tbuckley/cabot-search huggingface dataset:
POS0823 ARTHRITIS IN ADULT IGA VASCULITIS
Authors: A. Hočevar, V. Jurčić, M. Tomsic, Z. Rotar
Journal: Annals of the Rheumatic Diseases (2021)

Background: Arthritis is one of the main clinical features of IgA vasculitis (IgAV). Joint involvement represents the second most common manifestation in childhood IgAV with the predilection for the knees and ankles. Objectives: We aimed to describe the characteristics of arthritis in adult IgAV cohort. Methods: We analysed medical records of histologically proven adult IgAV cases, diagnosed between January 2010 and December 2020 at our secondary/tertiary rheumatology centre. The frequency, temporal occurrence and the localization of arthritis was recorded. In addition, we searched for potential differences in other IgAV features between patients with and without arthritis. Results: During the 132-month observation period we identified 328 new IgAV cases (59.5% males, median (IQR) age 64.3 (45.1; 76.1) years). Forty-eight (14.6%) patients developed arthritis. Arthritis was the first IgAV manifestation in 16 (4.9%) patients. Arthritis was mono-, oligo- and poly- articular (involving up to 15 joints) in 13 (4.0%), 25 (7.6%) and 10 (3.0%) patients, respectively. Arthritis was most common in wrists and ankles (each in 18 (37.5%) patients); metacarpophalangeal joints and knees (each in 11 (22.9%)); proximal interphalangeal joints (9 (18.8%)); elbows (8 (16.7%)) and metatarsophalangeal joints (4 (8.3%)). Clinical differences between IgAV patients with and without arthritis are presented in table 1. Patients with arthritis were significantly younger, more commonly developed gastrointestinal tract involvement compared to those without arthritis. Arthritis remitted in all with immunomodulatory treatment (given predominantly for necrotic skin purpura or visceral involvement). Follow up (FU) data accessible for 42/48 (87.5%) patients with arthritis showed that IgAV relapsed in 10 (23.8%) patients during a median (IQR) 24.5 (12.9; 40.7) month FU. Relapses were limited to skin and/or kidneys, there were no relapses of arthritis. Table 1. Clinical characteristics of IgA vasculitis patients with and without arthritis Clinical characteristics Arthritis IgAV (48) Non-arthritis IgAV (280) P value Male gender (%) 56.3 60.0 0.636 Age (years)* 49.9 (36.1-66.9) 65.4 (48.4;77.5) &lt;0.001 Ever smoker (%) 50.0 43.6 0.435 Prior infection (%) 43.8 30.4 0.094 Generalized purpura 50.0 50.0 1.0 Skin necroses (%) 35.4 49.6 0.085 GI involvement (%) 47.9 25.0 0.002 Renal involvement (%) 47.9 46.8 1.0 Elevated serum IgA (%) 33.3 (11/33) 52.0 (115/221) 0.061 Legend: * median and IQR; GI gastrointestinal; Conclusion: Arthritis in adult IgAV was frequently oligoarticular, involved large and small joints of both upper and lower extremities, and was not prone to chronic course and recurrence. In addition, we found an association between arthritis and gastrointestinal tract involvement. Disclosure of Interests: None declared
Abstract 15592: Need for Permanent Pacemaker Implantation and Long-Term Mortality After Isolated Surgical Aortic Valve Replacement: A State-Wide Analysis of 29,461 Patients
Authors: Gil Moskowitz, Gennaro Giustino, Gorav Ailawadi, Michael W Chu, A. Marc Gillinov, Marissa Miller, Patrick T O’Gara, Wendy C Taddei-Peters, Anuradha Lala, Donna M Mancini, Joseph DeRose, Annetine C Gelijns, Alan J Moskowitz, Natalia Egorova
Journal: Circulation (2019)

Introduction: Conduction disturbances requiring permanent pacemaker (PPM) implantation are relatively frequent complications of surgical aortic valve replacement (SAVR). However, their impact on lo...
Speech-Induced Atrial Tachycardia
Authors: André Zimerman, Andre d’Avila
Journal: New England Journal of Medicine (2021)

A 58-year-old man presented with dizziness and palpitations that occurred only while speaking. Continuous electrocardiographic monitoring (shown in a video) revealed atrial premature beats while the patient was speaking isolated words and sustained atrial tachycardia when he was speaking full sentences.
Low dose shikonin and anthracyclines coloaded liposomes induce robust immunogenetic cell death for synergistic chemo-immunotherapy
Authors: Jinbo Li, Shuang Zhou, Jiang Yu, Wenxu Cai, Yinxian Yang, Xiao Kuang, Hongzhuo Liu, Zhonggui He, Yongjun Wang
Journal: Journal of Controlled Release (2021)
POS0116 HEART RATE VARIABILITY IN PATIENTS WITH BEHCETS DISEASE
Authors: R. Goloeva, Z. Alekberova, T. Popkova, S. Glukhova, D. Novikova
Journal: Annals of the Rheumatic Diseases (2021)

Background: Behcet’s disease (BD) is a systemic vasculitis affecting all types and sizes of blood vessels. Heart rate variability (HRV) reflects sympathetic -parasympathetic imbalance in the autonomic NS regulation. Low HRV values are known as independent risk factor of death and non-fatal cardiovascular events in both - survivors of a myocardial infarction and in asymptomatic population. Objectives: The aim of this study is to evaluate HRV in BD pts vs healthy controls. Methods: The study group included 74 BD pts (53males/21females) with disease duration of 9,0 (5,0;15,0)/9,0 (7;20) years, and the control group - 32/15 age-matched healthy m/f. The following HRV parameters from 24h ECG ambulatory recording were assessed: MeanNN and time-domain variables, adjusted by MeanNN (SDNNn%, SDNNin%, RMSSDn%). Additionally, all traditional cardiovascular risk factors such as systolic blood pressure (SPB), smoking status, BMI values, dyslipidemia profile, ultrasonographic values of carotid intima-media thickness (IMT), and levels high sensitive CRP (hsCRP) as a marker of inflammation were evaluated. Results: In BD patients HRV values (RMSSDn%) were significantly lower compared to healthy controls (table 1). Table 1. HRV parameters in BD patients and control group Parameters Males Females BD (n=53) Control (n=32) BD (n=21) Control (n=15) Age, years 30 (24; 36) 30 (26; 35) 32 (26; 37) 28 (24; 31) MeanNN, ms 810 (732; 849) 782 (732; 835) 776 (708; 830) 764 (694; 832) SDNN n (%) 16,9 (13,6; 19,4) 17,2 (16,3; 21,1) 13,1 (11,3; 5,3) 12,2 (10,7; 14,6) SDNNi n (%) 6,8 (5,1; 8,1) 6,8 (5,0; 8,3) 7,1 (6,1; 7,7) 5,2 (4,9; 5,7) RMSSD n (%) 2,1 (1,5; 2,3)** 4,1 (2,7; 5,2)** 1,7 (1,4; 3,7)* 2,8 (2,2; 3,9)* Data are presented in median values and interquartile range, *p&lt;0,05, **p&lt;0,005 vs controls. There was a significant negative correlation in BD patients between HRV (SDNNin%) and age (r= -0,4; p =0,00), disease duration (r= -0,3; p =0,00), BMI (r= -0,2; p &lt;0,01), cholesterol levels (r= -0,3; p =0,00), LDLP (r= -0,3; p =0,00) and increased IMT (r= -0,2; p =0,04), and also between HRV (RMSSD%) and age (r= -0,2; p =0,04), disease duration (r= -0,2; p =0,01), cholesterol levels (r= -0,3; p =0,00), HDLP (r= -0,2; p =0,04); a positive correlation was established between HRV (SNNN%) and smoking (r= -0,2; p =0,04). The control group showed positive correlation between HRV (SNNN%) and increased IMT (r= 0,4; p =0,01). Conclusion: HRV reduction reflects impaired sympathetic -parasympathetic regulation in BD pts, associated with pts’ age, disease duration and presence of traditional cardiovascular risk factors: BMI, increased cholesterol levels, LDLP, and such asymptomatic manifestation of atherosclerosis as increased IMT. Disclosure of Interests: None declared
<scp>POEMS</scp> syndrome: Update on diagnosis, risk‐stratification, and management
Authors: Angela Dispenzieri
Journal: American Journal of Hematology (2015)

Disease overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome. Diagnosis: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria. Risk stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy. Risk‐adapted therapy: For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3–6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. The benefit of anti‐VEGF antibodies is conflicting. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 90:951–962, 2015. © 2015 Wiley Periodicals, Inc.
Aged hematopoietic stem cells are refractory to bloodborne systemic rejuvenation interventions
Authors: Theodore T. Ho, Paul V. Dellorusso, Evgenia V. Verovskaya, Sietske T. Bakker, Johanna Flach, Lucas K. Smith, Patrick B. Ventura, Olivia M. Lansinger, Aurélie Hérault, Si Yi Zhang, Yoon-A Kang, Carl A. Mitchell, Saul A. Villeda, Emmanuelle Passegué
Journal: The Journal of Experimental Medicine (2021)

While young blood can restore many aged tissues, its effects on the aged blood system itself and old hematopoietic stem cells (HSCs) have not been determined. Here, we used transplantation, parabiosis, plasma transfer, exercise, calorie restriction, and aging mutant mice to understand the effects of age-regulated systemic factors on HSCs and their bone marrow (BM) niche. We found that neither exposure to young blood, nor long-term residence in young niches after parabiont separation, nor direct heterochronic transplantation had any observable rejuvenating effects on old HSCs. Likewise, exercise and calorie restriction did not improve old HSC function, nor old BM niches. Conversely, young HSCs were not affected by systemic pro-aging conditions, and HSC function was not impacted by mutations influencing organismal aging in established long-lived or progeroid genetic models. Therefore, the blood system that carries factors with either rejuvenating or pro-aging properties for many other tissues is itself refractory to those factors.
POS0355 ASSOCIATIONS BETWEEN HLA-DRB1 SHARED EPITOPES ALLELES AND ANTI-RA33 ANTIBODIES IN DIFFERENT SUBSETS OF RHEUMATOID ARTHRITIS IN MALAYSIAN POPULATION
Authors: A. F. Nurul-Aain, S. S. Ch’ng, H. Baharuddin, M. Mohd Zain, I. S. Lau, H. Mohd Yusoof, A. Ahmad, A. Rosman, A. A. Siti-Aisyah, L. Padyukov, L. Alfredsson, L. Klareskog, J. Rönnelid, C. L. Too
Journal: Annals of the Rheumatic Diseases (2021)

Background: The mechanisms affecting anti-RA33 antibody’s involvement in RA pathogenesis is still unclear. Refining our understanding of anti-RA33’s role in RA in relation to known RA-associated genes and serological elements is needed. Objectives: We investigated the relationship between RA-associated HLA-DRB1 epitope (SE) allele and presence of anti-RA33 antibodies in different serological subsets of rheumatoid arthritis in a Malaysian population. Methods: Serum samples from 550 RA cases comprising seronegative (negative for anti-CCP2, IgG and IgM, n=250), seropositive (triple-autoantibody positive, n=150), singular anti-CCP2 positive (n=100), and double RF positive RA (n=50) were chosen from the Malaysian Epidemiological Investigations of RA (MyEIRA) case-control study. Three hundred MyEIRA population controls were used for comparison. All serum samples were assayed using a commercial anti-RA33 ELISA kit. All genetic samples were genotyped for four-digit HLA-DRB1 alleles using the PCR-SSO method on Luminex platform. Results: The proportions of anti-RA33 positive was 20.9% in all RA cases (i.e. 34% in RF only positive RA; 25% in seropositive RA; 18% in seronegative RA and 18% in anti-CCP2 only positive RA). The HLA-DRB1 shared epitope alleles were significantly associated with anti-RA33 positive in the seropositive RA subgroup (OR=6.9, 95% CI 1.4-34.8; p=0.02). We observed significant association between anti-RA33 negative and HLA-DRB1 SE alleles among the seropositive RA patients (OR=4.5, 95% CI 2.8-7.2; p&lt;0.001) and among CCP only positive RA (OR=4.4; 95% CI 2.6-7.4; p&lt;0.01). No association was observed between anti-RA33 status and HLA-DRB1 SE alleles in seronegative RA and RF only positive RA. Conclusion: The HLA-DRB1 SE alleles increased the risk of seropositive and CCP only positive RA independent of anti-RA33 positivity. References: [1]Boeters, Debbie M et al. “The 2010 ACR/EULAR criteria are not sufficiently accurate in the early identification of autoantibody-negative rheumatoid arthritis: Results from the Leiden-EAC and ESPOIR cohorts.” Seminars in arthritis and rheumatism vol. 47,2 (2017): 170-174. [2]de Brito Rocha, Sara et al. “Clinical and pathophysiologic relevance of autoantibodies in rheumatoid arthritis.” Advances in rheumatology (London, England) vol. 59,1 2. 17 Jan. 2019. Acknowledgements: The authors would like to thank the Director General of Health, Ministry of Health Malaysia for supporting this study. The authors are also indebted to participants for their kind participation. This study was financially supported by the Ministry of Health, Malaysia (JPP-IMR 08-012). Disclosure of Interests: None declared
Targeting cancer testis antigens in synovial sarcoma
Authors: Geoffrey Mitchell, Seth M Pollack, Michael J Wagner
Journal: Journal for ImmunoTherapy of Cancer (2021)

Synovial sarcoma (SS) is a rare cancer that disproportionately affects children and young adults. Cancer testis antigens (CTAs) are proteins that are expressed early in embryonic development, but generally not expressed in normal tissue. They are aberrantly expressed in many different cancer types and are an attractive therapeutic target for immunotherapies. CTAs are expressed at high levels in SS. This high level of CTA expression makes SS an ideal cancer for treatment strategies aimed at harnessing the immune system to recognize aberrant CTA expression and fight against the cancer. Pivotal clinical trials are now underway, with the potential to dramatically alter the landscape of SS management and treatment from current standards of care. In this review, we describe the rationale for targeting CTAs in SS with a focus on NY-ESO-1 and MAGE-A4, the current state of vaccine and T-cell receptor-based therapies, and consider emerging opportunities for future development.
AB0304 LEVELS AND TRENDS IN PREMATURE MORTALITY BURDEN DUE TO SYSTEMIC LUPUS ERYTHEMATOSUS IN CUBA
Authors: H. Hernández-Negrín
Journal: Annals of the Rheumatic Diseases (2021)

Background: Analysis of the causes of premature mortality is an essential function of public health surveillance. A variety of methods have been used to accurately assess and report the level and trends of premature mortality; however, many have significant limitations, particularly in capturing actual early deaths. Years of life lost (YLL) has been recognized as a robust and comprehensive measure of premature mortality. 1 Systemic lupus erythematosus (SLE) is among the leading causes of premature mortality burden in young women in the United States and premature mortality from SLE is increasing in Latin America. 2, 3 However, the premature mortality burden from SLE has not been fully characterized in Cuba. Objectives: To identify levels and trends in premature mortality burden due to SLE in Cuba during 2001-2018. Methods: Data were obtained from the mortality database of the Ministry of Public Health of Cuba (International Classification of Diseases-10, code 32 as underlying cause of death). YLL were calculated using the standard life expectancy of the WHO’s global health estimates. Age- and sex-specific YLL rates were calculated using the Cuban mid-year population estimates and the age-standardized YLL rate (ASYR) was estimated by the direct method using the WHO’s standard population. The 2001-2018 temporal trends were analyzed using Jointpoint software. Results: During 2001-2018, 1 475 patients died from SLE. These deaths contributed 66 605 YLL (women= 59 166; men=7 433), with a mean of 45.1 YLL per death (women= 45.2; men= 44.5). The 40-44 age group was the most affected in both sexes with 9 266 YLL (women=8 177; men=1 086). The ASYR was higher in 2018 than in 2001 for both sexes (Figure 1). A significantly increasing trend in ASYR due to SLE was identified throughout the period (average annual percent change [AAPC] =1.9; 95% confidence interval [CI] = 0.9 to 3.0), more pronounced in men (AAPC = 7.0; 95% CI = 3.5 to 10.6) than in women (AAPC = 1.3; 95% CI = 0.3 to 2.3). Conclusion: The high levels and growing trends in premature mortality burden from SLE in Cuba, demands its recognition as an important health problem and immediate actions that help mitigate it. References: [1]Martinez R, Soliz P, Caixeta R, Ordunez P. Reflection on modern methods: years of life lost due to premature mortality-a versatile and comprehensive measure for monitoring non-communicable disease mortality. Int J Epidemiol. 2019; 48(4): 1367-1376. [2]Yen EY, Singh RR. Lupus - an unrecognized leading cause of death in young women: Population-based study using nationwide death certificates, 2000-2015. Arthritis Rheumatol. 2018;70:1251–5. [3]Soares CR, Viana K, Moraes-Santos F, Vieira CL, Lamarão F, Iglesias-Rodriguez M. Mortality rate and premature mortality due to systemic erythematosus lupus in Latin America US and England and Wales. Value Health. 2015;18:A805–81. Disclosure of Interests: None declared
Getting toGether in Germinal centers
Authors: Liat Stoler-Barak, Ziv Shulman
Journal: Science Immunology (2021)

In this issue of Science Immunology, Gallman et al. reveal how S-geranylgeranyl-l-glutathione cleavage and transport support P2RY8-driven B cell confinement to the germinal centers and its role in lymphocyte homing to the bone marrow.
Social determinants of health are associated with physical therapy use: a systematic review
Authors: Amanda Day Braaten, Cannon Hanebuth, Holt McPherson, Dashae Smallwood, Samantha Kaplan, David Basirico, Derek Clewley, Zachary Rethorn
Journal: British Journal of Sports Medicine (2021)

Objective The purpose of this systematic review is to identify and summarise the social determinants of health (SDH) cited in the literature and evaluate their association with individuals using physical therapy services. Design A systematic review using qualitatively synthesised information to describe the association between SDH and physical therapy use. Data sources The electronic databases Medline, Embase and Scopus were searched from inception to February 2021, identifying observational and qualitative studies. Eligibility criteria Published studies included all adults, aged 18 or older, who independently sought to use physical therapy, in all practice settings from all geographical locations. Results Of the 9248 studies screened, 36 met the inclusion criteria for the review. The participants represented 8 countries and totaled 2 699 437. The majority of the papers reported moderate strength of association for each SDH. Female gender, non-Hispanic white race/ethnicity, increased education attainment, urban environment, access to transportation, employment, high socioeconomic status and private insurance were associated with higher likelihood of physical therapy use. Conclusion This systematic review identifies predisposing and enabling factors impacting physical therapy usage among adults in different countries and across physical therapy settings. The results of this study have implications for policy and future research regarding populations that have been shown to be using physical therapy services less, such as those with lower levels of education, those in a rural area, or those in a low socioeconomic class.
Single VHH-directed BCMA CAR-T cells cause remission of relapsed/refractory multiple myeloma
Authors: Lu Han, Ji-Shuai Zhang, Jian Zhou, Ke-Shu Zhou, Ben-Ling Xu, Lin-Lin Li, Bai-Jun Fang, Qing-Song Yin, Xing-Hu Zhu, Hu Zhou, Xu-Dong Wei, Hong-Chang Su, Bing-Xiang Zhang, Ya-Nan Wang, Bin Xiang, Quan-Li Gao, Yong-Ping Song
Journal: Leukemia (2021)
Is transoral incisionless fundoplication (TIF) an answer to post-peroral endoscopic myotomy gastroesophageal reflux? A multicenter retrospective study
Authors: Olaya I. Brewer Gutierrez, Kenneth J. Chang, Petros C. Benias, Alireza Sedarat, Mohamad H. Dbouk, Gala Godoy Brewer, David P. Lee, Patrick I. Okolo III, Marcia I. Canto, Mouen A. Khashab
Journal: Endoscopy (2021)

Abstract Background: The use of peroral endoscopic myotomy (POEM) for achalasia has a high incidence of post-procedural gastroesophageal reflux (GER). Transoral incisionless fundoplication (TIF) may be an ideal endoscopic treatment. We report our experience with the use of post-POEM TIF. Methods: In this multicenter retrospective study, post-POEM patients with GER who underwent TIF were included. The study end points were: (i) technical success; (ii) safety; (iii) effectiveness (changes in symptoms, scores, proton pump inhibitor [PPI] use, pH studies). Results: 12 patients underwent TIF after POEM, nine of whom had daily symptoms, with 91.7% requiring twice daily (BID) PPIs. Technical success was achieved in all patients. Two adverse events occurred. There were significant decreases in the percentage of patients on BID PPIs (P = 0.03), frequency of daily symptoms (P = 0.03), Reflux Severity Index questionnaire, and GERD Health-related Quality of Life scores (P = 0.03 and P = 0.003; n = 6). pH studies performed in seven of the patients showed a significant reduction in the mean DeMeester score (P = 0.05) and mean percentage acid exposure time (P = 0.04). Conclusion: Our experience suggests that TIF may be effective and safe in treating GER after POEM. Larger prospective trials are needed.
The Prognostic Impact of Minimal Residual Disease on Risk of Progression and Progression Free Survival in Acute Myeloid Leukemia
Authors: Betul Oran, Rima M. Saliba, Jeffrey L. Jorgensen, Mithun Vinod Shah, David Costa Marin, Sa A. Wang, Sairah Ahmed, Amin M. Alousi, Borje S Andersson, Qaiser Bashir, Stefan O. Ciurea, Katy Rezvani, Uday Popat, Gabriela Rondon, Partow Kebriaei, Elizabeth J Shpall, Richard E. Champlin
Journal: Blood (2017)