tbuckley/cabot-search huggingface dataset:
POS1319 RAYNAUD’S PHENOMENON IN A SINGLE CENTER COHORT OF TURKISH CHILDREN
Authors: Ş. Çağlayan, F. Demir, B. Sancar, K. Ulu, T. Coşkuner, B. Sözeri
Journal: Annals of the Rheumatic Diseases (2021)

Background: Raynaud’s phenomenon (RF) is a vasospastic condition characterized by episodic color changes of blanching, cyanosis, and hyperemia of the extremities of the body Objectives: We aim to examine the clinical presentation, capillaroscopic findings and disease associations of Raynaud’s phenomenon (RP) in pediatric patients Methods: We retrospectively enrolled a single-center cohort of 247 consecutive pediatric patients with RP admitted to Umraniye Training and Research Hospital, Pediatric Rheumatology Clinic, Istanbul, Turkey, since 2016. Medical records were analyzed for clinical presentation, disease associations, and physical examination and laboratory findings. Results: We reported 247 patients (152 female, 95 male) with RP. Their mean age at disease onset was 14.5±2.55 years and median age at diagnosis was 15.4± 2.21 years. In patients with secondary RP syndrome (10.9%), the age of complaint was found to be lower than others (p=0.03). Although most patients have affected upper extremity (hand and fingers), lower extremity (toes) involvement was also detected in 50 (20%) patients. In both the patients with primary and secondary RP, main triggering factor was found as cold exposure. Biphasic (53.6%) color changes were more seen in primary RP, although triphasic (51.8%) color changes were more in patients with secondary RP (p=0.00). The presence of antinuclear antibodies and abnormal nailfold capillaries were more seen in patients with secondary RP (28% vs 74% and 35.3% vs 66.7, respectively p=0.00). Digital ulcer was detected 5.2% of patients (mostly secondary). Out of 247, 31 patients were treated with low-dose aspirin, 47 with calcium blockers, 25 with low-dose aspirin and calcium blockers, 16 with iloprost and/or bosentan, while the remaining 140 did not receive any drug. Conclusion: Similar to adults, RP is more common in girls without an underlying disease. In patients with secondary RP, the symptoms. It has been shown that in patients with secondary RP, symptoms begin at a younger age and the ANA positivity and abnormal nailfold capillaries correlate. References: [1]Jones G, et al. Arthritis Rheum 2003;48:3518–352 [2]Garner R et al. BMJ Open . 2015; 5(3): e006389 [3]Nigrovic PA et al. Pediatrics 2003, 111 (4) 715-721 Disclosure of Interests: None declared
Update on the Diagnosis and Treatment of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome
Authors: Jack Khouri, Megan Nakashima, Sandy Wong
Journal: JAMA Oncology (2021)

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare plasma cell disorder characterized by demyelinating peripheral neuropathy and clonal plasma cell proliferation. Clinical manifestations are believed to be associated with a surge of inflammatory and angiogenic mediators, including interleukins and vascular endothelial growth factor (VEGF), elicited by clonal and polyclonal plasma cells. The clinical manifestations of POEMS syndrome can be debilitating; therefore, early diagnosis is essential. This review discusses several aspects of POEMS syndrome and includes the most recently published findings, with a special emphasis on diagnosis and treatment strategies.POEMS syndrome may be underdiagnosed because of its rarity, and it can be mistaken for chronic inflammatory demyelinating polyneuropathy; this misdiagnosis may lead to delayed therapy and progressive worsening of symptoms, especially neuropathy. Therefore, in addition to measurement of the VEGF level, patients with a monoclonal protein detected in blood and/or urine and neuropathy should be evaluated for POEMS syndrome with use of imaging to assess whether sclerotic bone lesions, effusions, and organomegaly are present. Clinical trials are scant, and treatment is largely based on small case series in which plasma cell-directed therapies, borrowed from the myeloma armamentarium, were used. High-dose melphalan and autologous hematopoietic cell transplantation may be offered to eligible patients. Lenalidomide and dexamethasone can be prescribed for patients who are ineligible for transplants. The main goals of therapy are to attain complete hematologic and VEGF responses and to reduce symptoms, although it may take up to 3 years for neurologic deficits to be ameliorated.POEMS syndrome should be considered in the differential diagnosis for patients who have peripheral neuropathy and paraproteinemia among other multisystem manifestations. The syndrome can be debilitating if not recognized early in its course; thus, appropriate diagnosis and treatment are important for optimal clinical outcomes.
AB0246 COMORBIDITIES APPEARING UNDER BIOLOGIC THERAPY: PREVALENCE AND ASSOCIATED FACTORS
Authors: T. El Joumani, T. Latifa, I. Hmamouchi, S. Ahid, R. Abouqal, L. Achemlal, I. El Bouchti, A. El Maghraoui, I. Ghozlani, H. Hassikou, T. Harzy, I. Linda, O. Mkinsi, R. Niamane, R. Bahiri, H. Rkain, F. Allali
Journal: Annals of the Rheumatic Diseases (2021)

Objectives: The aims of our study are to determine the new comorbidities appearing under biologic therapy, their prevalence, and the factors implicated in their appearance. Methods: It’s a multicentric historical-prospective cohort including 10 rheumatology departments of Moroccan University Hospitals. The data were collected from the national register of patients under biologic therapy supervised by the Moroccan Society of Rheumatology. An electronic follow-up questionnaire is completed every 6 months by the investigator. Results: The study included 418 patients: 224 with Rheumatoid Arthritis (RA) who represented 53.6% and 194 with spondyloarthropathy (SP) who represented 46.4%. The prevalence rate of comorbidities appearing after one year of treatment with biologic therapy was 15.7% in RA and 8.4% in SP. The rate of cardiovascular diseases was 12.9% (arterial hypertension, myocardial infarction and/or ischemic stroke), 6.4% was the same value of hypercholesterolemia and depression, diabetes 3.3%, ulcer 3.2%, and osteoporosis 9.7%. The sedentary rate was 54.8% and smoking was about 3.3%. No patient had developed hypertriglyceridemia or chronic obstructive pulmonary disease. In the group of patients with RA, the average age of the patients who had developed a new comorbidity was 51,8 ± 11,3 years, women represented 87.5%, the average of Body Mass Index (BMI) was 27,6 ± 5,9 and the average duration of the disease was 14,1 ± 9,2 years. The disease activity score (DAS28) had an average of 3,15 ± 1,47. 59.6% of patients used Rituximab, 23.8% Tociluzimab, 8.1% Etanercept, 5.8% Adalimumab, 0.18% Infliximab, and 0.9% Golimumab. In the group of patients with SP, the average age of the patients who had developed a new comobidity was 40,2 ± 13,7 years, men represented 63.4%, and the average of BMI was 24,3 ± 4,94. The activity of the desease, had an average Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) of 2,62 ± 1,79 and ankylosing Spondylitis Disease Activity Score (ASDAS) of 1,93 ± 1,09. Regarding the type of biologic therapy, 33.2% of patients used Etanercept, 30.1% Adalimumab, 24.9% Infliximab, 9.8% Golimumab, 1.6% Secukinumab, and 0.5% Tociluzimab. Conclusion: Our study showed a high prevalence of cardiovascular disease in patients under biologic therapy. This can be explained by the sedentary lifestyle secondary to rheumatic disease. Disclosure of Interests: None declared
POS0596 THE THERAPEUTIC TARGET IS REACHED MORE OFTEN IN RA PATIENTS STARTING TNF INHIBITORS WITH MODERATE ACTIVITY THAN WITH HIGH DISEASE ACTIVITY – AN ANALYSIS FROM THE ATTRA REGISTRY
Authors: K. Pavelka, L. Nekvindova, J. Zavada
Journal: Annals of the Rheumatic Diseases (2021)

Background: Treat to target guidelines recommend achieving remission or low disease activity in rheumatoid arthritis (RA). In the Czech Republic, anti-TNF therapy has been reimbursed only for patients with highly active RA until 2019, when pts with LDA have been also included in the reimbursement policy. Objectives: The aim of the study was to compare the results of treatment in patients with rheumatoid arthritis and moderate disease activity (MDA) or high disease activity (HDA) starting the therapy with the first anti-TNF drug in routine clinical practice. Methods: This was a retrospective cohort study of patients treated with the first anti-TNF drug in the Czech Republic. Propensity score was used to match pts starting anti-TNF in MDA or HDA for other important predictive factors. The propensity score was obtained by logistic regression with 10 covariates (gender, age, disease duration, seropositivity, comedication with MTX, comedication with other csDMARDs, number of previosu csDMARDs, anti-TNF drug),.Within the paired groups, one-dimensional logistic models were then calculated to estimate the odds of remission / low activity after 6 and 12 months of treatment. Results: A total of 2416 patients were analyzed in the study. 2231 patients had high activity (DAS 28&gt; 5.1) and 185 patients had moderate activity (3.2 &lt;DAS28 ≤ 5.1) at baseline. After 12 months, the low activity state (DAS28 &lt; 3.2) was achieved by patients with MDA in 74.0% and patients with HDA in 52.2%, (p &lt;0.001) (FIG 1). The differences btw groups were significant in all evaluated intervals of 0, 3, 6, 12 months. Furthermore, a comparison of patients with MDA and HAD by propensity score matching, who no longer differ in the baseline characteristics (except for disease activity and quality of life) was performed. Patients with MDA were 2.4x more likely to achieve remission or low activity according to DAS28-ESR after 6 months and 1.7x more likely to achieve remission or low activity after 12 months of treatment compared to patients with HDA at the beginning of treatment. Conclusion: This analysis of the results of treatment with anti-TNF drugs in the ATTRA Registry has shown that starting anti-TNF therapy in patients with RA and MDA leads more often to the target of remission or low activity, than in pts with HDA. Acknowledgement: Supported by the project of the Ministry of Health Care of the Czech Republic of the conceptual development of the research organization 00023728 Institute of Rheumatology Disclosure of Interests: Karel Pavelka Speakers bureau: Abbvie, Pfizer, Elli-Lilly, BMS, UCB, Sanofi, Novartis, Gilead, Paid instructor for: Abbvie, Consultant of: Abbvie, Pfizer, Elli-Lilly, BMS, UCB, Sanofi, Novartis, Gilead, Lucie Nekvindova: None declared, Jakub Zavada Speakers bureau: Abbvie, Elli-Lilly, UCB, Sanofi, Consultant of: Abbvie, UCB, Sanofi, Gilead
Targeted metabolomic profiling and prediction of cardiovascular events: a prospective study of patients with psoriatic arthritis and psoriasis
Authors: Keith Colaco, Ker-Ai Lee, Shadi Akhtari, Raz Winer, Paul Welsh, Naveed Sattar, Iain B McInnes, Vinod Chandran, Paula Harvey, Richard J Cook, Dafna D Gladman, Vincent Piguet, Lihi Eder
Journal: Annals of the Rheumatic Diseases (2021)

Objective In patients with psoriatic disease (PsD), we sought serum metabolites associated with cardiovascular (CV) events and investigated whether they could improve CV risk prediction beyond traditional risk factors and the Framingham Risk Score (FRS). Methods Nuclear magnetic resonance metabolomics identified biomarkers for incident CV events in patients with PsD. The association of each metabolite with incident CV events was analysed using Cox proportional hazards regression models first adjusted for age and sex, and subsequently for traditional CV risk factors. Variable selection was performed using penalisation with boosting after adjusting for age and sex, and the FRS. Results Among 977 patients with PsD, 70 patients had incident CV events. In Cox regression models adjusted for CV risk factors, alanine, tyrosine, degree of unsaturation of fatty acids and high-density lipoprotein particles were associated with decreased CV risk. Glycoprotein acetyls, apolipoprotein B and cholesterol remnants were associated with increased CV risk. The age-adjusted and sex-adjusted expanded model with 13 metabolites significantly improved prediction of CV events beyond the model with age and sex alone, with an area under the receiver operator characteristic curve (AUC) of 79.9 versus 72.6, respectively (p=0.02). Compared with the FRS alone (AUC=73.9), the FRS-adjusted expanded model with 11 metabolites (AUC=75.0, p=0.72) did not improve CV risk discrimination. Conclusions We identify novel metabolites associated with the development of CV events in patients with PsD. Further study of their underlying causal role may clarify important pathways leading to CV events in this population.
COVID-19 in Amazonas, Brazil, was driven by the persistence of endemic lineages and P.1 emergence
Authors: Felipe Gomes Naveca, Valdinete Nascimento, Victor Costa de Souza, André de Lima Corado, Fernanda Nascimento, George Silva, Ágatha Costa, Débora Duarte, Karina Pessoa, Matilde Mejía, Maria Júlia Brandão, Michele Jesus, Luciana Gonçalves, Cristiano Fernandes da Costa, Vanderson Sampaio, Daniel Barros, Marineide Silva, Tirza Mattos, Gemilson Pontes, Ligia Abdalla, João Hugo Santos, Ighor Arantes, Filipe Zimmer Dezordi, Marilda Mendonça Siqueira, Gabriel Luz Wallau, Paola Cristina Resende, Edson Delatorre, Tiago Gräf, Gonzalo Bello
Journal: Nature Medicine (2021)
AB0426 CANCER SCREENING IN IDIOPATHIC INFLAMMATORY MYOPATHIES: TEN YEARS EXPERIENCE FROM A SINGLE CENTER
Authors: E. Trallero-Araguás, A. Gil-Vila, X. Martínez-Gómez, M. Alvarado-Cardenas, M. Simo-Perdigó, J. Ros, I. Pinal-Fernandez, A. Selva-O’callaghan
Journal: Annals of the Rheumatic Diseases (2021)

Background: There is a well-recognized association between cancer and myositis, so cancer screening at diagnosis is recommended. Objectives: We aim to report the results of our cancer screening strategy and to ascertain the reliability of using PET/CT to identify cancer-associated myositis (CAM) in a large cohort of patients with myositis from a single center over 10 years. Methods: This retrospective observational study included all patients diagnosed with any type of myositis except for inclusion body myositis. Cancer screening strategy was individualized according to clinical and serological data, including PET/CT as the main test to detect occult cancer (OC). Procedures derived from a positive PET/CT were registered. Qualitative data expressed as percentages, and quantitative data as the median with the interquartile range were analyzed. A ROC curve was used to estimate the reliability of PET/CT for CAM diagnosis. Results: Seventy-seven out of 131 patients underwent a PET/CT for OC screening. The performance of the PET/CT in patients with myositis at disease onset yielded an area under the curve ROC of 0.87 (0.73-0.97) for CAM diagnosis. Invasive procedures in 7 (9%) patients without a final diagnosis of cancer did not cause derived complications. Patients not evaluated for OC did not develop cancer after a median follow-up of 3.3 years (1.7-6.7). Conclusion: Cancer screening strategy should be individualized. PET/CT at myositis onset seems to be an efficient approach to rule out CAM. This practice does not seem to significantly increase harm to patients related to the additional tests needed to clarify inconclusive results. Disclosure of Interests: None declared
AB0023 DENDRITIC CELLS AS A PROMINENT MARKERS OF AUTOIMMUNE DISEASES
Authors: M. Korolev, Y. Kurochkina, N. Banshhikova, V. Omelchenko, A. Akimova, E. Letyagina, O. Poveschenko
Journal: Annals of the Rheumatic Diseases (2021)

Background: Dendritic cells (DCs) are known to contribute to the pathogenesis of different autoimmune diseases. It is clear nowadays the role of DCs in rheumatoid arthritis (RA) but not well investigated in Axial spondylitis (AxSpA). DCs are a heterogeneous population and can be divided into groups: myeloid (mDCs) and plasmacytoid (pDCs). DCs can induce both immune response and tolerance. Objectives: To investigate the subpopulations of peripheral blood myeloid and plasmacytoid DCs in patients with early stage of RA (duration of illness up to 12 months) and AS. Methods: The study include sixty five patients with early forms of diseases including 55 patients with RA and 10 patients with AxSpA. Diagnosis RA was established according ACR/EULAR criteria (2010). Diagnosis AxSpA was established according ASAS criteria. All patients received conventional synthetic DMARDs. Thirty patients with osteoarthritis (OA) used as a control group. Analysis of the content of the B-lymphocytes, myeloid and plasmacytoid DCs was carried out by flow cytometry. B-lymphocytes, subtypes of peripheral blood DCs were characterized by the following phenotypes: myeloid DCs (CD3-CD14-CD19-HLA-DR + CD11c + CD123-), plasmacytoid DCs (CD3-CD14-CD19-HLA-DR + CD11c-CD123 +), B-lymphocytes (CD19 +). Analyses were performed before treatment and after 3 and 6 months. Results: Patients with early RA are characterized by significant evaluation of the population of myeloid DCs in comparison of patients with osteoarthritis (25.3% vs 21.5, p=0.005). Furthermore, the difference was found in the number of cells with the phenotype B-lymphocytes: 5.7% vs 3.1%, p = 0.0007). No significant differences were observed in the number of plasmocytoid DCs. After 3 and 6 month of observation we detected reducing amount of myeloid DCs 26.7% vs 20.1% vs 16.4% respectively. Disease activity according to DAS28 droped to low (4.32 to3.06, p=0.03). Patients with AxSpA are characterized a lower mDCs levels in compared with RA (19.3% vs 26.7, p=0.07). After 6 month of investigation we detected decreasing mDCs (19.3% to 14.2%, p=0.05). The percent of pDCs were constant and did not differ from the level of healthy donors. Conclusion: The data obtained indicate that early form of rheumatic diseases namely rheumatoid arthritis and axial spondylitis have the common features such as the dominance of mDCs and their decreasing in reduction of activity of disease. Disclosure of Interests: None declared
POS1467-HPR SEVERE RESPIRATORY INFECTIONS IN RHEUMATOID ARTHRITIS PATIENTS WITH BIOLOGIC THERAPY. COMPARATIVE STUDY BETWEEN VACCINATED AND NON VACCINATED PATIENTS
Authors: L. Domínguez, P. Rodriguez Cundin, T. Dierssen-Sotos, V. Calvo-Río, N. Vegas-Revenga, A. Corrales, N. Palmou-Fontana, D. Prieto-Peña, M. Calderón-Goercke, M. A. González-Gay, R. Blanco
Journal: Annals of the Rheumatic Diseases (2021)

Background: Rheumatoid arthritis (RA) patients are at increased risk of severe infections due to the disease itself, and the immunosuppressive treatment. Vaccination programs are designed to decrease the risk of infections. Objectives: In patients with RA treated with biologic therapy (BT) our aim was to assess a) the incidence of severe respiratory infections and b) to compare the risk between vaccinated and non vaccinated patients. Methods: Observational study of 431 patients diagnosed with RA that iniciated BT. One group of patients participated in a vaccination program from October 2011 to October 2016 (Group 1). The other group was not included in the vaccination program (Group 2). The follow-up was made until June 2017 with a minimum follow-up period of 8 months and a maximum of 5.5 years. Information on severe respiratory infections, defined as those that required hospitalization or at least one dose of intravenous antibiotic treatment at the emergency room, was retrieved from the hospital medical records. Results: We studied 431 patients (335 women/96 men); mean age 63.4±13.7 years. In the vaccination program (group 1 ) were included 299 (69.37%) patients and in the group 2 132 patients (30.63%). The main features of both groups are summarized in Table 1. During the follow-up, we registered 299 hospital admissions due to severe respiratory infections in both groups (incidence density 9.9 (95% CI: 6.9-13.6). In group 1, vaccinated patients, this incidence density was reduced to 7.1 (95% CI: 4.1-11.6). Figure 1. The vaccination program reduced the general incidence of severe respiratory infection in 44%. Conclusion: RA patients with BT included in the vaccination program present a lower incidence of severe respiratory infections compared with non vaccinated patients. Table 1. Main general features at BT onset Group 1 Vaccination program N=299 Group 2 Non vaccination program N=132 p Age (years) mean±SD 61.32±13.04 67.97±14.17 0.32 Women, n (%) 231 (77.3%) 105 (79.5%) 0.59 Duration of RA (months) mean±SD 73.24±10.4 112.62±60.2 Positive RF/ Positive ACPA, n (%) 177(59.2)/172 (57.52%) 93(70.5%)98 (74.24%) 0.02/0,01 Erosive disease, n (%) 116 (38.8%) 70 (53%) 0.06 Vasculitis, n (%) 15 (5%) 2 (1.5%) 0.08 Interstitial lung disease n (%) 12 (4%) 7 (5.3%) 0.54 Subcutaneous nodules n (%) 16 (5.4%) 6 (4.5%) 0.72 Corticosteroids 299 (100%) 132 (100%) 1 Number of conventional DMARDs, mean±SD 1.66±0.89 2.03±1.05 0.3 Figure 1. Disclosure of Interests: None declared
A comprehensive review of the multifaceted role of the microbiota in human pancreatic carcinoma
Authors: Gouri Pandya, Anuradha Kirtonia, Aishwarya Singh, Arul Goel, Chakrabhavi Dhananjaya Mohan, Kanchugarakoppal S Rangappa, Amit Kumar Pandey, Sonia Kapoor, Simran Tandon, Gautam Sethi, Manoj Garg
Journal: Seminars in Cancer Biology (2021)