tbuckley/cabot-search huggingface dataset:
AB0575 Retrospective survey of concomitant autoimmune diseases and autoantibodies in a cohort of patients with anca-associated vasculitis (AAV)
Authors: M Casal Moura, S Prieto-González, G Espígol-Frigolé, G Murgia, M Alba, J Hernández-Rodríguez, M Cid
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) – granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) have heterogenous clinic and biologic phenotypes. Sporadic reports indicate that some AAV patients may have other autoimmune diseases but the frequency of the association is unknown. <h3>Objectives</h3> Our purpose was to assess the frequency of other autoimmune diseases or autoantibodies in a well-defined cohort of AAV patients. <h3>Methods</h3> Retrospective survey of a cohort of patients regularly controlled at outpatient facility of an Autoimmune Disease Department in a 5-year period (2011–2016). Clinical and immunologic data were retrieved from electronic records. All patients were diagnosed and treated by the authors. <h3>Results</h3> We included 110 AAV patients – 36 (32.7%) GPA, 45 (40.9%) MPA and 29 (26.4%) EGPA. Regarding to ANCA specificity – 23 (20.9%) against proteinase 3 (PR3)-ANCA, 68 (61.8%) against myeloperoxidase (MPO)-ANCA and 19 (17.3%) were ANCA negative. Organ-specific auto-immune diseases was actively searched in 73 (81.1%) patients and was present in 43 (58.9%) patients. More prevalent autoimmune diseases diagnosed were: hypothyroidism (15,34.9%), pernicious anemia (12,27.9%), Sjögren syndrome (8,18.6%), hyperthyroidism (4,9.3%) and primary biliar cholangitis (3,7.0%). Autoantibodies more frequently positive were: ANAs 29 (67.4%), ASMA 27 (62.8%), anti-parietal cells 20 (46.5%), anti-thyreoglobulin 6 (14.0%), anti-peroxidase 12 (27.9%), anti-SSa 5 (11.6%), anti-SSb 3 (7.0%) and RF 14 (32.6%). Prevalence of organ-specific autoimmune disease was higher in MPA (67.4vs.32.6%,p=0.001) and ANCA-MPO carriers (81.4vs.18.6%,p&lt;0.001). Pernicious anemia was more prevalent in MPA (41.4vs.0%,p=0.027) and in ANCA-MPO (34.3vs.0%,p=0.054) and myositis was more prevalent in ANCA-PR3 (12.5vs.0%,p=0.037). ANAs positivity was more prevalent in ANCA-MPO carriers (74.3%vs.36.5%,p=0.048). Predictive factors for development of organ-specific autoimmune disease identified were: female gender (OR 2.710,95% CI 1.034–7.099,p=0.043), MPA syndrome (OR 3.578, 95% CI 1.334–9.523,p=0.011) and MPO carriers (OR 3.346, 95% CI 1.166- 9.601,p=0.025). <h3>Conclusions</h3> A substantial percentage of AAV patients, particularly MPA and anti MPO carriers, have associated autoimmune diseases and autoantibodies. The limitations of our study (retrospective assessment and lack of comparator) do not allow accurate estimation of prevalence. The severity of AAV and difficulties in management, may lead to overlooking of associated autoimmune diseases which appear to be frequent. Associated autoimmune disease may contribute to additional burden in AAV patients. (Supported by SAF 2014 57708-R). <h3>Disclosure of Interest</h3> None declared
SP0061 A day in the neutrophil's life
Authors: A Hidalgo
Journal: Annals of the Rheumatic Diseases (2017)

Immunity is essential for life, yet the strength of immune responses are not constant throughout the day. This oscillatory immunity reflects an adaptation of organisms to environmental changes that occur through day-night cycles, so as to optimize and concentrate effective responses to the times of maximal environmental threat. In my talk I will discuss our ongoing efforts to uncover the mechanisms by which neutrophils, the most abundant and aggressive of all immune cells, orchestrate temporal immunity. These mechanisms are reflected in diurnal changes in phenotype and function of neutrophils, which we refer to as neutrophil aging. We propose that the existence of a timed response of neutrophils governed by cell-intrinsic and –extrinsic mechanisms suggests that inflammatory disease co-opts ancestral processes to damage tissues. <h3>Disclosure of Interest</h3> None declared
Randomized phase III study of etoposide plus cisplatin versus irinotecan plus cisplatin in advanced neuroendocrine carcinoma of the digestive system: A Japan Clinical Oncology Group study (JCOG1213).
Authors: Chigusa Morizane, Nozomu Machida, Yoshitaka Honma, Takuji Okusaka, Narikazu Boku, Ken Kato, Junki Mizusawa, Hiroshi Katayama, Nobuyoshi Hiraoka, Hirokazu Taniguchi, Masafumi Ikeda, Yuichi Shibuya, Ayumu Hosokawa, Nobumasa Mizuno, Takeshi Sano, Masahiro Tsuda, Osamu Yokosuka, Yuko Kitagawa, Mitsuru Sasako, Junji Furuse
Journal: Journal of Clinical Oncology (2015)

TPS4143 Background: According to the World Health Organization grading system for neuroendocrine neoplasms, neuroendocrine carcinoma (NEC) is a poorly differentiated, high-grade malignant tumor, including small and large cell carcinoma. The primary sites of NEC vary, and NEC arising in the digestive system accounts for 20%–68% of extra-pulmonary NEC. Treatment guidelines for advanced extra-pulmonary NEC recommend platinum-based chemotherapy regimens, which are suitable for small-cell lung carcinoma. We previously reported that etoposide plus cisplatin (EP) and irinotecan plus cisplatin (IP) are commonly used as community standard regimens for NEC of the digestive system in Japan (Yamaguchi et al. Cancer Sci. 2014;105(9)). However, no randomized controlled trial has ever been conducted, and it is unknown which is more effective. This phase III study compares EP and IP in terms of overall survival for patients with recurrent or unresectable NEC of the digestive system. Methods: Eligibility criteria include chemotherapy-naïve patients with recurrent or unresectable, histologically or cytologically confirmed NEC of the digestive system, an ECOG PS of 0-1, age of 20–75 years, and no history of platinum agents. Eligible patients are randomized into the EP arm or the IP arm. In the EP arm, 100 mg/m2 of etoposide on days 1, 2, and 3 and 80 mg/m2 of cisplatin on day 1 are infused every 3 weeks. In the IP arm, 60 mg/m2 of irinotecan on days 1, 8, and 15 and 60 mg/m2 of cisplatin on day 1 are infused every 4 weeks. The primary endpoint is overall survival. Due to scarcity of this disease, we set the sample size to be 140 (70 patients per arm), based on the assumption of median survival time (MST) of 8 months by the inferior regimen and 12 months by the superior regimen (6 years accrual, 1 year follow-up), with a two-sided alpha of 10%, a power of ≥ 70%. Seventy-eight institutions belonging to JCOG are participating in this study (UMIN000014795). This study was activated in Aug 2014 and the current enrollment is 17 as of Jan 2015. Clinical trial information: UMIN000014795.
AB0275 The correlation of cartilage oligomeric matrix protein with sonographic knee cartilage thickness and disease characteristics in rheumatoid arthritis
Authors: S Rajalingham, S Rajalingam, H Hussein, R Sridharan, AA Wahab
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Cartilage Oligomeric Matrix Protein (COMP) is an extracellular protein which is primarily found in the cartilage and to a lesser extent in ligaments, meniscus, tendons and synovium. Experimental models of rheumatoid arthritis (RA) and osteoarthritis have pointed out that serum COMP levels are reflective of the cartilage turnover rate. <h3>Objectives</h3> To investigate the correlation of serum COMP levels with the articular cartilage damage based on sonographic knee cartilage thickness (KCT) and disease characteristics in RA. <h3>Methods</h3> A total of 61 RA patients and 27 healthy controls were recruited in this study. Serum samples were obtained from all subjects to determine the COMP levels. All subjects had bilateral ultrasound scan of their knees performed by a single radiologist; who was blinded to the details of the subjects. The KCT was based on the mean of measurements at 3 sites; the medial condyle, lateral condyle and intercondylar notch (Figure 1). Besides, the RA patients were assessed for their disease activity based on DAS 28. <h3>Results</h3> Serum COMP concentrations were significantly elevated in the RA patients compared to the controls (p=0.001). The serum COMP levels had an inverse relationship with bilateral KCT in RA subjects and the healthy controls. However, the association was statistically insignificant for bilateral knees in the control arm. COMP correlated significantly with disease activity based on DAS 28 (r =0.299, p=0.010), disease duration (r =0.439,p = &lt;0.05) and mean left KCT (r = -0.285,p=0.014) in RA (Table 1). Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) which are the traditional markers of inflammation; demonstrated a significant positive correlation with the DAS 28 scores (r =0.372, p=0.003 for ESR; r =0.305, p=0.017 for CRP) comparable to the serum COMP. However, neither ESR nor CRP had a significant association with the KCT, as opposed to the serum COMP. <h3>Conclusions</h3> The serum COMP is a promising biomarker in RA which reflects disease activity and damage to the articular cartilage. Serum COMP appeared superior to the traditional markers in RA i.e ESR and CRP in predicting sonographic KCT. <h3>References</h3> Andersson ML, Svensson B, Petersson IF, et al. Early increase in serum-COMP is associated with joint damage progression over the first five years in patients with rheumatoid arthritis. BMC Musculoskelet Disord. 2013;14:229. Happonen KE, Saxne T, Aspberg A, et al. Regulation of complement by cartilage oligomeric matrix protein allows for a novel molecular diagnostic principle in rheumatoid arthritis. Arthritis Rheum. 2010;62:3574–83. Happonen KE, Saxne T, Geborek P, et al. Serum COMP-C3b complexes in rheumatic diseases and relation to anti-TNF-alpha treatment. Arthritis Res Ther. 2012;14:R15. <h3>Disclosure of Interest</h3> None declared
SAT0428 Which criteria for inflammatory back pain in spondyloarthritis are optimal? data from the screening for axial spondyloarthritis in psoriasis, iritis, and colitis study (SASPIC)
Authors: R Carmona, J Yeung, A Masetto, L Martin, S Keeling, J Chan, D Mosher, O Ziouzina, J Paschke, A Carapellucci, RG Lambert, WP Maksymowych
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Criteria for inflammatory back pain (IBP) in spondyloarthritis (SpA) include the Calin, Berlin, and Assessments in SpA International Society (ASAS) criteria, although no studies have undertaken comparative validation to determine which are optimal versus the rheumatologist expert opinion gold standard assessment. <h3>Objectives</h3> We aimed to compare IBP criteria in unselected patients presenting with undiagnosed back pain to rheumatology practice. <h3>Methods</h3> The Screening for Axial Spondyloarthritis in Psoriasis, Iritis, and Colitis (SASPIC) Study is aimed at the development and validation of a triage strategy for detection of axial SpA in patients presenting with undiagnosed back pain. Consecutive patients ≤45 years of age with ≥3 months undiagnosed back pain with any one of psoriasis, acute anterior uveitis, or colitis diagnosed by the relevant specialist undergo routine clinical evaluation by a rheumatologist for axial SpA. The rheumatologist determines the presence or absence of IBP (defined as &lt;5 or &gt;5 on 0–10 scale, respectively) and axial SpA (yes/no) after the clinical evaluation and review of labs (B27, CRP) and imaging (x-ray, MRI). Clinical and laboratory data, radiographs and MRI scans are also assessed centrally for diagnosis of axial SpA (yes/no). We assessed sensitivity and specificity of each of the IBP criteria for diagnosis of IBP according to the local rheumatologist, diagnosis of axial SpA by local rheumatologist, diagnosis of axial SpA by central assessment, and concordant diagnosis of axial SpA by both local and central assessment. <h3>Results</h3> We recruited 185 patients (48.6% male, mean age 27.6 years, mean back pain duration 7.0 years) with iritis (30.3%), psoriasis (17.3%), Crohn9s (38.4%) and ulcerative colitis (18.9%). IBP and/or axial SpA were considered present in 65.2% and 47.3%, respectively, of all patients by the local rheumatologist. By central assessment axial SpA was considered present in 32.7% while amongst patients with a concordant diagnosis by both local and central assessment 37.2% were considered to have axial SpA. Sensitivity was comparatively high for all IBP criteria but specificity was poor. The Berlin criteria consistently performed best while the Calin criteria consistently performed worst (Table). <h3>Conclusions</h3> IBP criteria lack specificity for rheumatologist diagnosed IBP or axial SpA but the Berlin criteria have consistently better performance. <h3>Disclosure of Interest</h3> None declared
AB0411 Comparison of tuberculin skin test and interferon-gamma release assay screening in patients with rheumatoid arthritis starting anti-tumor necrosis factor therapy
Authors: DT Archimandriti, C Iliou, AT Tzallas, E Pelechas, AA Drosos, PV Voulgari
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Biologic agents provide clinical benefits in patients with Rheumatoid Arthritis (RA) but they are associated with an increased risk of tuberculosis (TB) infection. <h3>Objectives</h3> We investigated the accordance between the tuberculin skin test (TST) and the interferon-gamma (IFN-γ) release assay (IGRA) for the diagnosis of latent tuberculosis infection (LTBI) in patients with RA. The objective of our study was a direct comparison between the IGRA and the TST regarding LTBI diagnosis in RA patients before starting anti-TNF agents and at least one year after the initiation of treatment. <h3>Methods</h3> RA consecutive patients have been included in the study both before and after 1 year of the initiation of TNF-α antagonist therapy. A standard questionnaire was completed for each patient, including basic demographic data and concurrent immunosuppressive therapy (corticosteroids, disease-modifying anti-rheumatic drugs [DMARDs], synthetic and biologics). The patients were screened for LTBI using IGRA and TST methods. All analyses were performed with IBM-SPSS Statistics for Windows (version 21). The kappa coefficient (κ) was calculated to determine the concordance between the two tests: TST and IGRA. The strength of the agreement was considered "poor" for κ ≤0.20, "low-moderate" for 0.20 &lt; κ ≤0.40, moderate for 0.40 &lt; κ ≤0.60, "substantial" for 0.60 &lt; κ ≤0.80, and "optimal" for 0.80 &lt; κ ≤1. <h3>Results</h3> 78 patients (67 female, 11 male) have been screened with TST and IGRA. The accordance between TST and IGRA was 89.7% (κ 0.514, κ&lt;0.001). A κ value of 0.514 represents a moderate accordance between the two tests. 6 female patients converted TST (4 became positive, and 2 became negative). No patient developed active tuberculosis. <h3>Conclusions</h3> A moderate accordance between the two methods (TST and IGRA) was obtained. In the absence of a diagnostic gold standard for LTBI, different issues should be taken into consideration regarding the two methods (cost, laboratory expertise and equipment etc). More studies are needed in the field in order to elucidate the cost-effectiveness but also the appropriateness of both methods. <h3>Disclosure of Interest</h3> None declared
FRI0468 Radiographic hip involvement in patients with ankylosing spondylitis. a study of its prevalence and determining factors
Authors: X Michelena, M Lόpez de Recalde, H Borrell, J Lluch, P Juárez, I Morales, M Pascual, J-M Nolla, X Juanola
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Hip involvement in Ankylosing Spondylitis (AS) is the most frequent extraspinal manifestation and a common cause of disability and limited mobility, often ending with hip replacement. There are few studies analysing the radiographic changes of the hip and their relationship with other disease variables. <h3>Objectives</h3> To determine the factors associated with radiographic hip involvement and its prevalence. <h3>Methods</h3> A cross-sectional study was performed based on patients with AS, excluding the patients with associated psoriasis or inflammatory bowel disease. To assess radiographic hip involvement, we scored the last anteroposterior pelvic radiograph performed using the <i>Bath Ankylosing Spondylitis Radiology Index</i> (BASRI). Demographic, clinical, laboratory and radiographic data were collected and analysed. We considered the presence of hip disease with a BASRI-hip grade of at least 2. Statistical analysis: A descriptive study was performed. To compare differences we used a 1-way ANOVA test and Pearson chi-square. The statistical analyses were done using SPSS 24.0. p≤0.05 was considered statistically significant. <h3>Results</h3> 215 patients were identified, with a mean age of 52±13.6 years and 76.7% of the patients were male. The age at onset was 25.29±8.22 years. 86.4% of the patients were HLA-B27-positive. Regarding their treatment, 27.9% were under biological therapy. A hip replacement was needed in 14 patients (0.7%), with 6 of them requiring a bilateral hip replacement. The mean BASDAI score, BASFI score and ASDAS CRP index were 4.01±6.31; 4.73±8.8 and 2.17±1.01 respectively. The table shows the comparison between the two groups based on the presence of hip involvement (BASRI hip ≥2). Statistically significant differences were observed in the age, age at onset and presence of peripheral arthritis. Patients with hip involvement had higher scores in BASFI score, ASDAS CRP and ESR. Axial radiographic involvement assessed with axial BASRI score and total SASSSm was associated with hip disease and a significant association was seen between hip involvement and metrological parameters. <h3>Conclusions</h3> Radiographic hip involvement assessed with BASRI score is prevalent and it is related with the age at onset, disease activity and the presence of peripheral arthritis. These patients present more disability, less mobility and greater axial radiographic involvement. <h3>Disclosure of Interest</h3> None declared
FRI0391 A perfusion-metabolic mismatch in 99MTL and 123I-BMIPP scintigraphy predicts worse prognosis in systemic sclerosis patients with asymptomatic cardiac involvement
Authors: H Iida, H Hanaoka, T Kiyokawa, Y Takakuwa, T Okazaki, H Yamada, S Ozaki
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Background</h3> Cardiac involvement is a manifestation of systemic sclerosis (SSc) that contributes to significant mortality and morbidity. Since many SSc patients with cardiac involvement are asymptomatic and it may progress silently, early diagnosis is still challenging. The perfusion-metabolic mismatch in cardiac scintigraphy indicates functional abnormality due to myocardial injury and has been expected to detect early cardiac involvement in various diseases. However, its clinical utility has been poorly evaluated in patients with SSc. <h3>Objectives</h3> To evaluate clinical utility of perfusion and myocardial fatty acid metabolism mismatch in SSc patients without cardiac failure. <h3>Methods</h3> All patients who visited St. Marianna University Hospital from 2009 to 2015 and performed cardiac scintigraphy using <sup>99m</sup>Tl and <sup>123</sup>I-BMIPP were retrospectively evaluated. Patients who fulfilled American Collage of Rheumatology classification criteria of SSc and systemic lupus erythematosus (SLE) were selected. We subtracted %uptake of metabolism (<sup>123</sup>I-BMIPP) from that of perfusion (<sup>99m</sup>Tl) on each 17 myocardial segments standardized by American Heart Association. We compared sum of all the scores and each score in 3 coronary artery regions between patients with SSc and SLE. Furthermore, we evaluated incidence of cardiac death or cardiac failure depending on the scores of each coronary artery regions. <h3>Results</h3> Among 177 patients, we analyzed the data in 22 cases with SSc and 23 with SLE. The sum of all mismatch scores was not significantly different between the 2 groups (p=0.37). The mismatch score of left anterior descending coronary artery (LAD) region was significantly higher in SSc than SLE (p=0.05) (Figure 1A). We next divided SSc patients into 2 groups depending on degree of the LAD mismatch score and found the group with low score had higher incidence of cardiac death or cardiac failure (p=0.04) than that with high score (Figure 1B). <h3>Conclusions</h3> The low mismatch score in LAD region of cardiac scintigraphy could be associated with cardiac death or cardiac failure in SSc patients. Since myocardial fibrosis might replace viable cardiomyocytes leading to loss of ventricular volume, metabolic defect by scintigraphy might be less detectable. <h3>Disclosure of Interest</h3> None declared
THU0021 The analysis of PRF1, STX11, UNC13D and STXBP2 gene in systemic juvenile idiopathic arthritis with macrophage activation syndrome
Authors: P Wei, H Zeng
Journal: Annals of the Rheumatic Diseases (2017)

<h3>Objectives</h3> We investigated the clinical characteristics of 34 cases with systemic juvenile idiopathic arthritis (SJIA) and macrophage activation syndrome (MAS) and analyzed PRF1, UNC13D, STX11 and STXBP2 gene so as to figure out the genetic pathogenic mechanism. <h3>Methods</h3> The clinical characteristics of 34 cases with sJIA and MAS were retrospectively analyzed, and the coding sequences of PRF1, UNC13D, STX11 were amplified. DNAMAN software and NCBI BLAST were used to compare sequences. The chi-square test was employed to compare the distribution of allele and genotype frequencies between SLE patients and healthy controls. <h3>Results</h3> Among the 34 cases, 69% were males, and the average age was 6 years. 85% (29/34 cases) of the cases underwent genetic testing and four SNPS loci were detected: PRF1 c. 1061 c &gt; T (rs885822); UNC13D c. 659 c &gt; T (rs3744007); STXBP2 c. 1483 T &gt; c (rs10001) and STXBP2 c. 1616 A &gt; G (rs6791). Compared with control group, genotype and allele frequencies of PRF1 rs885822 and STXBP2 rs10001 among the MAS cases were of significant difference (rs885822: allele frequency 2 =4.52, p=0.03; genotype frequency: 2 =5.52, p=0.02. rs10001: allele frequency 2 =21.33, p=0.00; genotype frequency: 2 =19.58, p=0.00). There were no significant differences in genotype and allele frequencies of UNC13D rs3744007 and STXBP2 rs6791 between MAS group and control group (rs3744007: allele frequency 2 =1.89, p=0.17; genotype frequency: 2 =1.59, p=0.45. rs6791: allele frequency 2 =1.69, p=0.19; genotype frequency: 2 =2.09, p=0.35). The main characteristics of the cases were persistent fever, progressive hepatosplenomegaly, a sharp decline in blood cells, effusion, significantly increased serum liver enzymes, hyperlipidemia, etc. Some children had mucosal bleeding, neurological dysfunction, etc. More than 82% children had upper respiratory tract infection before MAS onset. 90% of children had remission, while three cases developed multiple organ failure and died. <h3>Conclusions</h3> Macrophage activation syndrome is fatal complication caused by immune imbalance. Early detection and treatment is the key to improve the prognosis. The SNP PRF1 rs885822 and STXBP2 rs1001 may be concurrent with the pathogenesis of SJIA-MAS. The SNP UNC13D rs3744007 and STXBP2 rs6791 may not play a role in SJIA-MAS. <h3>Disclosure of Interest</h3> None declared