tbuckley/cabot-search huggingface dataset:
What is axial spondyloarthritis? A latent class and transition analysis in the SPACE and DESIR cohorts
Authors: Alexandre Sepriano, Sofia Ramiro, Désirée van der Heijde, Floris van Gaalen, Pierre Hoonhout, Anna Molto, Alain Saraux, Roberta Ramonda, Maxime Dougados, Robert Landewé
Journal: Annals of the Rheumatic Diseases (2020)

Objectives To gain expert-judgement-free insight into the Gestalt of axial spondyloarthritis (axSpA), by investigating its ‘latent constructs’ and to test how well these latent constructs fit the Assessment of SpondyloArthritis international Society (ASAS) classification criteria. Methods Two independent cohorts of patients with early onset chronic back pain (SPondyloArthritis Caught Early (SPACE)) or inflammatory back pain (IBP) (DEvenir des Spondylarthopathies Indifférenciées Récentes (DESIR)) were analysed. Latent class analysis (LCA) was used to estimate the (unobserved) potential classes underlying axSpA. The best LCA model groups patients into clinically meaningful classes with best fit. Each class was labelled based on most prominent features. Percentage fulfilment of ASAS axSpA, peripheral SpA (pSpA) (ignoring IBP) or both classification criteria was calculated. Five-year data from DESIR were used to perform latent transition analysis (LTA) to examine if patients change classes over time. Results SPACE (n=465) yielded four discernible classes: ‘axial’ with highest likelihood of abnormal imaging and HLA-B27 positivity; ‘IBP+peripheral’ with 100% IBP and dominant peripheral symptoms; ‘at risk’ with positive family history and HLA-B27 and ‘no SpA’ with low likelihood for each SpA feature. LCA in DESIR (n=576) yielded similar classes, except for the ‘no-SpA’. The ASAS axSpA criteria captured almost all (SPACE: 98%; DESIR: 93%) ‘axial’ patients, but the ‘IBP+peripheral’ class was only captured well by combining the axSpA and pSpA criteria (SPACE: 78%; DESIR: 89%). Only 4% of ‘no SpA’ patients fulfilled the axSpA criteria in SPACE. LTA suggested that 5-year transitions across classes were unlikely (11%). Conclusion The Gestalt of axSpA comprises three discernible entities, only appropriately captured by combining the ASAS axSpA and pSpA classification criteria. It is questionable whether some patients with ‘axSpA at risk’ will ever develop axSpA.
Clonal hematopoiesis by SLIT1-mutated hematopoietic stem cells due to a breakdown of the autocrine loop involving Slit1 in acquired aplastic anemia
Authors: Kohei Hosokawa, Hiroki Mizumaki, Mahmoud I. Elbadry, Chizuru Saito, J. Luis Espinoza, An Thi Thanh Dao, Takamasa Katagiri, Ai Harashima, Akihiro Kikuchi, Akinori Kanai, Hirotaka Matsui, Toshiya Inaba, Masafumi Taniwaki, Yasuhiko Yamamoto, Shinji Nakao
Journal: Leukemia (2019)
SAT0226 INFLIXIMAB FOR THE TREATMENT OF REFRACTORY POLYARTERITIS NODOSA
Authors: Shira Ginsberg, Itzhak Rosner, Gleb Slobodin, Michael Rosenbaum, Lisa Kaly, Nizar Jiries, Nina Boulman, Abid Awisat, Haya Husseuin, Irina Novofastovski, Amal Silawy, Doron Rimar
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Polyarteritis Nodosa (PAN) is a necrotizing vasculitis predominantly affecting medium and small size arteries (1). Cyclophosphamide, a drug with narrow therapeutic range and poor safety profile, constitutes the treatment of choice for PAN vasculitis with major organ involvement. (2) <h3>Objectives</h3> To describe our clinical experience in treating refractory PAN with infliximab (a TNF inhibitor), a drug with good tolerability and better safety profile than cyclophosphamide. <h3>Methods</h3> Twenty-six PAN patients were admitted to our rheumatology unit between 2006 and 2017, of whom 9 patients, with severe and refractory disease, were treated with infliximab after failure of standard treatment. We describe herein the patients' characteristics, clinical manifestations, severity and response to infliximab treatment and review the current literature. <h3>Results</h3> Complete remission was defined as the absence of features of active disease and withdrawal of prednisone therapy. Significant improvement was defined as clinical improvement and prednisone dose reduction of at least 50% or a 50% reduction in immune modulatory medications other than prednisone. After 4 months of treatment, 8/9 (89%) patients achieved significant improvement, with two of them achieving complete remission. <h3>Conclusion</h3> We suggest anti TNF agents, and in particular infliximab, are relatively safe and efficacious treatment options in refractory PAN A randomized controlled trial should be done in order to objectively evaluate infliximab in PAN. <h3>References</h3> [1] Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65:1-11. [2] De Virgilio A, Greco A, Magliulo G, Gallo A, Ruoppolo G, Conte M, Martellucci S, de Vincentiis M. Polyarteritis nodosa: A contemporary overview. Autoimmun Rev. 2016; 15:564-70. <h3>Disclosure of Interests</h3> None declared
Novel Molecular Pathogenesis and Therapeutic Target in Acute Erythroid Leukemia
Authors: June Takeda, Kenichi Yoshida, Yasuhito Nannya, Lee-Yung Shih, Ayana Kon, Akinori Yoda, Yotaro Ochi, Yusuke Shiozawa, Tetsuichi Yoshizato, Cassandra M Kerr, Yuichi Shiraishi, Kenichi Chiba, Yasunobu Nagata, Akira Hangaishi, Toshiyuki Kitano, Ken Ishiyama, Hisashi Tsurumi, Yasushi Miyazaki, Nobuhiro Hiramoto, Takayuki Ishikawa, Akifumi Takaori-Kondo, Masahiro Nakagawa, Masashi Sanada, Hideyuki Nakazawa, Keisuke Kataoka, Ryunosuke Saiki, Hiroko Tanaka, Kensuke Usuki, Shuichi Miyawaki, Satoru Miyano, Arnold Ganser, Michael Heuser, Jaroslaw P. Maciejewski, Felicitas Thol, Hideki Makishima, Seishi Ogawa
Journal: Blood (2019)
THU0613 MRI AND ULTRASOUND (US) ASSESSMENT OF TEMPORO-MANDIBULAR JOINT (TMJ) INVOLVEMENT IN ADULT PATIENTS WITH DIAGNOSIS OF JUVENILE IDIOPATHIC ARTHRITIS (JIA) AND NON-JIA CHRONIC INFLAMMATORY ARTHROPATIES
Authors: Adriano Lercara, Gloria Crepaldi, Marco Davico, Stefano Cirillo, Sarah Marouen, Enrica Vandelli, Yves-Marie Pers, Claudia Lomater
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> TMJ is involved in about 50% of JIA cases, often bilateral and asymptomatic in up to 71% of cases<sup>1</sup>, leading to higher rate of dysfunction and anatomical abnormalities (as retrognathia, facial asymmetry, limited mouth opening, pain)<sup>2</sup>. To date MRI is the gold standard to study both TMJ inflammation and damage<sup>3</sup> but US is a widely used diagnostic imaging tool in rheumatic patients and may be a cheaper and more accessible alternative. <h3>Objectives</h3> To investigate the prevalence of TMJ involvement in young adults with JIA and young adults with non-JIA inflammatory rheumatisms, comparing MRI with US. <h3>Methods</h3> Patients were recruited prospectively in 2 clinical centers. Inclusion criteria were: patients &lt;35 years diagnosed with JIA who had undergone transition from the pediatric to the adult rheumatologist, and patients diagnosed with non-JIA inflammatory arthropathies. All patients underwent clinical examination for TMJs (tenderness to palpation, swelling, signs of damage such as joint crepitations, lateral deviation, retrognathia and decreased mouth opening) and MRI of the TMJs to detect inflammation (bone marrow edema, effusion, synovial thickening) or damage (condylar flattening, erosions, disk abnormalities). Blindly to MRI, US was performed to evaluate presence of inflammation (effusion and/or joint capsule abnormalities) or damage (erosion). MRIs and US with either inflammation or damage were considered pathological. Demographic and clinical characteristics were described using frequency or median and interquartile range (IQR), depending on the distribution of the variable. Differences between groups were analyzed using the Mann-Whitney U test, the χ² test and Cohen's correlation index when appropriate. US sensibility (Se) and specificity (Sp) were also calculated. <h3>Results</h3> 11 patients were included in the JIA group and 7 patients in the non-JIA group. Patients' and TMJs involvement characteristics were reported in Table 1. <h3>Conclusion</h3> The concordance between MRI and ultrasound is not optimal, and existing TMJ US protocols are based on children. Further studies are needed to develop a suitable US protocol to detect TMJ involvement in adult patients as a screening tool. <h3>References</h3> [1] Billiau AD, et al. J Rheumatol. 2007Sep;34(9):1925–33. [2] Arvidsson LZ, et al. Radiology2010Jul;256(1):191–200. [3] Hemke R, et al. Eur Radiol. 2014Feb;24(2):327–34. <h3>Disclosure of Interests</h3> None declared
Simultaneous B and T cell acute lymphoblastic leukemias in zebrafish driven by transgenic MYC: implications for oncogenesis and lymphopoiesis
Authors: Chiara Borga, Gilseung Park, Clay Foster, Jessica Burroughs-Garcia, Matteo Marchesin, Rikin Shah, Ameera Hasan, Syed T. Ahmed, Silvia Bresolin, Lance Batchelor, Teresa Scordino, Rodney R. Miles, Geertruy te Kronnie, James L. Regens, J. Kimble Frazer
Journal: Leukemia (2018)

Precursor-B cell acute lymphoblastic leukemia (pre-B ALL) is the most common pediatric cancer, but there are no useful zebrafish pre-B ALL models. We describe the first highly- penetrant zebrafish pre-B ALL, driven by human MYC. Leukemias express B lymphoblast-specific genes and are distinct from T cell ALL (T-ALL)—which these fish also develop. Zebrafish pre-B ALL shares in vivo features and expression profiles with human pre-B ALL, and these profiles differ from zebrafish T-ALL or normal B and T cells. These animals also exhibit aberrant lymphocyte development. As the only robust zebrafish pre-B ALL model and only example where T-ALL also develops, this model can reveal differences between MYC-driven pre-B vs. T-ALL and be exploited to discover novel pre-B ALL therapies.
Abstract 19748: Low Iron is an Independent Predictor for Adverse Outcomes in Patients With Acute Decompensated Heart Failure -From NARA-HF Study
Authors: Tomoya Ueda, Satomi Ishihara, Yasuki Nakada, Hitoshi Nakagawa, Hiroyuki Kawata, Rika Kawakami, Hiroyuki Okura, Yoshihiko Saito
Journal: Circulation (2017)

Introduction: The concept of cardio-renal-anemia syndrome has been widespread. Moreover, iron deficiency has been reported that it is associated with poor outcome with or without anemia in chronic ...
Long-term Cognitive Outcomes in Patients With Pediatric-Onset vs Adult-Onset Multiple Sclerosis
Authors: Kyla A. McKay, Ali Manouchehrinia, Lindsay Berrigan, John D. Fisk, Tomas Olsson, Jan Hillert
Journal: JAMA Neurology (2019)

Cognitive impairment in multiple sclerosis (MS) can lead to reduced quality of life, social functioning, and employment. Few studies have investigated cognitive outcomes among patients with pediatric-onset MS (POMS) over the long term.To compare long-term information-processing efficiency between patients with POMS and adult-onset MS (AOMS).This population-based longitudinal cohort study accessed the Swedish MS Registry (SMSreg), which collates information from all 64 neurology clinics in Sweden. Registered cases with definite MS in the SMSreg with an onset before April 15, 2018, and at least 2 Symbol Digit Modalities Test (SDMT) scores recorded were included. Only persons aged 18 to 55 years and with duration of disease of less than 30 years at the time of SDMT administration were included, to ensure comparable ranges between patients with POMS and AOMS. Of 8247 persons with an SDMT recorded in the SMSreg, 5704 met inclusion criteria, 300 (5.3%) of whom had POMS. Data were collected from April 1, 2006, through April 15, 2018 and analyzed from April through August 2018.Pediatric-onset MS (onset <18 years of age) vs AOMS (onset ≥18 years of age).Information-processing efficiency measured every 6 or 12 months by the SDMT. Linear mixed-effects models were used to compare all available SDMT scores between patients with POMS and those with AOMS. Persons with cognitive impairment (ever vs never) were identified using regression-based norms and compared between POMS and AOMS groups using logistic regression.Of the 5704 participants, 4015 were female (70.4%), and 5569 had a relapsing-onset disease course (97.6%). Most participants were exposed to a disease-modifying therapy (DMT) during follow-up (98.8%). Median age at baseline for the POMS group was 25.6 years (interquartile range, 21.0-31.7 years) and for the AOMS group, 38.3 years (interquartile range, 31.4-45.2 years). A total of 46 429 unique SDMT scores were analyzed. After adjustment for sex, age, disease duration, disease course, total number of SDMTs completed, oral or visual SDMT form, and DMT exposure, the SDMT score for patients with POMS was significantly lower than that of patients with AOMS (β coefficient, -3.59 [95% CI, -5.56 to -1.54]). The SDMT score for patients with POMS declined faster than that of patients with AOMS (β coefficient, -0.30 [95% CI, -0.42 tp -0.17]). The odds of cognitive impairment were also significantly elevated in the POMS group (odds ratio, 1.44; 95% CI, 1.06-1.98).In adulthood, patients with POMS demonstrated a more rapid reduction in information-processing efficiency over time and were more likely to experience cognitive impairment than patients with AOMS, independent of age or disease duration. Further investigation is required to understand the mechanisms by which early MS onset influences cognitive outcomes.
Abstract 20067: Mitral Valve Repair With Intervention to Subvalvular Apparatus Has a Potential to Improve the Outcome for Functional Mitral Regurgitation
Authors: Keiji Oi, Eiki Nagaoka, Tomohiro Mizuno, Tsuyoshi Hachimaru, Masafumi Yashima, Hidehito Kuroki, Tatsuki Fujiwara, Masashi Takeshita, Kenji Yokoyama, Hironobu Sakurai, Hirokuni Arai
Journal: Circulation (2017)

Introduction: A prospective randomized trial of surgical treatment for functional mitral regurgitation (FMR) has reported that there was no significant difference in 2-year survival between mitral ...
Electrically conductive nanomaterials for cardiac tissue engineering
Authors: Khadijeh Ashtari, Hojjatollah Nazari, Hyojin Ko, Peyton Tebon, Masoud Akhshik, Mohsen Akbari, Sanaz Naghavi Alhosseini, Masoud Mozafari, Bita Mehravi, Masoud Soleimani, Reza Ardehali, Majid Ebrahimi Warkiani, Samad Ahadian, Ali Khademhosseini
Journal: Advanced Drug Delivery Reviews (2019)
Abstract 31: Cardiovascular Health at Young and Middle Ages and Dementia in Older Age - The Chicago Heart Association Detection Project in Industry Study
Authors: Thanh Huyen T Vu, Donald M Lloyd-Jones, Cuiping Schiman, Lei Liu, Lihui Zhao, Kiang Liu, Ya-Chen Tina Shih, James F Fries, Martha L Daviglus, Norrina B Allen
Journal: Circulation (2017)

Background: Data are sparse regarding the association of cardiovascular health (CVH) in younger/middle age with the diagnosis of dementia later in life. Methods: We used linked data from the Chicago Heart Association Detection Project in Industry Study assessed in 1967-73 with fee-for-service Medicare claims and National Death Index data from 1991-2010. Participants were ≤65 years old in 1991 and Medicare eligible during 1991-2010. Baseline CVD risk factors included blood pressure, cholesterol, BMI, smoking, and diabetes. Participants were classified into four strata: favorable levels of all factors, 0 factors high but 1+ elevated, 1 high, and ≥2 high risk factors. ICD-9 codes were used to identify date of first dementia diagnosis. We used competing Cox models to estimate hazards for dementia in Medicare data after age 65 with competing event of all-cause mortality prior to dementia diagnosis. Covariates included baseline age, race, sex, and education attainment. Results: This study included 4,273 females and 8,381 males, 10.3% Black, baseline ages 18-48. Dementia was diagnosed in 5.1% of study participants. The prevalence of dementia diagnosis increased with increasing CVH burden from 3.4% to 6.4%. During 1991-2010 in Medicare, the average time from age 65 to the first dementia diagnosis was 7.7 years. Greater CVH in younger age was associated with a reduced risk of being diagnosed with dementia in later life. Hazards ratios of experiencing dementia in those with baseline favorable levels, 0 RF high, and 1 only high RF were lower by 36%, 29%, and 28% respectively as compared to those with 2+ high-risk factors (see Table - Model 1). Similar trends were observed when deaths were treated as competing events although the association was attenuated (Model 2). Patterns were similar when stratified by sex or race. Conclusions: Having a favorable CVH profile at younger age is associated with lower risk of dementia in older age. Improvements in CVH in younger age may translate to increased independence and quality of life later in life.
FRI0651 THE CLINICAL IMPLICATION OF NASAL BIOPSY FOR CLASSIFYING GRANULOMATOSIS WITH POLYANGIITIS IN PATIENTS WITH RHINOSINUSITIS: A SINGLE CENTRE RETROSPECTIVE STUDY
Authors: Juyoung Yoo, Sungsoo Ahn, Seungmin Jung, Jason Jungsik Song, Yongbeom Park, Sangwon Lee
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Granulomatosis with polyangiitis(GPA) mainly involves the upper and lower respiratory tracts and kidneys and induces necrotising vasculitis and granuloma. Nasal biopsy has been recommended in GPA-suspected patients to not only completely cure chronic rhinosinusitis, but also clearly discriminate its aetiologies. <h3>Objectives</h3> We investigated whether the classification of GPA could be made without nasal biopsy in immunosuppressant drug-naïve 45 patients with chronic rhinosinusitis who had previously been classified as GPA. <h3>Methods</h3> We retrospectively reviewed the medical records of 45 patients with GPA. Twenty-six patients exhibited chronic rhinosinusitis, among which 16 patients underwent nasal biopsy (10 with granuloma and 6 without granuloma). We applied the 2007 European Medicines Agency algorithm for the classification of GPA, the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis and the 2017 American College of Rheumatology/European League Against Rheumatism provisional classification criteria for GPA to them for reclassifying GPA. (Figure1) <h3>Results</h3> The mean age was 58.4 years and 17 patients were men. There were no differences in clinical and laboratory results between those with and without granuloma. Among 6 patients without granuloma on nasal biopsy, 3 patients with only ANCAs and chronic rhinosinusitis could be classified as GPA due to PR3-ANCA (or C-ANCA) positivity. Among 9 patients without nasal biopsy, 3 patients with only chronic rhinosinusitis could be classified as GPA due to GPA-specific lung lesions. (Table 1) When we excluded an item of granuloma in 10 GPA patients with granuloma on nasal biopsy, 4 patients without ANCAs could be classified as GPA due to GPA-specific lung lesions and cartilaginous involvement. (Table 2) <h3>Conclusion</h3> Nasal biopsy is necessary and useful for classifying GPA. However, nasal biopsy could be replaced with PR3-ANCA (or C-ANCA) positivity, GPA-specific lung lesions and cartilaginous involvement in GPA suspected patients with chronic rhinosinusitis. <h3>References</h3> [1] Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. [2] Comarmond C, Cacoub P. Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment. Autoimmun Rev. 2014;13(11):1121-5. <h3>Acknowledgement</h3> None <h3>Disclosure of Interests</h3> None declared
Abstract 1693: The Impact of Percutaneous Coronary Intervention on Renal Function in Diabetic Patients With Normal Baseline Serum Creatinine
Authors: Vikram Raya, Probal Roy, Ashesh Buch, Aamir Javaid, Teruo Okabe, Tina L Pinto Slottow, Daniel H Steinberg, Kimberly Smith, Zhenyi Xue, Lowell F Satler, Kenneth M Kent, William O Suddath, Augusto D Pichard, Joseph Lindsay, Ron Waksman
Journal: Circulation (2007)

Background: Diabetics are prone to contrast nephropathy. The outcome of diabetics with normal baseline serum creatinine (SCr) undergoing PCI remains under-evaluated. The present study aims to assess the incidence, outcome, and correlates of SCr rise post-PCI in this subset. Methods: The study cohort consisted of 570 consecutive diabetic patients with normal SCr (≤1.3mg/dl) who underwent non-emergent PCI. Patients presenting with AMI, shock, and age &gt;75 years were excluded. SCr rise was defined as ≥25% rise from baseline. Study endpoints were post-PCI SCr rise and major adverse cardiac events (MACE) at 6 months. Logistic regression was performed to identify predictors of SCr rise. Results: SCr rise occurred in 70 patients (incidence=12.3%). Patients with SCr rise were more likely to be female (55.7% vs. 35.5% p = 0.001) and have a history of congestive heart failure (24.2% vs. 14.7% p=0.048). Patients with SCr rise had greater entry site complications (hematoma, pseudoaneurysm) and need for blood transfusion (16.7% vs.1.7% p&lt;0.001). In-hospital mortality (8.6% vs. 0.2% p&lt;0.001) and length of stay (4.51±5.2 vs. 2.23±2.9 days p&lt;0.001) were significantly higher in patients with SCr rise. No study patient required dialysis. At 6 months, MACE was markedly higher in patients with SCr rise (21.4% vs. 6.0% p&lt;0.001) driven by death and revascularization. Independent predictors of SCr rise were female gender, blood transfusion, and lower body mass index. Conclusions: Post-PCI rise in SCr in diabetics with normal baseline values is common and is a marker of late adverse cardiac events. Greater emphasis needs to be placed on prevention and monitoring of rises in SCr in the diabetic population.