>Results</h3> We included 88 patients. Characteristics of the patients in the attached chart. No significant association was detected between the type and severity of haemophilia with the development of inhibitor. The results of the linear model only showed association (p=0.01) between the severity of haemophilia and AHRS, patients with moderate haemophilia presented a least square mean for the AHRS of 2.6 (0.3), whereas the serious ones had a value of 3.4 (0.2). The least squared means for patients with HA and HB were 2.9 (0.2) and 3.1 (0.4) respectively, the contrast did not reach statistical significance (p&gt;0.1), and the same occurred between patients who had generated or not inhibitor 2.8 (0.2) Vs 3.1 (0.3). Apart from the severity of haemophilia, the other factor that showed a significant and significant effect (p&lt;0.001) on the AHRS values was age. The estimated value for the regression coefficient of age on the radiological status was 0.05 (0.009). <h3>Conclusions</h3> Contrary to what has been observed in other studies, in our cohort it does not seem that the presence of inhibitor or the type of haemophilia has a negative effect on the severity of the radiological findings. The age and severity of haemophilia do seem to influence the radiological stage. <h3>Disclosure of Interest</h3> None declared
Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy
Authors: Margaret E. Smith, Hari Nathan
Journal: JAMA Network Open (2019)

Jason M. Foster, MD; Richard Sleightholm, BS; Asish Patel, MD; Valerie Shostrom, MS; Bradley Hall, MD; Beth Neilsen, BS; David Bartlett, MD; Lynette Smith, PhD
Intratumoral Injection of CpG-ODN Plus Systemic Ibrutinib Induces an Anti-Tumor Immune Response Affecting T Cell Subsets in the Microenvironment of Both Injected and Non-Injected Tumor Sites in Patients with Low-Grade Lymphoma
Authors: Debra K. Czerwinski, Matthew J. Frank, Tanaya Shree, Michael S Khodadoust, Steven R. Long, Ronald Levy
Journal: Blood (2018)
Abstract 17337: Multiple Biomarkers for the Prediction of Cardiovascular Death Within the Minnesota Heart Survey
Authors: Fred S Apple, Lyn M Steffen, Lesly A Pearce, MaryAnn M Murakami, Russell V Luepker
Journal: Circulation (2010)

Background: As understanding of pathophysiologic pathways for cardiovascular disease (CVD) improves, new biomarkers are emerging. We examined several novel biomarkers to determine their ability to ...
Activating KRAS, NRAS, and BRAF Mutants Enhance Proteasome Capacity and Reduce Endoplasmic Reticulum Stress in Multiple Myeloma, Thereby Promoting Plasma Cell Survival and Proteasome Inhibitor Resistance
Authors: Fazal Shirazi, Richard J. Jones, Isere Kuiatse, Zuzana Berkova, Hua Wang, Hans C. Lee, Samuel Hong, Ram Kumar Singh, Lawrence Robert Dick, Nibedita Chattopadhyay, Robert Z. Orlowski
Journal: Blood (2018)
Abstract 15443: Real World Effectiveness and Safety of Ticagrelor and Prasugrel as Compared With Clopidogrel Are Inconsistent With the Findings From Clinical Trials
Authors: Zhong Yuan, Alexei N Plotnikov, James Weaver, Ajit Londhe, Elliot Barnathan, John Strony
Journal: Circulation (2017)

Introduction: Clinical trials demonstrated better efficacy in preventing cardiovascular events for the new generation of P2Y12 receptor antagonists (prasugrel and ticagrelor) as compared with clopi...
Allogeneic Stem Cell Transplantation for Blast Crisis Chronic Myeloid Leukemia in the Era of Tyrosine Kinase Inhibitors — a Retrospective Study By the EBMT Chronic Malignancies Working Party
Authors: Aleksandar Radujkovic, Henric-Jan Blok, Arnon Nagler, Francis Ayuketang Ayuk, Jürgen Finke, Johanna Tischer, Jiri Mayer, Koc Yener, Federica SORA, Jakob R. Passweg, Jennifer Byrne, Pavel Jindra, Hendrik Veelken, Gérard Socié, Johan Maertens, Nicolaas Schaap, Arnold Ganser, Maija Itälä-Remes, Eleni Tholouli, Mutlu Arat, Vanderson G. Rocha, Per Ljungman, Yves Chalandon, Kroger Nicolaus
Journal: Blood (2018)
Cardiac contractility modulation improves long‐term survival and hospitalizations in heart failure with reduced ejection fraction
Authors: Stefan D. Anker, Martin Borggrefe, Hans Neuser, Marc‐Alexander Ohlow, Susanne Röger, Andreas Goette, Bjoern A. Remppis, Karl‐Heinz Kuck, Kevin B. Najarian, David D. Gutterman, Benny Rousso, Daniel Burkhoff, Gerd Hasenfuss
Journal: European Journal of Heart Failure (2019)