tbuckley/cabot-search huggingface dataset:
Diagnostic Criteria and Phenotypes of Pemphigoid and the Association With Gliptins
Authors: Asli Bilgic Temel, Dedee F. Murrell
Journal: JAMA Dermatology (2019)

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SAT0665 Synovitis as a predictor of erosions in the hand osteoarthritis
Authors: O. Balueva, A. Sarapulova, O. Teplyakova
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> The formation of bone erosions in hand osteoarthritis (HOA) is the result of the association of destructive and inflammatory changes in the joint and leads to a more aggressive course of the disease. X-ray examination assesses destructive bone changes, but there is no possibility to assess the condition of periarticular tissues and inflammatory changes objectively. Visualisation of both erosions and synovitis is possible during ultrasound examination of the hands. On the basis of this results it is possible to predict the further development of the disease. <h3>Objectives</h3> To determine the risk of erosion appearance and the features of progression in patients with hand osteoarthritis in a prospective study. <h3>Methods</h3> The study included 45 women which were 45–75 years old with the diagnosis of HOA, defined by criteria of the American College of Rheumatologists (ACR). 22 joints were examined at each patient by ultasound: distal interphalangeal joints (DIP), proximal interphalangeal joints (PIP), joints of the thumb and 1 st carpo-metacarpal joint (1 st CMC). Ultrasound examination included multiplane gray-scale investigation of the listed above groups of joints. The presence and severity of synovial hypertrophy and effusion followed by evaluation of the degree of synovitis was determined in the B-mode; the quantity and the sizes of osteophytes and bone erosions were evaluated in a similar way. The vascularisation of synovial hypertrophy was estimated in the power Doppler ultrasound. The study was started in 2013. In 3 years the study was repeated. The results were processed with STATISTICA 10.0 software (StatSoft Inc. USA) using the methods of nonparametric statistics. For the description of communication of qualitative signs we used coefficient "odds ratio" OR and χ2. The results were recognised statistically reliable at value p &lt;0.05. <h3>Results</h3> The total number of hand joints was evaluated using ultrasound 990. In the primary examination 391 joints had synovitis, 46 joints had synovitis and erosion. In 3 years the number of joints with synovitis and the number of joints with synovitis and erosion increased up to 443 and 73 respectively. In 2016 among 73 joints with synovitis and erosion, 40 joints were with erosion against the background of synovitis 3 years ego, and only in the 8 joints erosion were revealed where was no synovitis. Thus the frequency of erosion detection in hand joints at repeated examination was higher in the cases the synovitis was detected at primary examination: χ 2=16.38 p=0.0001; OR 4.373, CI 95% 2.01–9.47. <h3>Conclusions</h3> The synovitis in hand joints is a prognostic adverse factor of erosions appearance and transformation in the erosive subtype of osteoarthritis. The possibility one-time visualisation of inflammatory and destructive changes allows using ultrasound investigation at primary and differential diagnostics, and dynamic evaluation. <h3>Disclosure of Interest</h3> None declared
SAT0043 Il-33/st2-mediated inflammation in endothelial cell is directly aggravated by il-6 during lupus nephritis
Authors: S.L. Yu, Y.B. Huo, C.H. Huang, Y. Tao, W.H. Huang, C.K. Wong, L.S. Tam, H.N. Qiu, R.L. Chen, C.H. Huang, L. Zhong
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> "Alarmins" are prototypic endogenous pro-inflammatory factors as they are released from necotic cells and provoke local damage or systemic inflammation. Evidences are accumulating to support the inclusion of "Alarmins" as targets of autoreactivity as well as inducers in the pathogenesis of Systemic Lupus Erythematosus (SLE). Interleukin (IL)−33 is a novel member of the family of "Alarmins" because of its characteristics and functions in mediating host immune responses. On this background, we sought to determine the role of IL-33/ST2 axis in lupus pathogenesis. The role of IL-33/ST2 axis has not previously been described in lupus nephritis. <h3>Objectives</h3> This project will study the followings:<sup>1</sup> To determine whether IL-33 was present in renal glomerular endothelial cells;<sup>2</sup> To assess the functional and intracellular signal transuction mechanisms regulating the link between IL-33/ST2-mediated innate immunity and inflammation in human umbilical vein endothelial cells (HUVECs). <h3>Results</h3> This study, for the first time, showed that IL-33 was pathologically expressed in the kidney tissue of patients with lupus nephritis and not in that of subjects with relative normal renal tissues from atrophy. However, no significant difference was observed between patients with lupus nephritis and kidney cancer. Immunofluorescence (IF) for IL-33 in kidney was performed in lupus patients. IL-33 was clearly seen in glomeruli and also in peritubular areas. To determine whether the IL-33 staining in glomeruli area was in endothelium, multiple staining for IL-33, CD34 (a marker for endothelial cells) and 4',6-diamidino-2-phenylindole (DAPI) stained for cell nuclei was performed. Co-localization of IL-33 and DAPI in CD34 positive cells demonstrates IL-33 staining in the nucleus of glomerular endothelial cell of lupus kidney. Expression of intracellular but not surface ST2 was increased in plasmacytoid dendritic cells/pDC (CD16-CD14-CD85k+CD123+) of lupus patients when compared with healthy controls. Incubation of HUVECs with IL-33 and/or IL-12 increased the production of IL-6, but IL-4, IL-5, IL-6, IL-13 and TNF-αwas not produced. <h3>Conclusions</h3> As a result of external stimuli or infection, renal glomerular endothelial cells undergo cellular death and release the "Alarmin", IL-33, to alert the lupus immune system. Released IL-33 interact with their target cells, pDC via their specific receptor ST2 to subsequently induce innate and adaptive responses, activate inflammatory pathways in the pathogenesis of lupus nephritis. <h3>Acknowledgements</h3> This work was supported by National Science Foundation of China (81300585), Science and Technology Planning Project of Guangdong Province (2014A020212322) and Natural Science Foundation of Guangdong Province (2015A030313477). <h3>Disclosure of Interest</h3> None declared
<i>Texas v United States</i>: The Affordable Care Act Is Constitutional and Will Remain So
Authors: Lawrence O. Gostin
Journal: JAMA (2019)

On December 14, 2018, in a widely reported decision, a federal judge in Texas ruled that the entire Affordable Care Act (ACA) is unconstitutional. The judge reasoned that since the ACA’s “individual mandate” is unconstitutional, the rest of the law cannot stand without it. However, the ACA will remain in place pending appeal, and it is highly unlikely that this ruling will stand.
Clinical Predictors of T Cell Fitness for CAR T Cell Manufacturing and Efficacy in Multiple Myeloma
Authors: Ehren Dancy, Alfred L. Garfall, Adam D. Cohen, Joseph A Fraietta, Megan Davis, Bruce L Levine, Don L. Siegel, Edward A. Stadtmauer, Dan T. Vogl, Adam Waxman, Aaron P. Rapoport, Michael C. Milone, Carl H June, J. Joseph Melenhorst
Journal: Blood (2018)
Dysregulated protocadherin-pathway activity as an intrinsic defect in induced pluripotent stem cell–derived cortical interneurons from subjects with schizophrenia
Authors: Zhicheng Shao, Haneul Noh, Woong Bin Kim, Peiyan Ni, Christine Nguyen, Sarah E. Cote, Elizabeth Noyes, Joyce Zhao, Teagan Parsons, James M. Park, Kelvin Zheng, Joshua J. Park, Joseph T. Coyle, Daniel R. Weinberger, Richard E. Straub, Karen F. Berman, Jose Apud, Dost Ongur, Bruce M. Cohen, Donna L. McPhie, Judith L. Rapoport, Roy H. Perlis, Thomas A. Lanz, Hualin Simon Xi, Changhong Yin, Weihua Huang, Teruyoshi Hirayama, Emi Fukuda, Takeshi Yagi, Sulagna Ghosh, Kevin C. Eggan, Hae-Young Kim, Leonard M. Eisenberg, Alexander A. Moghadam, Patric K. Stanton, Jun-Hyeong Cho, Sangmi Chung
Journal: Nature Neuroscience (2019)
AB0391 Clinical, serological and treatment analysis of rhupus syndrome: a retrospective monocentric study
Authors: P. Rottenberg, F. Jouen, I. Marie, T. Lequerré, O. Vittecoq
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Rhupus syndrome is a rare condition in which rheumatoid arthritis (RA) features overlap with systemic lupus erythematosus (SLE) manifestations. Disease characterisation tends to improve, but some uncertainties remain, and therapeutic management has still to be defined. <h3>Objectives</h3> We aimed to perform a study of the patients followed at the Rouen University Hospital with rhupus diagnosis using both recent criteria and expert diagnosis, in order to perform an analysis of the clinical, radiological and biological characteristics, and to report the therapeutic procedures carried out. <h3>Methods</h3> We performed a retrospective research work covering a period of 10 years, using diagnosis code extraction for both RA and SLE from database of the medical information department, as well as biological data extraction concerning ACPA testing, anti-dsDNA and anti-RNP antibodies. Patients satisfying both ACR/EULAR 2010 and SLICC 2012 classification criteria for RA and SLE respectively, or with an expert diagnosis of rhupus were included. A screening of medical records was performed to collect clinical, biological and treatment data for each patient. <h3>Results</h3> Sixteen patients were identified and 12 fulfilled both classification criteria. RA most often preceded rhupus, and clinical analysis found a predominant articular pattern at initial and established disease, with erosive arthropathy (n=11). Skin involvement was the most frequent associated manifestation (n=12). Among other associated manifestations, serious events were reported, including active glomerulonephritis (n=2), ischaemic stroke (n=1) and myocardial infarction (n=1). Immunological profiles showed positivity for (n=14), anti-dsDNA (n=9) and ACPA (n=9). Ten patients required biological DMARD, in addition to conventional treatment. All types of RA approved bDMARD were used. Rituximab was the most prescribed (n=9) and the most effective, with sustained response in 5 patients. <h3>Conclusions</h3> Rhupus is a rare condition, with predominant articular pattern, but serious SLE-related manifestations can occur. In rhupus refractory to conventional treatment, biologics, and particularly rituximab, are a promising therapeutic approach. <h3>Disclosure of Interest</h3> None declared
OP0239 Pregnancy outcomes in patients with iga vasculitis
Authors: J Nossent, W. Raymond, H. Keen, C. Inderjeeth, D. Preen
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> IgA vasculitis (IgAV) is usually a self-limiting condition, but women with a history of IgAV are reported to have an increased risk of complications during pregnancy. There is however little international data regarding obstetric outcomes and risk factors for IgAV patients <sup>1</sup>. <h3>Objectives</h3> To compare pregnancy outcomes between matched controls and patients hospitalised for IgA vasculitis in Western Australia (WA), where birth centres attached to public hospitals are the principal source for delivery of free midwifery-led care. <h3>Methods</h3> Cohort study of IgAV patients (ICD-9-CM 287.0/ICD-10-AM D69.0) using non-exposed age-matched controls (1:3), where pregnancy-related events and outcomes (see table 1 for ICD codes) were extracted from state-wide hospital morbidity data for period 1980 to 2015. Results are presented as odds ratios (95% CI) versus controls. <h3>Results</h3> Pregnancy-related contacts (n=1,440) occurred in 49.5% of all female IgAV patients (n=347) and in 37% of all female controls (n=914). IgAV patients were younger at first pregnancy (23.7 vs 26.4 years, p&lt;0.01) and had a higher overall mean number of hospital pregnancy contacts than controls (5.4 vs 3.1, p&lt;0.001). IgAV patients experienced 168 uncomplicated live births, 93 complicated (including preterm) deliveries and 32 abortive pregnancies. The risk for abortion (OR 1.3, CI: 0.7 to 2.3), complicated delivery (OR 0.9, CI: 0.7 to 1.3) or classification as high-risk pregnancy (OR 0.94, CI: 0.74 to 1.24) was similar for IgAV patients compared to controls, despite a higher risk of hypertensive disorders (OR 4.4 CI2.1–9.1). <h3>Conclusions</h3> In this large population-based longitudinal study, the risk for abortion or complicated pregnancies/deliveries was not increased for IgAV patients. The more frequent hospital contacts and increased risk of hypertension during pregnancy in IgAV patients had little impact on pregnancy outcome. <h3>Reference</h3> [1] Tayabali S, Andersen K, Yoong W. Diagnosis and management of Henoch-Schonlein purpura in pregnancy: A review of the literature. Arch Gynecol Obstet2012;286:825–29. <h3>Acknowledgements</h3> Supported by an unrestricted grant from the Arthritis Foundation of Western Australia. We acknowledge the contribution by Data Linkage WA. <h3>Disclosure of Interest</h3> None declared
THU0103 Evaluation of serum Β-2 microglobulin levels and its relationship with disease activity in rheumatoid arthritis
Authors: A.B. Kelesoglu Dincer, M. Torgutalp, E.G. Aydemir Gülöksüz, M.E. yayla, E. Uslu Yurteri, I.E. Okatan, S. Sezer, G. Kinikli, T.M. Turgay, A. Ateş
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> β−2 microglobulin (β2MG) is produced and secreted from T and B lymphocytes. In autoimmune diseases, due to lymphocyte activation and proliferation, serum β2MG level is expected to be higher than normal. In a few studies, increased serum and synovial fluid β2MG levels in rheumatoid arthritis (RA) were reported. <h3>Objectives</h3> The aim of this study was to evaluate the concentration of β2MG in peripheral blood in RA patients and to show its relationship with disease activity. <h3>Methods</h3> In this study, 121 RA patients and 50 sex and age matched healthy controls were enrolled. Patients who had other inflammatory diseases or any kind of malignancy were excluded. <h3>Results</h3> The mean age of RA patients (97 female and 24 male) were 55 years (±10.9 years) and mean disease duration was 9 years (min: 6 months, max: 42 years). The demographic data of patients and healthy controls are shown in table 1. There was a statistically significant difference between haemoglobin, erythrocyte sedimentation rate and C-reactive protein levels between two groups (p&lt;0.001,p&lt;0.001,p=0.001 respectively). Serum β2MG levels were higher in RA group than healthy controls.(p&lt;0.001) When RA patients were grouped according to disease activity as remission to low disease activity(DAS28-ESR&lt;3.2) (n=65) and moderate to severe disease activity(DAS28-ESR≥3.2) (n=56), the serum β2MG levels were higher in DAS28-ESR≥3.2 group than in DAS28-ESR&lt;3.2 group (p=0.003). The difference between serum β2MG levels were statistically significant in low and high disease activity groups when grouped both according to DAS-ESR and DAS-CRP(p=0.003 and p=0.006, respectively). The seropositivity between two groups were similiar (p=0.385), although serum ESR, CRP levels and the rate of morning stiffness were significantly higher in high disease activity group. The results were shown in table 2. According to Spearmann correlation test, the serum β2MG level and DAS-ESR and DAS-CRP were positively correlated which were both statistically significant. (r=0.378, p&lt;0.001 and r=0.324, p&lt;0.001 respectively). <h3>Conclusions</h3> Our results show that serum β2MG concentration increase in RA and is higher in active patients. It can be concluded that serum β2MG may be an appropriate parametre to monitor disease activity in rheumatoid arthritis. <h3>Disclosure of Interest</h3> None declared
THU0426 Ethnic variation in systemic sclerosis morbidity and mortality
Authors: H. Al-Sheikh, Z. Ahmad, S.R. Johnson
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Systemic sclerosis (SSc) is an uncommon connective tissue disease characterised by pathological skin thickening and can involve multiple internal organs. Ethnic variations in SSc have been reported in clinical manifestations, severity of the disease as well as survival. <h3>Objectives</h3> Our aim was to compare the survival and disease manifestations across ethnicity among SSc patients. <h3>Methods</h3> The Toronto Scleroderma Program is the largest single-centre, multiethnic, longitudinal SSc cohort in Canada. Patients are followed every 6 to 12 months using a standardised protocol. Patients who fulfilled the American College of Rheumatology-European League Against Rheumatism classification criteria for SSc and are 16 years of age or older were included in our retrospective cohort study. The study period was 1970–2017. Ethnicity was self-reported and was categorised as: Caucasian, African-American, Hispanic, Arab, East-Asian, First Nations or Persian. The primary outcome was the time from diagnosis to death from all causes. Secondary outcomes were differences in disease duration, SSc subtype, clinical manifestations, and serology. Survival probabilities and median survival times were determined using Kaplan-Meier survival curves. Cox proportional hazard models were used to estimate adjusted survival. <h3>Results</h3> 1005 subjects were evaluated, the majority of whom were Caucasian (n=745 (74%), African-American n=58 (6%)), South Asian (n=69 (7%)), and East Asian (n=80 (8%)). Compared to Caucasians, East Asians less frequently had calcinosis (29% versus 9%, p=0.002), and esophageal dysmotility (88% versus 69%, p=0.002); African-Americans more frequently had interstitial lung disease (31% versus 53%, p=0.007); and First Nation subjects more frequently had diffuse cutaneous disease (35% versus 56%, p=0.02) and diabetes (5% versus 33%, p=0.03). There were no differences across ethnicities in the prevalence of pulmonary hypertension, renal crisis, or digital ulcers. We found no difference in the short-term survival across ethnicities. However, in the long-term, there was trend for Hispanic subject to have better survival (81.3% (95%CI 63, 100), while First Nations (58.3% (95%CI 25, 100) and South Asian subjects (52.6% (95%CI 32, 87) had worst survival at 15 years and 20 years, respectively. East Asians appear to have the longest median survival time 43.3 years. <h3>Conclusions</h3> Ethnic variations in disease SSc disease manifestations are observed. However, in the setting of a universal health care system, this does not result in significant differences in survival. <h3>Disclosure of Interest</h3> None declared
THU0463 Complement factors of the alternative pathway in gpa and mpa
Authors: S. Fukui, K. Ichinose, K.-E. Sada, M. Harigai, A. Kawakami
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> In antineutrophil cytoplasm autoantibody (ANCA)-associated vasculitis (AAV), involvement of complements, especially alternative pathway of complement, has been reported in researches using mouse models. In human, while some studies have identified levels of C3 as a renal prognostic factor, entire complement factors in alternative pathway have not been evaluated. <h3>Objectives</h3> To evaluate complement profiles of AAV patients at diagnosis and at 6 months after treatments (Month 6). <h3>Methods</h3> In total, 91 incident cases of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) based on the European Medicines Agency algorithm were enrolled. They are a part of participants of the Japanese nationwide, prospective, inception cohort study from 22 tertiary Japanese institutions (RemIT-JAV-RPGN). Multiplex complement bead assays to measure levels of C1q, C2, C3, C3b/iC3b, C4, C4b, C5, C5a, C9, Factor D, Factor I, MBL, factor B, factor H, and properdin were performed using preserved sera at diagnosis and at Month 6. We compared complement levels at diagnosis of AAV patients with those of age- and sex-matched healthy donors, and compared those of the AAV patients at diagnosis and Month 6. <h3>Results</h3> Seventy-two of 91 patients had serum both at baseline and Month 6. Compared with healthy donors, GPA patients had significantly higher levels of C2, C5a, and Factor B, and significantly lower levels of C3b/iC3b, C4b, and properdin. MPA patients had significantly higher levels of C2 and Factor D, and significantly lower levels of C3b/iC3b, C4, C5, Factor H, and properdin (table 1). At baseline, GPA had significantly higher levels of C4, Factor B and Factor H, and had significantly lower levels of C4b and Factor D compared to MPA. There are no significant differences in levels of C3, C5, Factor D, MBL, and properdin using Wilcoxon signed-rank test between at diagnosis and Month 6 both in GPA and MPA. Factor I significantly decreased at Month 6 only in GPA. Other complement factors significantly decreased at Month 6 both in GPA and MPA. GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis; HD, health donor. <h3>Conclusions</h3> We found some differences in complement factors among GPA, MPA, and healthy donors. There were no differences of levels of C3, C5, Factor D, and properdin, which suggested involvements of alternative pathway, both in GPA and MPA between at diagnosis and Month 6. <h3>Disclosure of Interest</h3> None declared
Dietary Fructose and Microbiota-Derived Short-Chain Fatty Acids Promote Bacteriophage Production in the Gut Symbiont Lactobacillus reuteri
Authors: Jee-Hwan Oh, Laura M. Alexander, Meichen Pan, Kathryn L. Schueler, Mark P. Keller, Alan D. Attie, Jens Walter, Jan-Peter van Pijkeren
Journal: Cell Host & Microbe (2019)