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2. Replication of PTPRC as genetic biomarker of response to TNF inhibitors in patients with rheumatoid arthritis

Author

Ferreiro-Iglesias, A, Montes, A, Perez-Pampin, E, Cañete, J D, Raya, E, Magro-Checa, C, Vasilopoulos, Y, Sarafidou, T, Caliz, R, Ferrer, M A, Joven, B, Carreira, P, Balsa, A, Pascual-Salcedo, D, Blanco, F J, Moreno-Ramos, M J, Fernández-Nebro, A, Ordóñez, M C, Alegre-Sancho, J J, Narváez, J, Navarro-Sarabia, F, Moreira, V, Valor, L, García-Portales, R, Marquez, A, Martin, J, Gómez-Reino, J J, and Gonzalez, A

Published
2016
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3. Update of EULAR recommendations for the treatment of systemic sclerosis

Author

Kowal-Bielecka O., Fransen J., Avouac J., Becker M., Kulak A., Allanore Y., Distler O., Clements P., Cutolo M., Czirjak L., Damjanov N., Del Galdo F., Denton C. P., Distler J. H. W., Foeldvari I., Figelstone K., Frerix M., Furst D. E., Guiducci S., Hunzelmann N., Khanna D., Matucci-Cerinic M., Herrick A. L., Van Den Hoogen F., Van Laar J. M., Riemekasten G., Silver R., Smith V., Sulli A., Tarner I., Tyndall A., Welling J., Wigley F., Valentini G., Walker U. A., Zulian F., Muller-Ladner U., Daikeler T., Lanciano E., Becvar R., Tomcik M., Gindzienska-Sieskiewicz E., Cuomo G., Iudici M., Rednic S., Vlachoyiannopoulos P. G., Caporali R., Carreira P. E., Novak S., Minier T., Kucharz E. J., Gabrielli A., Moroncini G., Airo' P., Hesselstrand R., Martinovic D., Radic M., Marasovic-Krstulovic D., Braun-Moscovici Y., Balbir-Gurman A., Lo Monaco A., Caramaschi P., Morovic-Vergles J., Henes J., Ortiz Santamaria V., Heitmann S., Krasowska D., Seidel M. F., Hasler P., Pereira Da Silva J. A., Salvador M. J., Stamenkovic B., Stankovic A., Tikly M., Ananieva L. P., Beretta L., Szucs G., Szamosi S., de la Puente Bujidos C., Midtvedt O., Hoffmann-Vold A. -M., Launay D., Hachulla E., Riccieri V., Ionescu R., Opris D., Mihai C., Herrgott I., Beyer C., Ingegnoli F., von Muhlen C. A., Alegre-Sancho J. J., Beltran-Catalan E., Aringer M., Fantana J., Leuchten N., Tausche A. -K., De Langhe E., Vanthuyne M., Anic B., Baresic M., Mayer M., Uprus M., Otsa K., Yavuz S., Granel B., Azevedo V. F., Muller C., Jimenez S. A., Popa S., Agachi S., Zenone T., Stebbings S., Dockerty J., Vacca A., Schollum J., Veale D. J., Toloza S., Xu D., Olas J., Rosato E., Foti R., Adler S., Dan D., Wiesik-Szewczyk E., Olesinska M., Kayser C., Fathi N., de la Pena Lefebvre P. G., Imbert B., Kowal-Bielecka, O., Fransen, J., Avouac, J., Becker, M., Kulak, A., Allanore, Y., Distler, O., Clements, P., Cutolo, M., Czirjak, L., Damjanov, N., Del Galdo, F., Denton, C. P., Distler, J. H. W., Foeldvari, I., Figelstone, K., Frerix, M., Furst, D. E., Guiducci, S., Hunzelmann, N., Khanna, D., Matucci-Cerinic, M., Herrick, A. L., Van Den Hoogen, F., Van Laar, J. M., Riemekasten, G., Silver, R., Smith, V., Sulli, A., Tarner, I., Tyndall, A., Welling, J., Wigley, F., Valentini, G., Walker, U. A., Zulian, F., Muller-Ladner, U., Daikeler, T., Lanciano, E., Becvar, R., Tomcik, M., Gindzienska-Sieskiewicz, E., Cuomo, G., Iudici, M., Rednic, S., Vlachoyiannopoulos, P. G., Caporali, R., Carreira, P. E., Novak, S., Minier, T., Kucharz, E. J., Gabrielli, A., Moroncini, G., Airo', P., Hesselstrand, R., Martinovic, D., Radic, M., Marasovic-Krstulovic, D., Braun-Moscovici, Y., Balbir-Gurman, A., Lo Monaco, A., Caramaschi, P., Morovic-Vergles, J., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Seidel, M. F., Hasler, P., Pereira Da Silva, J. A., Salvador, M. J., Stamenkovic, B., Stankovic, A., Tikly, M., Ananieva, L. P., Beretta, L., Szucs, G., Szamosi, S., de la Puente Bujidos, C., Midtvedt, O., Hoffmann-Vold, A. -M., Launay, D., Hachulla, E., Riccieri, V., Ionescu, R., Opris, D., Mihai, C., Herrgott, I., Beyer, C., Ingegnoli, F., von Muhlen, C. A., Alegre-Sancho, J. J., Beltran-Catalan, E., Aringer, M., Fantana, J., Leuchten, N., Tausche, A. -K., De Langhe, E., Vanthuyne, M., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Granel, B., Azevedo, V. F., Muller, C., Jimenez, S. A., Popa, S., Agachi, S., Zenone, T., Stebbings, S., Dockerty, J., Vacca, A., Schollum, J., Veale, D. J., Toloza, S., Xu, D., Olas, J., Rosato, E., Foti, R., Adler, S., Dan, D., Wiesik-Szewczyk, E., Olesinska, M., Kayser, C., Fathi, N., de la Pena Lefebvre, P. G., Imbert, B., UCL - SSS/IREC/SLUC - Pôle St.-Luc, UCL - (MGD) Service de rhumatologie, Kowal Bielecka, Otylia, Fransen, Jaap, Avouac, Jerome, Becker, Mike, Kulak, Agnieszka, Allanore, Yannick, Distler, Oliver, Clements, Philip, Cutolo, Maurizio, Czirjak, Laszlo, Damjanov, Nemanja, del Galdo, Francesco, Denton, Christopher P., Distler, Jörg H. W., Foeldvari, Ivan, Figelstone, Kim, Frerix, Marc, Furst, Daniel E., Guiducci, Serena, Hunzelmann, Nicola, Khanna, Dinesh, Matucci Cerinic, Marco, Herrick, Ariane L., van den Hoogen, Frank, van Laar, Jacob M., Riemekasten, Gabriela, Silver, Richard, Smith, Vanessa, Sulli, Alberto, Tarner, Ingo, Tyndall, Alan, Welling, Joep, Wigley, Frederic, Valentini, Gabriele, Walker, Ulrich A., Zulian, Francesco, Müller Ladner, Ulf, Daikeler, Thoma, Lanciano, Elisabetta, Becvã¡r, Radim, Tomcik, Michal, Gindzienska Sieskiewicz, Ewa, Iudici, Michele, Rednic, Simona, Vlachoyiannopoulos, Panayiotis G., Caporali, Roberto, Carreira, Patricia E., Novak, Srdan, Minier, Tã¼nde, Kucharz, Eugene J., Gabrielli, Armando, Moroncini, Gianluca, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Radic, Mislav, Marasovic Krstulovic, Daniela, Braun Moscovici, Yolanda, Monaco, Andrea Lo, Morovic Vergles, Jadranka, Culo, Melanie I., Henes, Jã¶rg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Michalska Jakubus, Malgorzata, Seidel, Matthias F., Klinik III, Medizinische, Hasler, Paul, Da Silva, José A. Pereira, Salvador, Maria J., Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Ananieva, Lidia P., Beretta, Lorenzo, Szucs, Gabriella, Szamosi, Szilvia, de la Puente Bujidos, Carlo, Midtvedt, Øyvind, Hoffmann Vold, Anna Maria, Launay, David, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra, Opris, Daniela, Mihai, Carina, Herrgott, Ilka, Beyer, Christian, Ingegnoli, Francesca, von Mühlen, Carlos Alberto, Alegre Sancho, Juan José, Beltran Catalan, Emma, Aringer, Martin, Fantana, Julia, Leuchten, Nicolai, Tausche, Anne Kathrin, Langhe, Ellen De, Vanthuyne, Marie, Anic, Branimir, Bareå¡ic, Marko, Mayer, Miroslav, Ãœprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Jimenez, Sergio A., Popa, Serghei, Agachi, Svetlana, Zenone, Thierry, Stebbings, Simon, Dockerty, Joanne, Vacca, Alessandra, Schollum, Joanna, Veale, Douglas J., Toloza, Sergio, Xu, Dong, Olas, Jacek, Rosato, Edoardo, Foti, Rosario, Adler, Sabine, Dan, Diana, Wiesik Szewczyk, Ewa, Olesinska, Marzena, Kayser, Cristiane, Fathi, Nihal, de la Peña Lefebvre, Paloma García, Imbert, Bernard, and Cuomo, Giovanna

Subjects
Endothelin Receptor Antagonists, Lung Diseases, Kidney Disease, Delphi Technique, Gastrointestinal Diseases, systemic sclerosis, Scleroderma Renal Crisis, Placebo-controlled study, Angiotensin-Converting Enzyme Inhibitors, Lung Disease, Scleroderma, 0302 clinical medicine, Glucocorticoid, Phosphodiesterase 5 Inhibitor, Immunology and Allergy, skin and connective tissue diseases, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti. Interna medicina, integumentary system, treatment, genetics and molecular biology (all), Hematopoietic Stem Cell Transplantation, cyclophosphamide, methotrexate, Pulmonary, Orvostudományok, Serotonin Uptake Inhibitor, 3. Good health, Europe, Systematic review, Hypertension, Serotonin Uptake Inhibitors, Cyclophosphamide, Methotrexate, Systemic Sclerosis, Treatment, Fingers, Fluoxetine, Glucocorticoids, Humans, Hypertension, Pulmonary, Kidney Diseases, Phosphodiesterase 5 Inhibitors, Prostaglandins I, Pyrazoles, Pyrimidines, Raynaud Disease, Rheumatology, Scleroderma, Systemic, Ulcer, Immunology, Biochemistry, Genetics and Molecular Biology (all), 030211 gastroenterology & hepatology, Endothelin Receptor Antagonist, Selective Serotonin Reuptake Inhibitors, medicine.drug, Human, medicine.medical_specialty, Gastrointestinal Disease, Klinikai orvostudományok, Riociguat, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, medicine, Finger, biochemistry, Intensive care medicine, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences. Internal Medicine, Systemic Sclerosi, 030203 arthritis & rheumatology, business.industry, Systemic, Angiotensin-Converting Enzyme Inhibitor, medicine.disease, Transplantation, Clinical research, Pyrimidine, immunology and allergy, rheumatology, immunology, Pyrazole, Physical therapy, business, Rheumatism
Abstract

The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows. All centres from the EULAR Scleroderma Trials and Research group were invited to submit and select clinical questions concerning SSc treatment using a Delphi approach. Accordingly, 46 clinical questions addressing 26 different interventions were selected for systematic literature review. The new recommendations were based on the available evidence and developed in a consensus meeting with clinical experts and patients. The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma renal crisis and gastrointestinal involvement. Compared with the 2009 recommendations, the 2016 recommendations include phosphodiesterase type 5 (PDE-5) inhibitors for the treatment of SSc-related RP and DUs, riociguat, new aspects for endothelin receptor antagonists, prostacyclin analogues and PDE-5 inhibitors for SSc-related PAH. New recommendations regarding the use of fluoxetine for SSc-related RP and haematopoietic stem cell transplantation for selected patients with rapidly progressive SSc were also added. In addition, several comments regarding other treatments addressed in clinical questions and suggestions for the SSc research agenda were formulated. These updated data-derived and consensus-derived recommendations will help rheumatologists to manage patients with SSc in an evidence-based way. These recommendations also give directions for future clinical research in SSc.

Published
2017
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5. Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis. a 10-year longitudinal study from the EUSTAR database

Author

Wirz, E. G., Jaeger, V. K., Allanore, Y., Riemekasten, G., Hachulla, E., Distler, O., Airo, P., Carreira, P. E., Tikly, M., Vettori, S., Gurman, A. B., Damjanov, N., Muller-Ladner, U., Distler, J., Li, M., Hausermann, P., Walker, U. A., Ananieva, L., Heitmann, S., Rednic, S., Jimenez, S., Riccieri, V., Szmyrka-Kaczmarek, M., Farge, D., Lapadula, G., Matucci-Cerinic, M., Guiducci, S., Hunzelmann, N., Rosa Pozzi, M., Mihai, C., Veale, D., Hesselstrand, R., Mariok, E., Smith, V., Kucharz, E. J., Czirjak, L., Martinovic, D., Solanki, K., Mihaela Ancuta, C., Sibilia, J., Paola, C., Hassanien, M., Kahl, S., Woods, A., Vanthuyne, M., Ruxandra, I., Radominski, S. C., Lo Monaco, A., Corrado, A., Koehm, M., Maurizio, M., Radim, B., Loyo, E., Uprus, M., Pellerito, R., Zenone, T., Gabrielli, A., Kowal-Bielecka, O., Rozman, B., Scorza, R., Ann Saketkoo, L., Midtvedt, O., von Muhlen, C. A., Henes, J., Branimir, A., Hasler, P., Yavuz, S., Villiger, P., Krummel-Lorenz, B., Posa, M., Engelhart, M., Denton, C., Krasowska, D., de la Pena Lefebvre, P. G., Cozzi, F., Mouthon, L., Rosato, E., Carlo, S., Alegre Sancho, J. J., Mallia, C., Limonta, M., Seidel, M., Foti, R., Stamp, L., Ullman, S., Stebbings, S., Ortiz Santamaria, V., Del Galdo, F., De Langhe, E., Mathieu, A., Sunderkotter, C., Eyerich, K., Stamenkovic, B., Novak, S., Sampaio-Barros, P. D., Kayser, C., Litinsky, I., Couto, M., University of Zurich, Walker, U A, Wirz, Eg, Jaeger, Vk, Allanore, Y, Riemekasten, G, Hachulla, E, Distler, O, Airò, P, Carreira, Pe, Tikly, M, Vettori, Serena, Balbir Gurman, A, Damjanov, N, Müller Ladner, U, Distler, J, Li, M, Häusermann, P, Walker, Ua, and Eustar, Coauthors

Subjects
Male, Genetics and Molecular Biology (all), Pathology, Longitudinal study, Time Factors, Databases, Factual, Epidemiology, systemic sclerosis, 2745 Rheumatology, Kaplan-Meier Estimate, Severity of Illness Index, Biochemistry, Scleroderma, Risk Factors, Medizinische Fakultät, Immunology and Allergy, Longitudinal Studies, Prospective Studies, 610 Medicine & health, integumentary system, Incidence (epidemiology), Incidence, 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, Connective tissue disease, 3. Good health, Autoantibodies, Systemic Sclerosis, Cohort, 2723 Immunology and Allergy, Female, Adult, medicine.medical_specialty, Immunology, General Biochemistry, Genetics and Molecular Biology, Sex Factors, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Skin Ulcer, medicine, Humans, ddc:610, Proportional Hazards Models, 2403 Immunology, Scleroderma, Systemic, business.industry, medicine.disease, Clinical trial, Biochemistry, Genetics and Molecular Biology (all), Scleroderma, Diffuse, business
Abstract

Objectives To longitudinally map the onset and identify risk factors for skin sclerosis and digital ulcers (DUs) in patients with systemic sclerosis (SSc) from an early time point after the onset of Raynaud9s phenomenon (RP) in the European Scleroderma Trials and Research (EUSTAR) cohort. Methods 695 patients with SSc with a baseline visit within 1 year after RP onset were followed in the prospective multinational EUSTAR database. During the 10-year observation period, cumulative probabilities of cutaneous lesions were assessed with the Kaplan–Meier method. Cox proportional hazards regression analysis was used to evaluate risk factors. Results The median modified Rodnan skin score (mRSS) peaked 1 year after RP onset, and was 15 points. The 1-year probability to develop an mRSS ≥2 in at least one area of the arms and legs was 69% and 25%, respectively. Twenty-five per cent of patients developed diffuse cutaneous involvement in the first year after RP onset. This probability increased to 36% during the subsequent 2 years. Only 6% of patients developed diffuse cutaneous SSc thereafter. The probability to develop DUs increased to a maximum of 70% at the end of the 10-year observation. The main factors associated with diffuse cutaneous SSc were the presence of anti-RNA polymerase III autoantibodies, followed by antitopoisomerase autoantibodies and male sex. The main factor associated with incident DUs was the presence of antitopoisomerase autoantibodies. Conclusion Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening.

Published
2016

11. Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort

Author

Muriel Elhai, Nanthara Sritharan, Marouane Boubaya, Alexandra Balbir-Gurman, Elise Siegert, Eric Hachulla, Jeska de Vries-Bouwstra, Gabriela Riemekasten, Jörg H W Distler, Edoardo Rosato, Francesco Del Galdo, Fabian A Mendoza, Daniel E Furst, Carlos de la Puente, Anna-Maria Hoffmann-Vold, Armando Gabrielli, Oliver Distler, Coralie Bloch-Queyrat, Yannick Allanore, Marco Matucci Cerinic, Ulrich Walker, Florenzo Iannone, Suzana Jordan, Radim Becvar, Otylia Kowal Bielecka, Maurizio Cutolo, Giovanna Cuomo, Claudia Kedor, Simona Rednic, Jérome Avouac, P. Vlachoyiannopoulos, C. Montecucco, Jiri Stork, Murat Inanc, Patricia E. Carreira, Srdan Novak, László Czirják, Michele Iudici, Eugene J. Kucharz, Elisabetta Zanatta, Katja Perdan-Pirkmajer, Bernard Coleiro, Gianluca Moroncini, Dominique Farge Bancel, Paolo Airò, Roger Hesselstrand, Mislav Radic, Yolanda Braun-Moscovici, Andrea Lo Monaco, Nicolas Hunzelmann, Raffaele Pellerito, Alessandro Giollo, Jadranka Morovic-Vergles, Christopher Denton, Madelon Vonk, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Paul Hasler, Michaela Kohm, Ivan Foeldvari, Gianluigi Bajocchi, Maria João Salvador, Bojana Stamenkovic, Carlo Francesco Selmi, Mohammed Tikly, Lidia P. Ananieva, Ariane Herrick, Ulf Müller-Ladner, Raffaele De Palma, Merete Engelhart, Gabriela Szücs, Cristina Sobrino Grande, Øyvind Midtvedt, David Launay, Valeria Riccieri, Ruxandra Maria Ionescu, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Susanne Ullman, Carlos Alberto von Mühlen, Maria Rosa Pozzi, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Branimir Anic, Maria Üprus, Sule Yavuz, Brigitte Granel, Carolina de Souza Müller, Joanna Busquets, Svetlana Agachi, Simon Stebbings, D'Alessandro Mathieu, Percival D. Sampaio-Barros, Lisa Stamp, Kamal Solanki, Douglas Veale, Esthela Loyo, Mengtao Li, Walid Ahmed Abdel Atty Mohamed, Antonietta Gigante, Fahrettin Oksel, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Britta Maurer, Jacob van Laar, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Giuseppina Abignano, Goda Seskute, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianchi, Ivan Castellví, Massimiliano Limonta, Doron Rimar, Maura Couto, François Spertini, Antonella Marcoccia, Sarah Kahl, Ivien M. Hsu, Thierry Martin, Sergey Moiseev, Lorinda S. Chung, Tim Schmeiser, Dominik Majewski, Zbigniew Zdrojewski, Julia Martínez-Barrio, Vera Bernardino, Sabine Sommerlatte, Yair Levy, Elena Rezus, Omer Nuri Pamuk, Piercarlo Sarzi Puttini, Hadi Poormoghim, Ina Kötter, Francis Gaches, Laura Belloli, Petros Sfikakis, Juliana Markus, Gary R Feldman, Ana-Maria Ramazan, H.U. Scherer, Marie-Elise Truchetet, Alain Lescoat, Lorenzo Dagna, J.M. van Laar, Lidia Rudnicka, Susana Oliveira, Fabiola Atzeni, Masataka Kuwana, Arsene Mekinian, Mickaël Martin, Yoshiya Tanaka, Elhai, M., Sritharan, N., Boubaya, M., Balbir-Gurman, A., Siegert, E., Hachulla, E., de Vries-Bouwstra, J., Riemekasten, G., Distler, J. H. W., Rosato, E., Del Galdo, F., Mendoza, F. A., Furst, D. E., de la Puente, C., Hoffmann-Vold, A. -M., Gabrielli, A., Distler, O., Bloch-Queyrat, C., Allanore, Y., Matucci Cerinic, M., Walker, U., Iannone, F., Jordan, S., Becvar, R., Kowal Bielecka, O., Cutolo, M., Cuomo, G., Kedor, C., Rednic, S., Avouac, J., Vlachoyiannopoulos, P., Montecucco, C., Stork, J., Inanc, M., Carreira, P. E., Novak, S., Czirjak, L., Iudici, M., Kucharz, E. J., Zanatta, E., Perdan-Pirkmajer, K., Coleiro, B., Moroncini, G., Farge Bancel, D., Airo, P., Hesselstrand, R., Radic, M., Braun-Moscovici, Y., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Giollo, A., Morovic-Vergles, J., Denton, C., Vonk, M., Damjanov, N., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Hasler, P., Kohm, M., Foeldvari, I., Bajocchi, G., Salvador, M. J., Stamenkovic, B., Selmi, C. F., Tikly, M., Ananieva, L. P., Herrick, A., Muller-Ladner, U., De Palma, R., Engelhart, M., Szucs, G., Sobrino Grande, C., Midtvedt, O., Launay, D., Riccieri, V., Ionescu, R. M., Sha, A., Gheorghiu, A. M., Sunderkotter, C., Ingegnoli, F., Mouthon, L., Smith, V., Cantatore, F. P., Ullman, S., Alberto von Muhlen, C., Pozzi, M. R., Eyerich, K., Wiland, P., Vanthuyne, M., Alegre-Sancho, J. J., Herrmann, K., De Langhe, E., Anic, B., Uprus, M., Yavuz, S., Granel, B., de Souza Muller, C., Busquets, J., Agachi, S., Stebbings, S., Mathieu, D. A., Sampaio-Barros, P. D., Stamp, L., Solanki, K., Veale, D., Loyo, E., Li, M., Abdel Atty Mohamed, W. A., Gigante, A., Oksel, F., Tanaseanu, C. -M., Foti, R., Ancuta, C., Maurer, B., van Laar, J., Kayser, C., Fathi, N., Garcia de la Pena Lefebvre, P., Sibilia, J., Litinsky, I., Abignano, G., Seskute, G., Saketkoo, L. A., Kerzberg, E., Bianchi, W., Castellvi, I., Limonta, M., Rimar, D., Couto, M., Spertini, F., Marcoccia, A., Kahl, S., Hsu, I. M., Martin, T., Moiseev, S., Chung, L. S., Schmeiser, T., Majewski, D., Zdrojewski, Z., Martinez-Barrio, J., Bernardino, V., Sommerlatte, S., Levy, Y., Rezus, E., Nuri Pamuk, O., Sarzi Puttini, P., Poormoghim, H., Kotter, I., Gaches, F., Belloli, L., Sfikakis, P., Markus, J., Feldman, G. R., Ramazan, A. -M., Scherer, H. U., Truchetet, M. -E., Lescoat, A., Dagna, L., van Laar, J. M., Rudnicka, L., Oliveira, S., Atzeni, F., Kuwana, M., Mekinian, A., Martin, M., and Tanaka, Y.

Subjects
Rheumatology, Immunology, Immunology and Allergy
Abstract

Background: The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous subtypes versus stratification by autoantibody status in systemic sclerosis. Methods: For this cohort study, we assessed people with systemic sclerosis in the multicentre international European Scleroderma Trials and Research (EUSTAR) database. Individuals positive for systemic-sclerosis autoantibodies of two specificities were excluded, and remaining individuals were classified by cutaneous subtype, according to their systemic sclerosis-specific autoantibodies, or both. We assessed the performance of each model to predict overall survival, progression-free survival, disease progression, and different organ involvement. The three models were compared by use of the area under the curve (AUC) of the receiver operating characteristic and the net reclassification improvement (NRI). Missing data were imputed. Findings: We assessed the database on July 26, 2019. Of 16 939 patients assessed for eligibility, 10 711 patients were included: 1647 (15·4%) of 10 709 were male, 9062 (84·6%) were female, mean age was 54·4 (SD 13·8) years, and mean disease duration was 7·9 (SD 8·2) years. Information regarding cutaneous subtype was available for 10 176 participants and antibody data were available for 9643 participants. In the prognostic analysis, there was no difference in AUC for overall survival (0·82, 95% CI 0·81–0·84 for cutaneous only vs 0·84, 0·82–0·85 for antibody only vs 0·84, 0·83–0·86 for combined) or for progression-free survival (0·70, 0·69–0·71 vs 0·71, 0·70–0·72 vs 0·71, 0·70–0·72). However, at 4 years the NRI showed substantial improvement for the antibody-only model compared with the cutaneous-only model in prediction of overall survival (0·57, 0·46–0·71 for antibody only vs 0·29, 0·19–0·39 for cutaneous only) and disease progression (0·36, 0·29–0·46 vs 0·21, 0·14–0·28). The antibody-only model did better than the cutaneous-only model in predicting renal crisis (AUC 0·72, 0·70–0·74 for antibody only vs 0·66, 0·64–0·69 for cutaneous only) and lung fibrosis leading to restrictive lung function (AUC 0·76, 0·75–0·77 vs 0·71, 0·70–0·72). The combined model improved the prediction of digital ulcers and elevated systolic pulmonary artery pressure, but did poorly for cardiac involvement. Interpretation: The autoantibody-only model outperforms cutaneous-only subsetting for risk stratifying people with systemic sclerosis in the EUSTAR cohort. Physicians should be aware of these findings at the time of decision making for patient management. Funding: World Scleroderma Foundation.

Published
2022

12. Predictors of disease worsening defined by progression of organ damage in diffuse systemic sclerosis: A European Scleroderma Trials and Research (EUSTAR) analysis

Author

Becker, Mike, Graf, Nicole, Sauter, Rafael, Allanore, Yannick, Curram, John, Denton, Christopher P., Khanna, Dinesh, Matucci-Cerinic, Marco, Pena, Janethe de Oliveira, Pope, Janet E., Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, 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, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Ege Üniversitesi, University of Zurich, Distler, Oliver, Chizzolini, Carlo, Allai, Daniela, Becker, M., Graf, N., Sauter, R., Allanore, Y., Curram, J., Denton, C. P., Khanna, D., Matucci-Cerinic, M., de Oliveira Pena, J., Pope, J. E., Distler, O., Guiducci, S., Walker, U., Jaeger, V., Bannert, B., Lapadula, G., Becvarare, R., Cutolo, M., Valentini, G., Siegert, E., Rednic, S., Montecucco, C., Carreira, P. E., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Allai, D., Kucharz, E. J., Cozzi, F., Rozman, B., Mallia, C., Gabrielli, A., Bancel, D. F., Airo, P., Hesselstrand, R., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Hunzelmann, N., Pellerito, R., Caramaschi, P., Black, C., Damjanov, N., Henes, J., Santamaria, V. O., Heitmann, S., Seidel, M., Pereira Da Silva, J. A., Stamenkovic, B., Selmi, C. F., Tikly, M., Denisov, L. N., Muller-Ladner, U., Engelhart, M., Hachulla, E., Riccieri, V., Ionescu, R. M., Mihai, C., Sunderkotter, C., Kuhn, A., Schett, G., Distler, J., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Alegre-Sancho, J. J., Krummel-Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anne, E., Westhovens, R., De Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Radominski, S. C., de Souza Muller, C., Azevedo, V. F., Popa, S., Zenone, T., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Visalli, E., Benenati, A., Amato, G., Ancuta, C., Chirieac, R., Villiger, P., Adler, S., Dan, D., de la Pena Lefebvre, P. G., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Del Galdo, F., Venalis, A., Saketkoo, L. A., Lasky, J. A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Brucato, A. L., Lupi, E., Spertini, F., Ribi, C., Buss, G., Martin, T., Guffroy, A., Poindron, V., Chung, L., Schmeiser, T., Zebryk, P., Riso, N., Riemekasten, G., Rezus, E., and Sarzi Puttini, P.

Subjects
INVOLVEMENT, SELECTION, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, diffuse, predictive factors, systemic sclerosis, 2745 Rheumatology, epidemiologic methods, morbidity, Disease, Immunology, General Biochemistry, Genetics and Molecular Biology, Immunology and Allergy, Rheumatology, INTERSTITIAL LUNG-DISEASE, disease worsening, mortality, predictive factors, systemic sclerosis, predictive factor, disease worsening, DESIGN, middle aged, Medicine and Health Sciences, FIBROSIS, scleroderma, SKIN THICKNESS SCORE, mortality, skin and connective tissue diseases, Prospective cohort study, humans, lung diseases, ddc:616, education.field_of_study, heart diseases, integumentary system, clinical trials as topic, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, 10051 Rheumatology Clinic and Institute of Physical Medicine, Interstitial lung disease, follow-up studies, ddc, female, Cohort, 2723 Immunology and Allergy, europe, Life Sciences & Biomedicine, CLINICAL-TRIALS, survival rate, medicine.medical_specialty, Population, 610 Medicine & health, disease progression, male, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, Severity of illness, REGRESSION, medicine, severity of illness index, education, Survival rate, METAANALYSIS, 2403 Immunology, Science & Technology, Scleroderma, Systemic, business.industry, MORTALITY, systemic, medicine.disease, prospective studies, Clinical trial, prognosis, scleroderma, diffuse, scleroderma, systemic, Scleroderma, Diffuse, business
Abstract

PubMed: 31227488, Objectives Mortality and worsening of organ function are desirable endpoints for clinical trials in systemic sclerosis (SSc). The aim of this study was to identify factors that allow enrichment of patients with these endpoints, in a population of patients from the European Scleroderma Trials and Research group database. Methods Inclusion criteria were diagnosis of diffuse SSc and follow-up over 12±3 months. Disease worsening/organ progression was fulfilled if any of the following events occurred: new renal crisis; decrease of lung or heart function; new echocardiography-suspected pulmonary hypertension or death. In total, 42 clinical parameters were chosen as predictors for the analysis by using (1) imputation of missing data on the basis of multivariate imputation and (2) least absolute shrinkage and selection operator regression. Results Of 1451 patients meeting the inclusion criteria, 706 had complete data on outcome parameters and were included in the analysis. Of the 42 outcome predictors, eight remained in the final regression model. There was substantial evidence for a strong association between disease progression and age, active digital ulcer (DU), lung fibrosis, muscle weakness and elevated C-reactive protein (CRP) level. Active DU, CRP elevation, lung fibrosis and muscle weakness were also associated with a significantly shorter time to disease progression. A bootstrap validation step with 10 000 repetitions successfully validated the model. Conclusions The use of the predictive factors presented here could enable cohort enrichment with patients at risk for overall disease worsening in SSc clinical trials. © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY. Published by BMJ., Bayer Bayer, United Kingdom Université Paris Descartes Li Ka Shing Foundation, LKSF University of Michigan, U-M, 1Department of Rheumatology and the Centre of experimental Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Big Data institute, li Ka shing Centre for Health information and Discovery, nuffield Department of Medicine, University of Oxford, Oxford, UK 4Rheumatology a Department, Paris Descartes University, sorbonne Paris Cité, Cochin Hospital, Paris, France 5Data science and analytics, Bayer plc, Reading, UK 6UCl Division of Medicine, Royal Free Campus, london, UK 7Division of Rheumatology, Department of internal Medicine, University of Michigan scleroderma Program, University of Michigan, ann arbor, Michigan, Usa 8Department of experimental and Clinical Medicine, University of Florence, Florence, italy 9Bayer Us llC, Whippany, new Jersey, Usa 10Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Ontario, Canada Acknowledgements The R-code for the linear Mi-lassO was received from Qixuan Chen.21 Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., Contributors study conception and design, acquisition of data, analysis and interpretation of data and drafting and revising the article: OD and MB; analysis and interpretation of data: OD, MB, Rs and nG. all authors have critically reviewed and approved the final submitted version to be published. Funding This study was supported by a grant from Bayer aG. Bayer employees are coauthors of this paper and supported the study design and interpretation of the data, but otherwise Bayer had no influence on the study., Competing interests MOB declares no conflict of interest. OD has had consultancy relationships with actelion, Bayer, Biogen idec, Boehringer ingelheim, Chemomab, espeRare foundation, Genentech/Roche, GsK, inventiva, italfarmaco, lilly, medac, Medimmune, Mitsubishi Tanabe Pharma, Pharmacyclics, novartis, Pfizer, sanofi, sinoxa and UCB in the area of potential treatments of scleroderma and its complications. OD has received research funding from actelion, Bayer, Boehringer ingelheim, Mitsubishi Tanabe Pharma and Roche in the area of potential treatments of scleroderma and its complications. OD has a patent for mir-29 licensed for the treatment of systemic sclerosis. DK has consultancy relationships and/or has received grant/research support from Bayer, Bristol-Myers squibb, Boehringer ingelheim, Genentech/Roche, niH, Pfizer, sanofi-aventis Pharmaceuticals, actelion Pharmaceuticals Us, Chemomab, Corbus, Covis, Cytori, eicos, eMD serono, Gilead, GlaxosmithKline, and UCB Pharma. He is a shareholder of eicos. CPD has consultancy relationships with and/or has received speakers’ bureau fees from actelion Pharmaceuticals Us, Bayer aG, GlaxosmithKline, Csl Behring, Merck serono, Roche Pharmaceuticals, Genentech and Biogen iDeC inc., inventiva, sanofi-aventis Pharmaceuticals and Boehringer ingelheim. JeP has consultancy relationships with and/or has received grant/research support from actelion, Bayer aG, Bristol-Myers squibb, Merck, Pfizer inc. and Roche. MM-C has consultancy relationships and/ or has received grant/research support from Pfizer, Bristol-Myers squibb, actelion, UCB Pharma, Bayer, Chemomab, Genentech/Roche, inventiva and lilly. Ya has consultancy relationships with and/or has received grant/research support from actelion, Pharmaceuticals Us, Bayer aG, Bristol-Myers squibb, inventiva, Medac, Pfizer inc., Roche Pharmaceuticals, Genentech and Biogen iDeC inc., sanofi-aventis Pharmaceuticals and servier. JdOP and JC are employees of Bayer. nTG has nothing to disclose.

Published
2019

13. Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the european scleroderma trials and research (eustar) cohort

Author

Wu, Wanlong, Jordan, Suzana, Graf, Nicole, Pena, Janethe de Oliveira, Curram, John, Allanore, Yannick, Matucci-Cerinic, Marco, Pope, Janet E., Denton, Christopher P., Khanna, Dinesh, Distler, Oliver, Guiducci, Serena, Walker, Ulrich, Jaeger, Veronika, Bannert, Bettina, Lapadula, Giovanni, Becvarare, Radim, Cutolo, Maurizio, Valentini, Gabriele, Siegert, Elise, Rednic, Simona, Montecucco, C., Carreira, Patricia E., Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Allai, Daniela, Kucharz, Eugene J., Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Gabrielli, Armando, Bancel, Dominique Farge, Airo, Paolo, Hesselstrand, Roger, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Pellerito, Raffaele, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Seidel, Matthias, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Denisov, Lev N., Mueller-Ladner, Ulf, Engelhart, Merete, Hachulla, Eric, Riccieri, Valeria, Ionescu, Ruxandra Maria, Mihai, Carina, Sunderkoetter, Cord, Kuhn, Annegret, Schett, Georg, Distler, Joerg, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Anne, Erler, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Popa, Sergei, Zenone, Thierry, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, 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, Chirieac, Rodica, Villiger, Peter, Adler, Sabine, Dan, Diana, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Del Galdo, Francesco, Venalis, Algirdas, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Martin, Thierry, Guffroy, Aurelien, Poindron, Vincent, Chung, Lori, Schmeiser, Tim, Zebryk, Pawel, Riso, Nuno, Riemekasten, Gabriela, Rezus, Elena, Puttini, Piercarlo Sarzi, Wu, W., Jordan, S., Graf, N., de Oliveira Pena, J., Curram, J., Allanore, Y., Matucci-Cerinic, M., Pope, J. E., Denton, C. P., Khanna, D., Distler, O., Guiducci, S., Walker, U., Jaeger, V., Bannert, B., Lapadula, G., Becvarare, R., Cutolo, M., Valentini, G., Siegert, E., Rednic, S., Montecucco, C., Carreira, P. E., Novak, S., Czirjak, L., Varju, C., Chizzolini, C., Allai, D., Kucharz, E. J., Cozzi, F., Rozman, B., Mallia, C., Gabrielli, A., Bancel, D. F., Airo, P., Hesselstrand, R., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Hunzelmann, N., Pellerito, R., Caramaschi, P., Black, C., Damjanov, N., Henes, J., Santamaria, V. O., Heitmann, S., Seidel, M., Pereira Da Silva, J. A., Stamenkovic, B., Selmi, C. F., Tikly, M., Denisov, L. N., Muller-Ladner, U., Engelhart, M., Hachulla, E., Riccieri, V., Ionescu, R. M., Mihai, C., Sunderkotter, C., Kuhn, A., Schett, G., Distler, J., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Cantatore, F. P., Corrado, A., Ullman, S., Iversen, L., Pozzi, M. R., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Alegre-Sancho, J. J., Krummel-Lorenz, B., Saar, P., Aringer, M., Gunther, C., Anne, E., Westhovens, R., De Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Uprus, M., Otsa, K., Yavuz, S., Radominski, S. C., de Souza Muller, C., Azevedo, V. F., Popa, S., Zenone, T., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C. -M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Visalli, E., Benenati, A., Amato, G., Ancuta, C., Chirieac, R., Villiger, P., Adler, S., Dan, D., de la Pena Lefebvre, P. G., Rubio, S. R., Exposito, M. V., Sibilia, J., Chatelus, E., Gottenberg, J. E., Chifflot, H., Litinsky, I., Del Galdo, F., Venalis, A., Saketkoo, L. A., Lasky, J. A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Brucato, A. L., Lupi, E., Spertini, F., Ribi, C., Buss, G., Martin, T., Guffroy, A., Poindron, V., Chung, L., Schmeiser, T., Zebryk, P., Riso, N., Riemekasten, G., Rezus, E., Sarzi Puttini, P., Ege Üniversitesi, Chizzolini, Carlo, Allali, Danièle, University of Zurich, and Distler, Oliver

Subjects
INVOLVEMENT, Male, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, diffuse, Time Factors, Databases, Factual, Skin Diseases/etiology/mortality/physiopathology, PREDICTION, Fibrosi, 2745 Rheumatology, Diffuse/complications/mortality/pathology, Immunology, General Biochemistry, Genetics and Molecular Biology, Immunology and Allergy, Rheumatology, Kaplan-Meier Estimate, ddc:616.07, Severity of Illness Index, Scleroderma, Cohort Studies, PROGNOSTIC-FACTORS, Fibrosis, Medicine and Health Sciences, scleroderma, Lung, Skin, integumentary system, progressive skin fibrosis, Lung function decline, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, 10051 Rheumatology Clinic and Institute of Physical Medicine, DEATH, Middle Aged, ddc, Europe, VARIABILITY, factual, Cohort, Visceral organ progression, 2723 Immunology and Allergy, Disease Progression, Female, Survival Analysi, Life Sciences & Biomedicine, Cohort study, Human, Adult, Skin/pathology, medicine.medical_specialty, databases, All-cause death, risk analysis, diffuse cutaneous systemic sclerosis, 610 Medicine & health, IMPROVEMENT, Systemic Sclerosis, Skin Diseases, THICKNESS SCORE, VALIDATION, Databases, FEV1/FVC ratio, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, medicine, Humans, Factual, Survival analysis, 2403 Immunology, Science & Technology, Proportional hazards model, business.industry, Surrogate endpoint, MORTALITY, Skin Disease, fibrosis, Progressive skin fibrosi, Lung/physiopathology, biomarkers, Diffuse cutaneous systemic sclerosi, medicine.disease, Survival Analysis, all-cause death, lung function decline, visceral organ progression, adult, cohort studies, databases, factual, disease progression, female, humans, Scleroderma, Diffuse, Cohort Studie, business
Abstract

PubMed: 30852552, Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ?7, valid mRSS at 12±3 months after baseline and ?1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ?25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ?10% (53.6% vs 34.4%; p, Bayer Bayer, 1Department of Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Clinical Development Pulmonology, Bayer Us llC, Whippany, new Jersey, Usa 4Data science and analytics, Bayer plc, Reading, UK 5Rheumatology a Department, Paris Descartes University, inseRM U1016, sorbonne, Paris Cité, Cochin Hospital, Paris, France 6Division of Rheumatology, University of Florence, Florence, italy 7Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Western Ontario, Canada 8Department of Rheumatology, Royal Free Hospital, University College london, london, UK 9scleroderma Program, Department of internal Medicine, Division of Rheumatology, University of Michigan, ann arbor, Michigan, Usa Acknowledgements The authors thank nicole schneider for excellent administration and data entry into the eUsTaR cohort. Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., This study was supported by a grant from Bayer aG.

Published
2019

14. Longitudinal association between nailfold capillaroscopy and incident interstitial lung disease: A EUSTAR database analysis.

Author

Velauthapillai A, de Vries-Bouwstra JK, Henes J, Czirjak L, Dagna L, Riemekastan G, Allanore Y, Foti R, Alegre-Sancho JJ, Maurer B, Cuomo G, Spierings J, Martin T, Truchetet ME, van den Ende CH, and Vonk MC

Abstract

Objectives: To evaluate (1) the association between nailfold capillaroscopy pattern and 5-year risk for incident interstitial lung disease and (2) the association between transition in nailfold capillaroscopy pattern and risk of incident interstitial lung disease., Methods: Data of adult patients from the EUSTAR database fulfilling the ACR-EULAR criteria with a disease duration ⩽5 years, having a scleroderma pattern at nailfold capillaroscopy with high-resolution computed tomography confirmed absence of interstitial lung disease (i.e. baseline) was used. Interstitial lung disease-free survival was assessed for up to 5 years of follow-up with a Cox proportional hazards model stratified on nailfold capillaroscopy pattern at baseline. The association of annual transition in nailfold capillaroscopy pattern on the risk to develop interstitial lung disease was assessed with a mixed logistic regression analysis., Results: Out of 771 eligible patients, 283 (37%) had an early pattern, 377 (49%) had an active pattern, and 111 (14%) had a late pattern. The Cox proportional hazard model including the identified confounders did not show an association between severity of nailfold capillaroscopy pattern at baseline and increased risk for interstitial lung disease during 5-year follow-up (hazard ratio (95 confidence interval; p value): 1.09 (0.86-1.39; 0.47)). The mixed logistic regression analysis revealed an increased annual risk for incident interstitial lung disease with increasing severity of capillaroscopic pattern (odds ratio (95% confidence interval); p value 3.76 (1.99-7.11; <0.01))., Conclusion: Our study shows that worsening of nailfold capillaroscopy has a strong association with an increased annual risk to develop interstitial lung disease. Of note, a worse scleroderma pattern at nailfold capillaroscopy is not associated with the long-term risk to develop interstitial lung disease., Competing Interests: The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: A.V.: none declared. J.K.B.: received consulting fees from Abbvie, Janssen and Boehringer Ingelheim, and received research grants from Roche, Galapagos Janssen, NVLE and ReumaNederland; all payments were made to her institution. J.H.: speakers bureau and consulting fees from ABBVIE,Astra-Zeneca, Boehringer Ingelheim, BMS, GSK, Janssen Pharmaceutical, Novartis, Roche, Pfizer and UCB. L.C.: none for this manuscript. L.D.: none for this manuscript. G.R.: none declared. Y.A.: received consulting fees from Boehringer Ingelheim, Janssen Pharmaceutical Companies, Astra-Zeneca, Topadur, Abbvie, Galderma, Medsenic, Prometheus regarding the management of systemic sclerosis. R.F.: none declared. J.J.A-S.: none for this manuscript. B.M.: grants from Novartis, consulting fees from Novartis, Boehringer Ingelheim, Jannsen-Cilag, GSK; speaker fees from Boehringer Ingelheim, GSK, Novartis, Otsuka, MSD; congress support from Medtalk, Pfizer, Roche, Actelion, Mepha, and MSD; and patent mir-29 for the treatment of systemic sclerosis (US8247389, EP2331143). G.C.: none for this manuscript. J.S.: received research grants from Boehringer Ingelheim and ReumaNederland; all payments were made to her institution. T.M.: none for this manuscript. M.E.T.: received consultancy and speaker fees from Boehringer Ingelheim, Pfizer, Galapagos, MSD and Lilly. C.H.M.E.: none declared. M.C.V.: received research grants from Boehringer Ingelheim, Ferrer and Galapagos; received consulting fees from Boehringer Ingelheim and Janssen Pharmaceutical Companies of Johnson & Johnson; received speaker fees from Boehringer Ingelheim, GSK, Janssen Pharmaceutical Companies of Johnson & Johnson, MSD, and Novartis; and is treasurer of EUSTAR and steering committee member of the ERN ReCONNET., (© The Author(s) 2025.)

Published
2025
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15. Analysis of factors involved in the development of humoral response to vaccination against SARS-CoV-2 in patients with rheumatic pathology under biological treatment.

Author

Flores-Fernández E, Vázquez-Gomez I, Valls-Pascual E, Valera-Ribera C, Andújar-Brazal P, and Alegre-Sancho JJ

Subjects
Humans, Infant, SARS-CoV-2, Prospective Studies, Vaccination, Rituximab therapeutic use, Immunologic Factors, Agammaglobulinemia, COVID-19 prevention & control, Vaccines, Rheumatic Diseases drug therapy
Abstract

Background and Objective: In the context of the SARS-CoV-2 pandemic, the development of new vaccines and their efficacy in patients with immune-mediated rheumatic diseases has been a target to investigate. The objective of this study is to evaluate the vaccine response rate in patients with immune-mediated rheumatic diseases under treatment with immunomodulators, including rituximab (RTX), as well as the influence of possible factors involved in the vaccination response in these patients., Material and Methods: A single-centre, prospective cohort study was conducted in 130 patients with immune-mediated rheumatic disease on treatment with immunomodulators, including RTX, who received the full course of vaccination against SARS-CoV-2 with BioNTech/Pfizer, Moderna/Lonza, AstraZeneca, or Janssen between April and October 2021. Demographic factors such as age, sex, type of immune-mediated disease, immunomodulatory treatment and type of vaccine were analysed, as well as serological markers including anti-SARS-CoV-2 IgG antibody levels measured one and six months after vaccination, CD19+ lymphocyte levels and the presence or absence of hypogammaglobulinemia. A statistical analysis was performed to assess the influence of the different variables collected in the study on the antibody titres., Results: A sample of 130 patients was studied, 41 under treatment with RTX and 89 with other immunomodulators. A lower vaccination response rate was observed in patients with RTX (12/34, 36.7%) one month after the primary vaccination compared to 96.5% (82/85) of patients who did not receive this drug and did respond. In the analysis of secondary variables, hypogammaglobulinemia was significantly associated with lack of development of a vaccine response. The administration of the last RTX cycle in the 6 months prior to vaccination and low CD19+ levels (<20 mg/dL) also had a negative influence on the development of a vaccine response. In the group of patients who were not receiving RTX treatment, the vaccination response was like that observed in the general population. We did not observe statistically significant differences in the vaccine response based on immunomodulatory treatment other than RTX, concomitant corticosteroid treatment, type of immune-mediated pathology, age, or sex., Discussion and Conclusions: In patients with rheumatic diseases receiving immunomodulatory treatment, the response to vaccination against SARS-CoV-2 is comparable to the general population, except in the case of patients receiving RTX, who have a lower response rate (around 36.7%) which is associated with factors such as hypogammaglobulinemia, pre-vaccination CD19+ lymphocyte levels, and a period between vaccination and the last dose of RTX of less than 6 months. It is important to take these factors into consideration to optimize vaccination in these patients., (Copyright © 2023 The Author(s). Published by Elsevier España, S.L.U. All rights reserved.)

Published
2023
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16. Immunoglobulins in systemic sclerosis management. A large multicenter experience.

Author

Tandaipan J, Guillén-Del-Castillo A, Simeón-Aznar CP, Carreira PE, De la Puente C, Narváez J, Lluch J, Rubio-Rivas M, Alegre-Sancho JJ, Bonilla G, Moriano C, Casafont-Sole I, García-Vicuña R, Ortiz-Santamaría V, Riera E, Atienza-Mateo B, Blanco R, Galisteo C, Gonzalez-Martin JJ, Pego-Reigosa JM, Pros A, Heredia S, and Castellví I

Subjects
Female, Humans, Male, Immunoglobulins, Intravenous therapeutic use, Retrospective Studies, Skin, Multicenter Studies as Topic, Observational Studies as Topic, Scleroderma, Systemic complications, Scleroderma, Systemic drug therapy, Myositis drug therapy
Abstract

Objective: To analyze the effectiveness and safety of intravenous immunoglobulin (IVIG) given in routine care to patients with systemic sclerosis (SSc)., Methods: A retrospective multicenter observational study was conducted in SSc patients treated with IVIG. We collected data on epidemiological parameters and clinical outcomes. Firstly, we assessed changes in organ manifestations during IVIG treatment. Secondly, we analyzed the frequency of adverse effects. The following parameters were collected from baseline to the last follow-up: the patient's weight, modified Rodnan Skin Score (mRSS), modified manual muscle strength scale (MRC), laboratory test(creatine kinase(CK), hemoglobin and protein levels), The University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 (UCLA GIT 2.0) questionnaire, pulmonary function tests, and echocardiography., Results: Data were collected on 78 patients (82% females; 59% with diffuse SSc). Inflammatory idiopathic myopathy was the most frequent concomitant overlap disease (41%). The time since Raynaud's phenomenon and SSc onset were 8.8 ± 18 and 6.2 ± 6.7 years respectively. The most frequent IVIG indication was myositis (38/78), followed by gastrointestinal (27/78) and cutaneous (17/78) involvement. The median number of cycles given were 5. 54, 53 and 9 patients have been treated previously with glucocorticoids, synthetic disease-modifying antirheumatic drugs and biologic therapies respectively. After IVIG use we found significant improvements in muscular involvement (MRC ≥ 3/5 92% IVIG, p = 0.001 and CK levels from 1149 ± 2026 UI to 217 ± 224 UI, p = 0.02), mRSS (15 ± 12.4 to 13 ± 12.5, p = 0.015) and improvement in total score of UCLA GIT 2.0 (p = 0.05). None Anti-RNA polymerase III patients showed an adequate response in gastrointestinal involvement (0/7) in comparison with other antibodies (0 vs. 25, p = 0,039). Cardiorespiratory involvement remained stable. A total of 12 adverse events were reported with only one withdrawn due to serious adverse effect., Conclusions: this study suggest that IVIG may improve myositis, gastrointestinal and skin involvement in SSc patients treated in routine care and seems to have a good safety profile., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Published
2023
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17. Snyder-Robinson syndrome: differential diagnosis of osteogenesis imperfecta.

Author

Valera Ribera C, Martinez-Ferrer À, Flores Fernández E, Vázquez Gómez I, Orenes Vera A, Valls Pascual E, Ybáñez García D, and Alegre Sancho JJ

Subjects
Diagnosis, Differential, Humans, X-Linked Intellectual Disability diagnosis, X-Linked Intellectual Disability genetics, Osteogenesis Imperfecta diagnosis, Osteogenesis Imperfecta drug therapy, Osteogenesis Imperfecta genetics, Spermine Synthase genetics
Abstract

Snyder-Robinson syndrome is an extremely rare genetic disorder, caused by mutations of the spermine synthase gene. We report a novel case of Snyder-Robinson syndrome, caused by a de novo mutation and first misdiagnosed with osteogenesis imperfecta. Clinical features, course, and genetic analysis are presented. The patient was treated with bisphosphonates for a decade, until developing an atypical femoral fracture. Teriparatide was then administered for 2 years and then changed to denosumab every 6 months, improving his bone density mass and preventing further fractures., (© 2021. International Osteoporosis Foundation and National Osteoporosis Foundation.)

Published
2022
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18. Complete heart block in an adult with systemic lupus erythematosus and recent onset of hydroxychloroquine therapy.

Author

Comín-Colet J, Sánchez-Corral MA, Alegre-Sancho JJ, Valverde J, López-Gómez D, Sabaté X, Juan-Mas A, and Esplugas E

Subjects
Adolescent, Adult, Antirheumatic Agents adverse effects, Antirheumatic Agents therapeutic use, Female, Humans, Hydroxychloroquine adverse effects, Hydroxychloroquine therapeutic use, Lupus Erythematosus, Systemic drug therapy, Middle Aged, Heart Block etiology, Lupus Erythematosus, Systemic complications
Abstract

Complete heart block (CHB) is a rare complication of systemic lupus erythematosus (SLE), mainly seen during an acute flare-up of the disease or after high-dose long-term treatment with antimalarial drugs, although anti-Ro and anti-RNP antibodies have also been implied by some authors. A 40-y-old woman developed CHB in the context of an acute flare-up of SLE, first diagnosed three years ago, having recently commenced hydroxychloroquine (HCQ) treatment. Anti-Ro and anti-RNP antibodies were also positive. No features of myocarditis were found. A temporary pacemaker was required and complete resolution was achieved on steroid therapy with withdrawal of antimalarial therapy. The characteristics of previous cases are well publicised and discussion focuses on the possible aetiology and pathogenesis of the present case.

Published
2001
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19. Septic arthiritis due to Prevotella bivia in a patient with rheumatoid arthritis.

Author

Alegre-Sancho JJ, Juanola X, Narvaez FJ, and Roig-Escofet D

Subjects
Adrenal Cortex Hormones adverse effects, Aged, Arthritis, Infectious diagnosis, Arthritis, Infectious drug therapy, Bacteroidaceae Infections drug therapy, Humans, Male, Metronidazole administration & dosage, Arthritis, Infectious microbiology, Arthritis, Rheumatoid complications, Bacteroidaceae Infections complications, Bacteroidaceae Infections etiology, Prevotella isolation & purification, Prevotella physiology
Abstract

Prevotella bivia is a gram-negative anaerobic bacteria traditionally classified in the genus Bacteroides, and usually reported in obstetric and gynecologic infections. To date, there has been only one description of infectious arthritis secondary to this germ. We report the first case of septic arthritis due to Prevotella bivia in a patient with severe, long-lasting rheumatoid arthritis (RA) treated with low doses of corticosteroids. RA is a well-known predisposing factor to septic arthritis, whose causes are multifactorial (general immunosuppression induced by the disease and its therapy, presence of prosthetic joints, etc.). However, infectious arthritis due to anaerobic bacteria is rarely observed. In general, clinical presentation varies widely: insidious onset, apyrexia, and lack of inflammatory signs or systemic disturbance are frequent features. Joint infection is generally secondary to hematogenous spread, the Bacteroides fragilis group being the most commonly isolated pathogens. Early diagnosis and prompt treatment, with drainage and debridement of the affected joint and specific antimicrobial treatment with Metronidazole are essential for a successful outcome.

Published
2000

20. Sudden cochlear hearing loss in a patient with Behçet's disease.

Author

Narváez J, Valverde-García J, Alegre-Sancho JJ, Juanola X, Clavaguera MT, and Roig-Escofet D

Subjects
Behcet Syndrome drug therapy, Behcet Syndrome physiopathology, Female, Humans, Middle Aged, Prednisone therapeutic use, Behcet Syndrome complications, Cochlear Diseases etiology, Hearing Disorders etiology
Abstract

A Behçet's disease patient developed sudden cochlear hearing loss during a flare of her disease. Prednisone and cyclosporin therapy was ineffective, probably because it was initiated late. Sensorineural hearing loss is a rare but underrecognized complication of various forms of vasculitis such as Wegener's granulomatosis, polyarteritis nodosa, giant cell arteritis and Behçet's disease. Its importance lies in the need for an early diagnosis, since prompt treatment with steroids and immunosuppressive agents may lead to restoration of hearing.

Published
1998

22. Pelvic arteriovenous malformation as a rare cause of sciatica.

Author

Narváez J, Narváez JA, Alegre-Sancho JJ, Juanola X, Aguilera C, Valverde-Garcia J, and Roig-Vilaseca D

Subjects
Aged, Aortography, Humans, Magnetic Resonance Imaging, Male, Sacrococcygeal Region abnormalities, Arteriovenous Malformations complications, Low Back Pain etiology, Sacrococcygeal Region blood supply, Sciatica etiology
Published
1997

23. Cauda equina tumour presenting as atypical sciatica.

Author

Narváez J, Alegre-Sancho JJ, Clavaguera MT, Juanola X, and Roig-Escofet D

Subjects
Adult, Diagnosis, Differential, Humans, Magnetic Resonance Imaging, Male, Pain etiology, Pain pathology, Peripheral Nervous System Neoplasms complications, Peripheral Nervous System Neoplasms pathology, Sciatica complications, Sciatica pathology, Cauda Equina, Peripheral Nervous System Neoplasms diagnosis, Sciatica diagnosis
Published
1997
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