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51. POS0063 PROGRESSIVE INTERSTITIAL LUNG DISEASE IS FREQUENT ALSO IN LATE DISEASE STAGES IN SYSTEMIC SCLEROSIS PATIENTS FROM EUSTAR

Author

Hoffmann-Vold, A. M., primary, Brunborg, C., additional, Airò, P., additional, Ananyeva, L. P., additional, Czirják, L., additional, Guiducci, S., additional, Hachulla, E., additional, Li, M., additional, Mihai, C., additional, Riemekasten, G., additional, Sfikakis, P., additional, Valentini, G., additional, Kowal-Bielecka, O., additional, Allanore, Y., additional, and Distler, O., additional

Published
2022
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52. POS1203 INCREASED PROTEASE-ACTIVATED RECEPTOR 1 AUTOANTIBODIES ARE ASSOCIATED WITH SEVERE COVID-19

Author

Tran, F., primary, Scharmacher, A., additional, Grasshoff, H., additional, Schinke, S., additional, Kaeding, N., additional, Bernades, J., additional, Humrich, J. Y., additional, Cabral-Marques, O., additional, Gaede, K., additional, Lange, C., additional, Rupp, J., additional, Rosenstiel, P., additional, Hoyer, B. F., additional, Schulze-Forster, K., additional, Heidecke, H., additional, Halpert, G., additional, Amital, H., additional, Shoenfeld, Y., additional, Schreiber, S., additional, and Riemekasten, G., additional

Published
2022
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56. OP0158 COHORT ENRICHMENT STRATEGIES FOR PROGRESSIVE INTERSTITIAL LUNG DISEASE IN SYSTEMIC SCLEROSIS FROM EUSTAR

Author

Hoffmann-Vold, A. M., primary, Brunborg, C., additional, Airò, P., additional, Ananyeva, L. P., additional, Czirják, L., additional, Guiducci, S., additional, Hachulla, E., additional, Li, M., additional, Mihai, C., additional, Riemekasten, G., additional, Sfikakis, P., additional, Valentini, G., additional, Kowal-Bielecka, O., additional, Allanore, Y., additional, and Distler, O., additional

Published
2022
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68. The Predict Study: low risk for digital ulcer development in patients with systemic sclerosis with increasing disease duration and lack of topoisomerase-1 antibodies

Author

Hunzelmann, N., Riemekasten, G., Becker, M. O., Moinzadeh, P., Kreuter, A., Melchers, I., Mueller-Ladner, U., Meier, F., Worm, M., Lee, H., Herrgott, I., Pfeiffer, C., Fierlbeck, G., Henes, J., Juche, A., Zeidler, G., Mensing, H., Günther, C., Sárdy, M., Burkhardt, H., Koehm, M., Kuhr, K., Krieg, T., and Sunderkötter, C.

Published
2016
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70. Outcomes of limited cutaneous systemic sclerosis patients: Results on more than 12,000 patients from the EUSTAR database

Author

Frantz C., Huscher D., Avouac J., Hachulla E., Balbir-Gurman A., Riemekasten G., Siegert E., Lazzaroni M. -G., Carreira P. E., Vettori S., Zanatta E., Ullman S., Czirjak L., Kowal-Bielecka O., Distler O., Matucci-Cerinic M., Allanore Y, Cuomo Giovanna, University of Zurich, Allanore, Yannick, Frantz, C., Huscher, D., Avouac, J., Hachulla, E., Balbir-Gurman, A., Riemekasten, G., Siegert, E., Lazzaroni, M. -G., Carreira, P. E., Vettori, S., Zanatta, E., Ullman, S., Czirjak, L., Kowal-Bielecka, O., Distler, O., Matucci-Cerinic, M., Allanore, Y, and Cuomo, Giovanna

Subjects
0301 basic medicine, Skin score, Limited cutaneous systemic sclerosis, Male, Databases, Factual, Fibrosi, Limited cutaneous systemic sclerosi, Immunology, Digital ulcer, 610 Medicine & health, Interstitial lung disease, Disease, Outcomes, computer.software_genre, Scleroderma, 03 medical and health sciences, FEV1/FVC ratio, Databases, 0302 clinical medicine, Scleroderma, Limited, medicine, Immunology and Allergy, Humans, In patient, Limited, Lung, Factual, Skin, 030203 arthritis & rheumatology, Peripheral Vascular Diseases, 2403 Immunology, integumentary system, Database, business.industry, 10051 Rheumatology Clinic and Institute of Physical Medicine, Digital ulcers, Middle Aged, medicine.disease, Fibrosis, Female, Clinical trial, 030104 developmental biology, 2723 Immunology and Allergy, business, computer
Abstract

Objectives: Limited cutaneous systemic sclerosis (LcSSc) is the most common subset of SSc but it has been overlooked in the past years. At a time at which clinical trials focus on diffuse cutaneous SSc (DcSSc) we aimed at clarifying the outcomes of LcSSc and at evaluating whether potential drug positioned in DcSSc may also be used in LcSSc. Methods: The EUSTAR database was used to investigate skin, lung and peripheral vasculopathy outcomes in LcSSc. Worsening of skin fibrosis, ILD and peripheral vasculopathy were defined by an increase in modified Rodnan skin score (mRSS) > 3.5 points, a decrease of FVC > 10% in patients with ILD at baseline, and by the development of new digital ulcers (DU) in patients without DU at baseline. Results: 8013 LcSSc and 4786 DcSSc patients were included. In contrast to DcSSc, skin disease was remarkably stable in the majority of LcSSc patients with >80% having a change lower than ±4 units of mRSS at 12, 24 and 36 months follow-up. Conversely, FVC changes over time were very similar between LcSSc and DcSSc. Regarding DU, numbers of patients with new DU over time seemed to be almost similar between the two subsets. Conclusions: LcSSc patients have a low mRSS at baseline with marginal changes with time. Conversely, SSc-ILD can be as progressive as in DcSSc supporting the inclusion of LcSSc patients in SSc-ILD trials and suggesting potential benefit of any anti-ILD drugs. Similarly, although slightly less common, DU should receive the same attention in the two subsets.

Published
2020

73. IMMUNOSUPPRESSION WITH TARGETED DMARDS REDUCES MORBIDITY AND MORTALITY IN PRE-CAPILLARY PULMONARY HYPERTENSION ASSOCIATED WITH SYSTEMIC SCLEROSIS: A EUSTAR ANALYSIS.

Author

Bruni, C., Tofani, L., Fretheim, H., Weber, Y., Hachulla, E., Carreira, P., Giuggioli, D., Airò, P., Siegert, E., Müller-Ladner, U., Matucci-Cerinic, M., Riemekasten, G., Aznar, C. P. Simeon, De Vries-Bouwstra, J., Saketkoo, L. A., Distler, J., Balbir-Gurman, A., Castellví, I., Zanatta, E., and Smith, V.

Published
2023
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77. POS0834 LONG-TERM OUTCOME OF SSC ASSOCIATED ILD: IMPROVED SURVIVAL IN PPI TREATED PATIENTS

Author

Kreuter, M., primary, Bonella, F., additional, Kathrin, K., additional, Henes, J., additional, Siegert, E., additional, Riemekasten, G., additional, Blank, N., additional, Pfeiffer, C., additional, Müller-Ladner, U., additional, Kreuter, A., additional, Korsten, P., additional, Juche, A., additional, Schmalzing, M., additional, Worm, M., additional, Jandova, I., additional, Susok, L., additional, Schmeiser, T., additional, Guenther, C., additional, Keyszer, G., additional, Ehrchen, J., additional, Ramming, A., additional, Kötter, I., additional, Lorenz, H. M., additional, Moinzadeh, P., additional, and Hunzelmann, N., additional

Published
2021
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80. POS0318 CLINICAL PHENOTYPE IN SCLERODERMA PATIENTS WITH ANTI-TOPOISOMERASE I POSITIVITY AND LIMITED CUTANEOUS FORM: DATA FROM THE EUSTAR DATABASE

Author

Zanatta, E., primary, Huscher, D., additional, Airò, P., additional, Balbir-Gurman, A., additional, Siegert, E., additional, Ortolan, A., additional, Matucci-Cerinic, M., additional, Cozzi, F., additional, Riemekasten, G., additional, Hoffmann-Vold, A. M., additional, Distler, O., additional, Gabrielli, A., additional, Heitmann, S., additional, Hunzelmann, N., additional, Montecucco, C., additional, Morovic-Vergles, J., additional, Ribi, C., additional, Doria, A., additional, and Allanore, Y., additional

Published
2021
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81. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database

Author

Hoffmann-Vold, A.-M. Allanore, Y. Alves, M. Brunborg, C. Airó, P. Ananieva, L.P. Czirják, L. Guiducci, S. Hachulla, E. Li, M. Mihai, C. Riemekasten, G. Sfikakis, P.P. Kowal-Bielecka, O. Riccardi, A. Distler, O.

Subjects
respiratory system, behavioral disciplines and activities, respiratory tract diseases
Abstract

Objectives To identify overall disease course, progression patterns and risk factors predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD), using data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up. Methods Eligible patients with SSc-ILD were registered in the EUSTAR database and had measurements of forced vital capacity (FVC) at baseline and after 12±3 months. Long-term progressive ILD and progression patterns were assessed in patients with multiple FVC measurements. Potential predictors of ILD progression were analysed using multivariable mixed-effect models. Results 826 patients with SSc-ILD were included. Over 12±3 months, 219 (27%) showed progressive ILD: either moderate (FVC decline 5% to 10%) or significant (FVC decline >10%). A total of 535 (65%) patients had multiple FVC measurements available over mean 5-year follow-up. In each 12-month period, 23% to 27% of SSc-ILD patients showed progressive ILD, but only a minority of patients showed progression in consecutive periods. Most patients with progressive ILD (58%) had a pattern of slow lung function decline, with more periods of stability/improvement than decline, whereas only 8% showed rapid, continuously declining FVC; 178 (33%) experienced no episode of FVC decline. The strongest predictive factors for FVC decline over 5 years were male sex, higher modified Rodnan skin score and reflux/dysphagia symptoms. Conclusion SSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important. Novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage. © Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY. Published by BMJ.

Published
2021

82. Estimated glomerular filtration rate is a marker of mortality in the European Scleroderma Trials and Research Group (EUSTAR) database

Author

Gigante A, Hoffmann-Vold AM, Fegatelli DA, Gabrielli A, Leodori G, Coleiro B, De Santis M, Dagna L, Alegre-Sancho JJ, Montecucco C, Carreira PE, Balbir-Gurman A, Doria A, Riemekasten G, Airò P, Distler J, Distler O, Rosato E, and EUSTAR collaborators

Subjects
EUSTAR, glomerular filtration rate, integumentary system, systemic sclerosis, scleroderma renal crisis
Abstract

Objectives The study aim was to evaluate the estimated glomerular filtration rate (eGFR), its association with clinical disease and its predictive ability with respect to mortality in SSc patients from the European Scleroderma Trials and Research Group (EUSTAR) database. Methods SSc patients from the EUSTAR database who had items required for the calculation of eGFR at a baseline visit and a second follow-up visit available were included. A cut-off eGFR value of 60 ml/min was chosen for all SSc patients, and 30 ml/min for those with scleroderma renal crisis (SRC). Cox regression and competing risk analysis were performed to evaluate the use of eGFR as a predictive factor of mortality. Results A total of 3650 SSc patients were included in this study. The median serum level of creatinine and the mean of eGFR were 0.8 mg/dl (interquartile range = 0.6-0.9) and 86.6 +/- 23.7 ml/min, respectively. The eGFR was significantly lower in patients with pulmonary hypertension. Overall survival (OS) was significantly reduced in SSc patients with eGFR < 60 ml/min compared with patients with eGFR >= 60 ml/min [OS at 5 years 0.763 (95% CI: 0.700, 0.814) vs 0.903 (95% CI: 0.883, 0.919; P < 0.001)]. In multivariable analysis, OS was associated with male gender (P < 0.01), systolic pulmonary arterial pressure (sPAP) (P < 0.001) and eGFR (P < 0.001). The cumulative incidence of deaths due to SSc was associated with increased sPAP (P < 0.001) and reduced eGFR (P < 0.05). The OS at 5 years of 53 SRC patients was not significantly different between SSc patients with eGFR > 30 ml/min and those with eGFR Conclusion eGFR represents a predictive risk factor for overall survival in SSc. The eGFR, however, does not represent a risk factor for death in SRC.

Published
2021

83. A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc

Author

Saketkoo, L.A., Frech, T., Varjú, C., Domsic, R., Farrell, Jessica, Gordon, J.K., Mihai, C., Sandorfi, N., Shapiro, L., Poole, J., Volkmann, E.R., Lammi, M., McAnally, K., Alexanderson, H., Pettersson, H., Hant, F., Kuwana, M., Shah, A.A., Smith, V., Hsu, V., Kowal-Bielecka, O., Assassi, S., Cutolo, M., Kayser, C., Shanmugam, V.K., Vonk, M.C., Fligelstone, K., Baldwin, N., Connolly, K., Ronnow, A., Toth, B., Suave, M., Farrington, S., Bernstein, E.J., Crofford, L.J., Czirják, L., Jensen, K., Hinchclif, M., Hudson, M., Lammi, M.R., Mansour, J., Morgan, N.D., Mendoza, F., Nikpour, M., Pauling, J., Riemekasten, G., Russell, A.M., Scholand, M.B., Seigart, E., Rodriguez-Reyna, T.S., Hummers, L., Walker, U., Steen, V., Saketkoo, L.A., Frech, T., Varjú, C., Domsic, R., Farrell, Jessica, Gordon, J.K., Mihai, C., Sandorfi, N., Shapiro, L., Poole, J., Volkmann, E.R., Lammi, M., McAnally, K., Alexanderson, H., Pettersson, H., Hant, F., Kuwana, M., Shah, A.A., Smith, V., Hsu, V., Kowal-Bielecka, O., Assassi, S., Cutolo, M., Kayser, C., Shanmugam, V.K., Vonk, M.C., Fligelstone, K., Baldwin, N., Connolly, K., Ronnow, A., Toth, B., Suave, M., Farrington, S., Bernstein, E.J., Crofford, L.J., Czirják, L., Jensen, K., Hinchclif, M., Hudson, M., Lammi, M.R., Mansour, J., Morgan, N.D., Mendoza, F., Nikpour, M., Pauling, J., Riemekasten, G., Russell, A.M., Scholand, M.B., Seigart, E., Rodriguez-Reyna, T.S., Hummers, L., Walker, U., and Steen, V.

Abstract

Item does not contain fulltext, Systemic sclerosis (SSc), the most lethal of rheumatologic conditions, is the cause of death in >50% of SSc cases, led by pulmonary fibrosis followed by pulmonary hypertension and then scleroderma renal crisis (SRC). Multiple other preventable and treatable SSc-related vascular, cardiac, gastrointestinal, nutritional and musculoskeletal complications can lead to disability and death. Vascular injury with subsequent inflammation transforming to irreversible fibrosis and permanent damage characterizes SSc. Organ involvement is often present early in the disease course of SSc, but requires careful history-taking and vigilance in screening to detect. Inflammation is potentially reversible provided that treatment intensity quells inflammation and other immune mechanisms. In any SSc phenotype, opportunities for early treatment are prone to be under-utilized, especially in slowly progressive phenotypes that, in contrast to severe progressive ILD, indolently accrue irreversible organ damage resulting in later-stage life-limiting complications such as pulmonary hypertension, cardiac involvement, and malnutrition. A single SSc patient visit often requires much more physician and staff time, organization, vigilance, and direct management for multiple organ systems compared to other rheumatic or pulmonary diseases. Efficiency and efficacy of comprehensive SSc care enlists trending of symptoms and bio-data. Financial sustainability of SSc care benefits from understanding insurance reimbursement and health system allocation policies for complex patients. Sharing care between recognised SSc centers and local cardiology/pulmonary/rheumatology/gastroenterology colleagues may prevent complications and poor outcomes, while providing support to local specialists. As scleroderma specialists, we offer a practical framework with tools to facilitate an optimal, comprehensive and sustainable approach to SSc care. Improved health outcomes in SSc relies upon recogntion, management and, to the

Published
2021

84. CLINICAL PHENOTYPE IN SCLERODERMA PATIENTS WITH ANTI-TOPOISOMERASE I POSITIVITY AND LIMITED CUTANEOUS FORM: DATA FROM THE EUSTAR DATABASE

Author

Zanatta, E., Huscher, D., Airo, P., Balbir-Gurman, A., Siegert, E., Ortolan, A., Matucci-Cerinic, M., Cozzi, F., Riemekasten, G., Hoffmann-Vold, A. M., Distler, O., Gabrielli, A., Heitmann, S., Hunzelmann, N., Montecucco, C., Morovic-Vergles, J., Ribi, C., Doria, A., Allanore, Y., Zanatta, E., Huscher, D., Airo, P., Balbir-Gurman, A., Siegert, E., Ortolan, A., Matucci-Cerinic, M., Cozzi, F., Riemekasten, G., Hoffmann-Vold, A. M., Distler, O., Gabrielli, A., Heitmann, S., Hunzelmann, N., Montecucco, C., Morovic-Vergles, J., Ribi, C., Doria, A., and Allanore, Y.

Published
2021

85. LONG-TERM OUTCOME OF SSC ASSOCIATED ILD: IMPROVED SURVIVAL IN PPI TREATED PATIENTS

Author

Kreuter, M., Bonella, F., Kathrin, K., Henes, J., Siegert, E., Riemekasten, G., Blank, N., Pfeiffer, C., Mueller-Ladner, U., Kreuter, A., Korsten, P., Juche, A., Schmalzing, M., Worm, M., Jandova, I., Susok, L., Schmeiser, T., Guenther, C., Keyszer, G., Ehrchen, J., Ramming, A., Koetter, I., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Kathrin, K., Henes, J., Siegert, E., Riemekasten, G., Blank, N., Pfeiffer, C., Mueller-Ladner, U., Kreuter, A., Korsten, P., Juche, A., Schmalzing, M., Worm, M., Jandova, I., Susok, L., Schmeiser, T., Guenther, C., Keyszer, G., Ehrchen, J., Ramming, A., Koetter, I., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.

Published
2021

86. A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc

Author

Saketkoo, LA, Frech, T, Varju, C, Domsic, R, Farrell, J, Gordon, JK, Mihai, C, Sandorfi, N, Shapiro, L, Poole, J, Volkmann, ER, Lammi, M, McAnally, K, Alexanderson, H, Pettersson, H, Hant, F, Kuwana, M, Shah, AA, Smith, V, Hsu, V, Kowal-Bielecka, O, Assassi, S, Cutolo, M, Kayser, C, Shanmugam, VK, Vonk, MC, Fligelstone, K, Baldwin, N, Connolly, K, Ronnow, A, Toth, B, Suave, M, Farrington, S, Bernstein, EJ, Crofford, LJ, Czirjak, L, Jensen, K, Hinchclif, M, Hudson, M, Lammi, MR, Mansour, J, Morgan, ND, Mendoza, F, Nikpour, M, Pauling, J, Riemekasten, G, Russell, A-M, Scholand, MB, Seigart, E, Rodriguez-Reyna, TS, Hummers, L, Walker, U, Steen, V, Saketkoo, LA, Frech, T, Varju, C, Domsic, R, Farrell, J, Gordon, JK, Mihai, C, Sandorfi, N, Shapiro, L, Poole, J, Volkmann, ER, Lammi, M, McAnally, K, Alexanderson, H, Pettersson, H, Hant, F, Kuwana, M, Shah, AA, Smith, V, Hsu, V, Kowal-Bielecka, O, Assassi, S, Cutolo, M, Kayser, C, Shanmugam, VK, Vonk, MC, Fligelstone, K, Baldwin, N, Connolly, K, Ronnow, A, Toth, B, Suave, M, Farrington, S, Bernstein, EJ, Crofford, LJ, Czirjak, L, Jensen, K, Hinchclif, M, Hudson, M, Lammi, MR, Mansour, J, Morgan, ND, Mendoza, F, Nikpour, M, Pauling, J, Riemekasten, G, Russell, A-M, Scholand, MB, Seigart, E, Rodriguez-Reyna, TS, Hummers, L, Walker, U, and Steen, V

Abstract

Systemic sclerosis (SSc), the most lethal of rheumatologic conditions, is the cause of death in >50% of SSc cases, led by pulmonary fibrosis followed by pulmonary hypertension and then scleroderma renal crisis (SRC). Multiple other preventable and treatable SSc-related vascular, cardiac, gastrointestinal, nutritional and musculoskeletal complications can lead to disability and death. Vascular injury with subsequent inflammation transforming to irreversible fibrosis and permanent damage characterizes SSc. Organ involvement is often present early in the disease course of SSc, but requires careful history-taking and vigilance in screening to detect. Inflammation is potentially reversible provided that treatment intensity quells inflammation and other immune mechanisms. In any SSc phenotype, opportunities for early treatment are prone to be under-utilized, especially in slowly progressive phenotypes that, in contrast to severe progressive ILD, indolently accrue irreversible organ damage resulting in later-stage life-limiting complications such as pulmonary hypertension, cardiac involvement, and malnutrition. A single SSc patient visit often requires much more physician and staff time, organization, vigilance, and direct management for multiple organ systems compared to other rheumatic or pulmonary diseases. Efficiency and efficacy of comprehensive SSc care enlists trending of symptoms and bio-data. Financial sustainability of SSc care benefits from understanding insurance reimbursement and health system allocation policies for complex patients. Sharing care between recognised SSc centers and local cardiology/pulmonary/rheumatology/gastroenterology colleagues may prevent complications and poor outcomes, while providing support to local specialists. As scleroderma specialists, we offer a practical framework with tools to facilitate an optimal, comprehensive and sustainable approach to SSc care. Improved health outcomes in SSc relies upon recogntion, management and, to the

Published
2021

90. The autoimmune disease-associated IL2RA locus is involved in the clinical manifestations of systemic sclerosis

Author

Martin, J-E, Carmona, F D, Broen, J C A, Simeón, C P, Vonk, M C, Carreira, P, Ríos-Fernández, R, Espinosa, G, Vicente-Rabaneda, E, Tolosa, C, García-Hernández, F J, Castellví, I, Fonollosa, V, González-Gay, M A, Sáez-Comet, L, Portales, R García, de la Peña, P García, Fernández-Castro, M, Díaz, B, Martínez-Estupiñán, L, Coenen, M, Voskuyl, A E, Schuerwegh, A J, Vanthuyne, M, Houssiau, F, Smith, V, de Keyser, F, De Langhe, E, Riemekasten, G, Witte, T, Hunzelmann, N, Kreuter, A, Palm, Ø, Chee, M M, van Laar, J M, Denton, C, Herrick, A, Worthington, J, Koeleman, B P C, Radstake, T R D J, Fonseca, C, and Martín, J

Published
2012
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98. 30-jährige Patientin mit plötzlich aufgetretenen hämorrhagischen Nekrosen beider Beine

Author

Graßhoff, H, Janusch, H, Sailer, VW, Arnold, S, Humrich, J, Riemekasten, G, and Lamprecht, P

Subjects
ddc: 610, 610 Medical sciences, Medicine
Abstract

Vorgeschichte: Aufnahme einer 30-jährigen Patientin mit großflächigen, konfluierenden Ekchymosen beider Beine, die sich im Verlauf weniger Stunden ausgebildet hatten. Vier Jahre zuvor war ein systemischer Lupus erythematodes (SLE) mit Schmetterlingserythem, Coombs-Test positiver hämolytischer[zum vollständigen Text gelangen Sie über die oben angegebene URL], Deutscher Rheumatologiekongress 2020, 48. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 34. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh)

Published
2020
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99. Racial differences in systemic sclerosis disease presentation: A European Scleroderma Trials and Research group study

Author

Jaeger, Veronika K, Tikly, Mohammed, Dong, Xu, Siegert, Elise, Hachulla, Eric, Airò, Paolo, Valentini, Gabriele, Matucci Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia, Allanore, Yannick, Müller-Ladner, Ulf, Ananieva, Lidia P, Balbir-Gurman, Alexandra, Distler, Jörg H W, Czirják, Laszlo, Mengtao, Li, Henes, Jörg, Jimenez, Sergio A, Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P, Delgaldo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich, A, Randone, Sb, Bannert, B, Iannone, F, Maurer, B, Jordan, S, Dobrota, R, Becker, M, Mihai, C, Becvarare, R, Tomčík, M, Bielecka, Ok, Gindzienska-Sieskiewicz, E, Karaszewska, K, Cutolo, M, Pizzorni, C, Paolino, S, Sulli, A, Ruaro, B, Alessandri, E, Riccardi, A, Giacco, V, Messitini, V, Irace, R, Kedor, C, Casteleyn, V, Hilger, J, Hoeppner, J, Rednic, S, Szabo, I, Petcu, A, Avouac, J, Camelia, F, Desbas, C, Vlachoyiannopoulos, P, Montecucco, C, Caporali, R, Cavagna, L, Stork, J, Inanc, M, Joven, Be, Novak, S, Anic, F, Varju, C, Minier, T, Chizzolini, C, Allai, D, Kucharz, Ej, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Dolnicar, As, Coleiro, B, Gabrielli, A, Manfredi, L, Benfaremo, D, Ferrarini, A, Bancel, Df, Hij, A, Lansiaux, P, Lazzaroni, Mg, Hesselstrand, R, Wuttge, D, Andréasson, R, Martinovic, D, Bozic, I, Radic, M, Braun-Moscovici, Y, Monaco, Al, Furini, F, Hunzelmann, N, Moinzadeh, P, Pellerito, R, Caimmi, C, Bertoldo, E, Morovic-Vergles, J, Culo, Im, Pecher, Ac, Santamaria, Vo, Heitmann, S, Codagnone, M, Pflugfelder, J, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Hasler, P, Kretschmar, S, Kohm, M, Bajocchi, G, Salvador, Mj, Silva, Japd, Stamenkovic, B, Stankovic, A, Selmi, Cf, Santis, M, Ceribelli, A, Garzanova, L, Koneva, O, Starovoytova, M, Herrick, A, Puppo, F, Negrini, S, Murdaca, G, Engelhart, M, Szücs, G, Szamosi, S, de la Puente, C, Grande, Cs, Villanueva, Mjg, Midtvedt, Sø, Hoffmann-Vold, Am, Launay, D, Sobanski, V, Riccieri, V, Vasile, M, Ionescu, Rm, Opris, D, Sha, A, Woods, A, Gheorghiu, Am, Bojinca, M, Sunderkötter, C, Ehrchen, J, Ingegnoli, F, Mouthon, L, Dunogue, B, Chaigne, B, Legendre, P, Cantatore, Fp, Corrado, A, Ullman, S, Iversen, L, von Mühlen CA, Pozzi, Mr, Eyerich, K, Lauffer, F, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Madej, M, Vanthuyne, M, Frédéric, H, Alegre-Sancho, Jj, Aringer, M, Herrmann, K, Günther, C, Westhovens, R, Langhe, E, Lenaerts, J, Anic, B, Baresic, M, Mayer, M, Üprus, M, Otsa, K, Yavuz, S, Granel, B, Radominski, Sc, De, C, Müller, S, Azevedo, Vf, Mendoza, F, Busquets, J, Popa, S, Agachi, S, Zenone, T, Pileckyte, M, Stebbings, S, Mathieu, A, Vacca, A, Sampaio-Barros, Pd, Stamp, L, Solanki, K, Silva, C, Schollum, J, Barns-Graham, H, Veale, D, O'Rourke, M, Loyo, E, Tineo, C, Paulino, G, Mohamed, Waaa, Rosato, E, Gigante, A, Oksel, F, Yargucu, F, Tanaseanu, Cm, Popescu, M, Dumitrascu, A, Tiglea, I, Foti, R, Visalli, E, Benenati, A, Amato, G, Ancuta, C, Villiger, P, Adler, S, Fröhlich, J, Kayser, C, Eduardo, Al, Fathi, N, Alii, S, Ahmed, M, Hasaneen, S, Hakeem, Ee, de la PG, Lefebvre, P, Martin, Jjg, Sibilia, J, Chatelus, E, Gottenberg, Je, Chifflot, H, Litinsky, I, Galdo, Fd, Abignano, G, Eng, S, Seskute, G, Butrimiene, I, Rugiene, R, Karpec, D, Pascal, M, Kerzberg, E, Bianchi, W, Bianchi, Bv, Bianchi, Dv, Barcellos, Y, Castellví, I, Millan, M, Limonta, M, Rimar, D, Rosner, I, Slobodin, G, Couto, M, Spertini, F, Ribi, C, Buss, G, Marcoccia, A, Bondanini, F, Ciani, A, Kahl, S, Hsu, Vm, Martin, T, Poindron, V, Meghit, K, Moiseev, S, Novikov, P, Chung, L, Kolstad, K, Stark, M, Schmeiser, T, Thiele, A, Majewski, D, Zdrojewski, Z, Zaneta, S, Wierzba, K, Martínez-Barrio, J, López-Longo, Fj, Bernardino, V, Moraes-Fontes, Mf, Rodrigues, Ac, Riemekasten, G, Sommerlatte, S, Jendreck, S, Arnold, S, Levy, Y, Rezus, E, Cardoneanu, A, Burlui, Am, Pamuk, On, Puttini, Ps, Talotta, R, Bongiovanni, S, Poormoghim, H, Andalib, E, Almasi, S, Kötter, I, Krusche, M, Cuomo, G, Danzo, F, Masini, F, Gaches, F, Michaud, M, Cartos, F, Belloli, L, Casu, C, Sfikakis, P, Tektonidou, M, Furst, D, Feldman, Gr, Ramazan, Am, Nurmambet, E, Miroto, A, Suta, C, Andronache, I, Huizinga, Twj, de Vries-Bouwstra, J., Chizzolini, Carlo, Jaeger, Veronika K, Tikly, Mohammed, Xu, Dong, Siegert, Elise, Hachulla, Eric, Airò, Paolo, Valentini, Gabriele, Matucci Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia, Allanore, Yannick, Müller-Ladner, Ulf, Ananieva, Lidia P, Balbir-Gurman, Alexandra, Distler, Jörg H W, Czirják, Laszlo, Li, Mengtao, Henes, Jörg, Jimenez, Sergio A, Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P, Delgaldo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich A, University of Zurich, Cerinic, Marco Matucci, Walker Ulrich, A, Randone, Silvia Bellando, Bannert, Bettina, Iannone, Florenzoaa, Maurer, Brittaab, Jordan, Suzanaab, Dobrota, Rucsandraab, Becker, Mikeab, Mihai, Carinaa, Becvarare, Radima, Tomcik, Michala, Bielecka, Otylia Kowala, Gindzienska-Sieskiewicz, Ewaa, Karaszewska, Katarzynaa, Cutolo, Maurizioa, Pizzorni, Carmena, Paolino, Sabrinaae, Sulli, Albertoa, Ruaro, Barbara, Alessandri, Elisa, Riccardi, Antonella, Giacco, Veronica, Messitini, Valentina, Irace, Rosaria, Kedor, Claudia, Casteleyn, Vincent, Hilger, Julia, Hoeppner, Jakob, Rednic, Simona, Szabo, Iulia, Petcu, Ana, Avouac, Jérome, Camelia, Frantz, Desbas, Carole, Vlachoyiannopoulos, Panayioti, Montecucco, Carlo Maurizio, Caporali, Roberto, Cavagna, Lorenzo, Stork, Jiri, Inanc, Murat, Joven, Beatriz E., Novak, Srdan, Anic, Felina, Varju, Cecilia, Minier, Tunde, Allai, Daniela, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Dolnicar, Alenka Sipek, Coleiro, Bernard, Gabrielli, Armando, Manfredi, Lucia, Benfaremo, Devi, Ferrarini, Alessia, Bancel, Dominique Farge, Hij, Adrian, Lazzaroni, Maria Grazia, Hesselstrand, Roger, Wuttge, Dirk, Andréasson, Kristofer, Martinovic, Duska, Bozic, Ivona, Radic, Mislav, Braun-Moscovici, Yolanda, Monaco, Andrea Lo, Furini, Federica, Hunzelmann, Nicola, Moinzadeh, Pia, Pellerito, Raffaele, Caimmi, Cristian, Bertoldo, Eugenia, Morovic-Vergles, Jadranka, Culo, Ivana Melanie, Pecher, Ann-Christian, Santamaria, Vera Ortiz, Heitmann, Stefan, Codagnone, Medeleine, Pflugfelder, Johanne, Krasowska, Dorota, Michalska-Jakubus, Malgorzata, Seidel, Matthia, Hasler, Paul, Kretschmar, Samuel, Kohm, Michaela, Bajocchi, Gianluigi, Salvador, Maria João, Da Silva, JoséAntonio Pereira, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Ceribelli, Angela, Garzanova, Ludmila, Koneva, Olga, Starovoytova, Maya, Herrick, Ariane, Puppo, Francesco, Negrini, Simone, Murdaca, Giuseppe, Engelhart, Merete, Szücs, Gabriela, Szamosi, Szilvia, De La Puente, Carlo, Grande, Cristina Sobrino, Villanueva, Maria Jesus Garcia, Midtve, Øyvindbw, Hoffmann-Vold, Anna-Mariabw, Launay, Davidbx, Sobanski, Vincentbx, Riccieri, Valeriaby, Vasile, Massimilianoby, Stefantoni, Katia, Ionescu, Ruxandra Maria, Opris, Daniela, Sha, Ami, Woods, Adrianne, Gheorghiu, Ana Maria, Bojinca, Mihai, Sunderkötter, Cord, Ehrchen, Jan, Ingegnoli, Francesca, Mouthon, Luc, Dunogue, Bertrand, Chaigne, Benjamin, Legendre, Paul, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Von Mühlen, Carlos Alberto, Pozzi, Maria Rosa, Eyerich, Kilian, Lauffer, Felix, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Vanthuyne, Marie, Frédéric, Houssiau, Alegre-Sancho, Juan Jose, Aringer, Martin, Herrmann, Kristine, Günther, Claudia, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Üprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastião Cezar, De Souza Müller, Carolina, Feijóazevedo, Valderílio, Mendoza, Fabian, Busquets, Joanna, Popa, Sergei, Agachi, Svetlana, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Jordan, Sarah, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Stamp, Lisa, Solanki, Kamal, Silva, Cherumi, Schollum, Joanne, Barns-Graham, Helen, Veale, Dougla, O'Rourke, Marie, Loyo, Esthela, Tineo, Carmen, Paulino, Glenny, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Fröhlich, Johanne, Kayser, Cristiane, Eduardo, Andrade Lui, Fathi, Nihal, Alii, Safa, Ahmed, Marrow, Hasaneen, Samar, El Hakeem, Eman, De La Peña Lefebvre, Paloma García, Martin, Jorge Juan Gonzalez, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Del Galdo, Francesco, Abignano, Giuseppina, Eng, Sookho, Seskute, Goda, Butrimiene, Irena, Rugiene, Rita, Karpec, Diana, Pascal, Melanie, Kerzberg, Eduardo, Bianchi, Washington, Bianchi, Breno Valdetaro, Bianchi, Dante Valdetaro, Barcellos, Yeda, Castellví, Ivan, Millan, Milena, Limonta, Massimiliano, Rimar, Doron, Rosner, Itzhak, Slobodin, Gleb, Couto, Maura, Spertini, Françoi, Ribi, Camillo, Buss, Guillaume, Marcoccia, Antonella, Bondanini, Francesco, Ciani, Aldo, Kahl, Sarah, Hsu, Vivien M., Martin, Thierry, Poindron, Vincent, Meghit, Kilifa, Moiseev, Sergey, Novikov, Pavel, Chung, Lori, Kolstad, Kathleen, Stark, Marianna, Schmeiser, Tim, Thiele, Astrid, Majewski, Dominik, Zdrojewski, Zbigniew, Zaneta, Smolenska, Wierzba, Karol, Martínez-Barrio, Julia, López-Longo, Francisco Javier, Bernardino, Vera, Moraes-Fontes, Maria Francisca, Rodrigues, Ana Catarina, Riemekasten, Gabriela, Sommerlatte, Sabine, Jendreck, Sebastian, Arnold, Sabrina, Levy, Yair, Rezus, Elena, Cardoneanu, Anca, Burlui, Alexandra Maria, Pamuk, Omer Nuri, Puttini, Piercarlo Sarzi, Talotta, Rossella, Bongiovanni, Sara, Poormoghim, Hadi, Andalib, Elham, Almasi, Simin, Kötter, Ina, Krusche, Matrin, Cuomo, Giovanna, Danzo, Fiammetta, Masini, Francesco, Gaches, Franci, Michaud, Martin, Cartos, Florian, Belloli, Laura, Casu, Cinzia, Sfikakis, Petro, Tektonidou, Maria, Furst, Daniel, Feldman, Gary R., Ramazan, Ana-Maria, Nurmambet, Emel, Miroto, Amalia, Suta, Cristina, Andronache, Iulia, Huizinga, Tom W. J., De Vries-Bouwstra, Jeska, and Walker, Ulrich A.

Subjects
Male, Vital capacity, Organ manifestations, systemic sclerosis, Type I, race difference, Systemic scleroderma, Gastroenterology, Scleroderma, immunology, 0302 clinical medicine, Diffusing capacity, middle aged, pulmonary hypertension, Medicine, Pharmacology (medical), 030212 general & internal medicine, organ manifestations, races, skin and connective tissue diseases, Lung, race, pathophysiology, African Continental Ancestry Group, ddc:616, integumentary system, disease course, Hazard ratio, Races, 10051 Rheumatology Clinic and Institute of Physical Medicine, Pulmonary, Middle Aged, Blacks, cohort analysis, Autoantibodie, 3. Good health, Asians, female, priority journal, DNA Topoisomerases, Type I, Black, centromere, Cohort, Hypertension, organ manifestation, Systemic sclerosis, Female, systemic sclerosi, Human, Adult, Asian Continental Ancestry Group, medicine.medical_specialty, Hypertension, Pulmonary, European Continental Ancestry Group, Black People, 610 Medicine & health, complication, Caucasian, White People, Article, lung, 03 medical and health sciences, Black person, Rheumatology, Asian People, forced vital capacity, Internal medicine, geographic distribution, Humans, controlled study, human, DNA topoisomerase, Aged, Autoantibodies, 030203 arthritis & rheumatology, Scleroderma, Systemic, Asian, business.industry, Whites, Systemic, Odds ratio, medicine.disease, Pulmonary hypertension, major clinical study, mortality, clinical feature, business, DNA Topoisomerases, autoantibody
Abstract

Objectives Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations. Methods SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses. Results The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP. AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001]. Conclusion Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality.

Published
2020

100. A cross-disease meta-GWAS identifies four new susceptibility loci shared between systemic sclerosis and Crohn’s disease

Author

González-Serna, D., Ochoa, E., López-Isac, E., Julià, A., Degenhardt, F., Ortego-Centeno, N., Radstake, T.R.D.J., Franke, A., Marsal, S., Mayes, M.D., Martín, J., Márquez, A., Assassi, S., Zhou, X., Tan, F.K., Arnett, F.C., Reveille, J.D., Gorlova, O., Chen, W.V., Ying, J., Gregersen, P.K., Lee, A.T., Voskuyl, A.E., de Vries-Bouwstra, J., Magro-Checa, C., Broen, J., Koeleman, B.P.C., Simeón, C.P., Fonollosa, V., Guillén, A., Carreira, P., Castellví, I., González-Gay, M.A., Ríos, R., Callejas-Rubio, J.L., Vargas-Hitos, J.A., García-Portales, R., Camps, M.T., Fernández-Nebro, A., González-Escribano, M.F., García-Hernández, F.J., Castillo, M.J., Aguirre, M.Á., Gómez-Gracia, I., Rodríguez-Rodríguez, L., Fernández-Gutiérrez, B., de la Peña, P.G., Vicente, E., Andreu, J.L., Fernández de Castro, M., López-Longo, F.J., Martínez, L., Espinosa, G., Tolosa, C., Pros, A., Rodríguez-Carballeira, M., Narváez, F.J., Rubio-Rivas, M., Ortiz-Santamaría, V., Madroñero, A.B., Díaz, B., Trapiella, L., Sousa, A., Egurbide, M.V., Fanlo-Mateo, P., Sáez-Comet, L., Díaz-González, F., Hernández, V., Beltrán, E., Román-Ivorra, J.A., Grau, E., Alegre-Sancho, J.J., Blanco-García, F.J., Oreiro, N., Freire, M., Balsa, A., Ortiz, A.M., Hunzelmann, N., Riemekasten, G., Distler, J.H.W., Witte, T., Airó, P., Beretta, L., Santaniello, A., Bellocchi, C., Lunardi, C., Moroncini, G., Gabrielli, A., and Scleroderma, Genetic, Consortium

Abstract

Genome-wide association studies (GWASs) have identified a number of genetic risk loci associated with systemic sclerosis (SSc) and Crohn’s disease (CD), some of which confer susceptibility to both diseases. In order to identify new risk loci shared between these two immune-mediated disorders, we performed a cross-disease meta-analysis including GWAS data from 5, 734 SSc patients, 4, 588 CD patients and 14, 568 controls of European origin. We identified 4 new loci shared between SSc and CD, IL12RB2, IRF1/SLC22A5, STAT3 and an intergenic locus at 6p21.31. Pleiotropic variants within these loci showed opposite allelic effects in the two analysed diseases and all of them showed a significant effect on gene expression. In addition, an enrichment in the IL-12 family and type I interferon signaling pathways was observed among the set of SSc-CD common genetic risk loci. In conclusion, through the first cross-disease meta-analysis of SSc and CD, we identified genetic variants with pleiotropic effects on two clinically distinct immune-mediated disorders. The fact that all these pleiotropic SNPs have opposite allelic effects in SSc and CD reveals the complexity of the molecular mechanisms by which polymorphisms affect diseases.

Published
2020

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