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1. Giant Cell Arteritis: State of the Art in Diagnosis, Monitoring, and Treatment

Author

Abid Awisat, Shiri Keret, Amal Silawy, Lisa Kaly, Itzhak Rosner, Michael Rozenbaum, Nina Boulman, Aniela Shouval, Doron Rimar, and Gleb Slobodin

Subjects
diagnosis, giant cell arteritis, monitoring, treatment, Medicine, Medicine (General), R5-920
Abstract

Giant cell arteritis (GCA) is the most prevalent subtype of vasculitis in adults. In recent years, there has been substantial improvement in the diagnosis and treatment of GCA, mainly attributed to the introduction of highly sensitive diagnostic tools, incorporation of modern imaging modalities for diagnosis and monitoring of large-vessel vasculitis, and introduction of highly effective novel biological therapies that have revolutionized the field of GCA. This article reviews state-of-the-art approaches for the diagnosis, monitoring, and treatment options of GCA.

Published
2023
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2. Effectiveness and safety of tocilizumab in patients with systemic sclerosis: a propensity score matched controlled observational study of the EUSTAR cohort

Author

Pavel I Novikov, Thierry Martin, Eric Hachulla, Vanessa Smith, Maurizio Cutolo, Yannick Allanore, Camillo Ribi, Oliver Distler, Britta Maurer, Paolo Airo, Cosimo Bruni, Veronica Codullo, Luc Mouthon, Marie Vanthuyne, Elisabetta Zanatta, Simona Rednic, Ulf Müller-Ladner, Nemanja Damjanov, Anna-Maria Hoffmann-Vold, Michele Iudici, Suzana Jordan, Jean Sibilia, Mengtao Li, Roger Hesselstrand, Elise Siegert, László Czirják, Armando Gabrielli, Dominique Farge Bancel, Yolanda Braun-Moscovici, Nicolas Hunzelmann, Raffaele Pellerito, Jörg Henes, Bojana Stamenkovic, Carlo Francesco Selmi, Mohammed Tikly, Merete Engelhart, Valeria Riccieri, Cord Sunderkötter, Francesca Ingegnoli, Francesco Paolo Cantatore, Susanne Ullman, Maria Rosa Pozzi, Piotr Wiland, Juan Jose Alegre-Sancho, Brigitte Krummel-Lorenz, Ellen De Langhe, Branimir Anic, Carolina de Souza Müller, Simon Stebbings, Alessandra Vacca, Lisa Stamp, Kamal Solanki, Douglas Veale, Esthela Loyo, Edoardo Rosato, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Tim Schmeiser, Piercarlo Sarzi Puttini, Fabio Cacciapaglia, Marie-Elise Truchetet, Muriel Elhai, Juan José Alegre Sancho, Ivan Castellví, Serena Vettori, Vivien M Hsu, Masataka Kuwana, Sule Yavuz, Radim Becvar, Patricia E Carreira, Eugene J Kucharz, Bernard Coleiro, Paul Hasler, Carlos de la Puente, Svetlana Agachi, Thierry Zenone, Francesco Del Galdo, Doron Rimar, Alessandro Giollo, Lidia P Ananieva, Ina Kötter, Mislav Radic, Dominik Majewski, Kristine Herrmann, Goda Seskute, Lorinda S Chung, Laura Belloli, Ulrike Held, FJ López-Longo, Joerg H W Distler, Maria João Salvador, Simon Kuster, Klaus Steigmiller, Ana-Maria Gheorghiu, Katarzyna Romanowska-Prochnicka, Yuichiro Shirai, Chris Denton, Ruxandra M Ionescu, Stefan Heitmann Vera Ortiz-Santamaria, Walid Ahmed Abdel Atty Mohamed, Paloma Garcíadela Peña Lefebvre, and Vera Bernardino

Subjects
Medicine
Abstract

Objectives Tocilizumab showed trends for improving skin fibrosis and prevented progression of lung fibrosis in systemic sclerosis (SSc) in randomised controlled clinical trials. We aimed to assess safety and effectiveness of tocilizumab in a real-life setting using the European Scleroderma Trial and Research (EUSTAR) database.Methods Patients with SSc fulfilling the American College of Rheumatology (ACR)/EULAR 2013 classification criteria, with baseline and follow-up visits at 12±3 months, receiving tocilizumab or standard of care as the control group, were selected. Propensity score matching was applied. Primary endpoints were the modified Rodnan skin score (mRSS) and FVC at 12±3 months compared between the groups. Secondary endpoints were the percentage of progressive/regressive patients for skin and lung at 12±3 months.Results Ninety-three patients with SSc treated with tocilizumab and 3180 patients with SSc with standard of care fulfilled the inclusion criteria. Comparison between groups did not show significant differences, but favoured tocilizumab across all predefined primary and secondary endpoints: mRSS was lower in the tocilizumab group (difference −1.0, 95% CI −3.7 to 1.8, p=0.48). Similarly, FVC % predicted was higher in the tocilizumab group (difference 1.5 (−6.1 to 9.1), p=0.70). The percentage of progressive/regressive patients favoured tocilizumab over controls. These results were robust regarding the sensitivity analyses. Safety analysis confirmed previously reported adverse event profiles.Conclusion Although this large, observational, controlled, real-life EUSTAR study did not show significant effectiveness of tocilizumab on skin and lung fibrosis, the consistency of direction of all predefined endpoints generates hypothesis for potential effectiveness in a broader SSc population.

Published
2022
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4. Use of platelet inhibitors for digital ulcers related to systemic sclerosis: EUSTAR study on derivation and validation of the DU-VASC model

Author

Alexandru Garaiman 1, Klaus Steigmiller 2, Catherine Gebhard 3, Carina Mihai 1, Rucsandra Dobrota 1, Cosimo Bruni 4, Marco Matucci-Cerinic 5, Joerg Henes 6, Jeska de Vries-Bouwstra 7, Vanessa Smith 8, Andrea Doria 9, Yannick Allanore 10, Lorenzo Dagna 11, Branimir Anić 12, Carlomaurizio Montecucco 13, Otylia Kowal-Bielecka 14, Mickael Martin 15, Yoshiya Tanaka 16, Anna-Maria Hoffmann-Vold 17, Ulrike Held 2, Oliver Distler 1, Mike Oliver Becker 1, EUSTAR Silvia Bellando Randone, Gemma Lepri, Ulrich Walker, Florenzo Iannone, Suzana Jordan, Radim Becvar, Ewa Gindzienska-Sieskiewicz, Katarzyna Karaszewska, Maurizio Cutolo, Giovanna Cuomo, Elise Siegert, Simona Rednic, Jérome Avouac, Carole Desbas, Roberto Caporali, Lorenzo Cavagna, Patricia E Carreira, Srdan Novak, László Czirják, Michele Iudici, Eugene J Kucharz, Elisabetta Zanatta, Bernard Coleiro, Gianluca Moroncini, Dominique Farge Bancel, Paolo Airò, Roger Hesselstr, Mislav Radic, Alexandra Balbir-Gurman, Nicolas Hunzelmann, Raffaele Pellerito, Alessandro Giollo, Jadranka Morovic-Vergles, Christopher Denton, Nemanja Damjanov, Ann-Christian Pecher, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Paul Hasler, Ivan Foeldvari, Maria João Salvador, Bojana Stamenkovic, Carlo Francesco Selmi, Lidia P Ananieva, Ariane Herrick, Ulf Müller-Ladner, Raffaele De Palma, Merete Engelhart, Gabriela Szücs, Carlos de la Puente, Øyvind Midtvedt, Torhild Garen, Håvard Fretheim, Eric Hachulla, Valeria Riccieri, Ruxandra Maria Ionescu, Ana Maria Gheorghiu, Cord Sunderkötter, Jörg Distler, Francesca Ingegnoli, Luc Mouthon, Francesco Paolo Cantatore, Susanne Ullman, Maria Rosa Pozzi, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Marko Baresic, Miroslav Mayer, Sule Yavuz, Brigitte Granel, Carolina de Souza Müller, Svetlana Agachi, Simon Stebbings, D'Alessandro Mathieu, Alessandra Vacca, Kamal Solanki, Douglas Veale, Esthela Loyo, Carmen Tineo, Mengtao Li, Edoardo Rosato, Fahrettin Oksel, Figen Yargucu, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Britta Maurer, Jacob van Laar, Marzena Olesinska, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Jorge Juan Gonzalez Martin, Jean Sibilia, Ira Litinsky, Francesco Del Galdo, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianch, Breno Valdetaro Bianchi, Ivan Castellví, Massimiliano Limonta, Doron Rimar, Maura Couto, François Spertini, Antonella Marcoccia, Sarah Kahl, Ivien M Hsu, Thierry Martin, Sergey Moiseev, Pavel Novikov, Lorinda S Chung, Tim Schmeiser, Dominik Majewski, Zbigniew Zdrojewski, Julia Martínez-Barrio, Vera Bernardino, Gabriela Riemekasten, Yair Levy, Elena Rezus, Omer Nuri Pamuk, Piercarlo Sarzi Puttini, Hadi Poormoghim, Ina Kötter, Francis Gaches, Laura Belloli, Petros Sfikakis, Daniel Furst, Ana-Maria Ramazan, H U Scherer, Tom W J Huizinga, Marie-Elise Truchetet, Alain Lescoat, Giacomo De Luca, Corrado Campochiaro, J M van Laar, Lidia Rudnicka, Susana Oliveira, Fabiola Atzeni, Masataka Kuwana, Arsene Mekinian, Cédric L, Mathieu Puyade, Pascal Roblot, Satoshi Kubo, Yasuyuki Todoroki, 1, Alexandru Garaiman, 2, Klaus Steigmiller, 3, Catherine Gebhard, 1, Carina Mihai, 1, Rucsandra Dobrota, 4, Cosimo Bruni, 5, Marco Matucci-Cerinic, 6, Joerg Hene, 7, Jeska de Vries-Bouwstra, 8, Vanessa Smith, 9, Andrea Doria, Allanore 10, Yannick, Dagna 11, Lorenzo, Anić 12, Branimir, Montecucco 13, Carlomaurizio, Kowal-Bielecka 14, Otylia, Martin 15, Mickael, Tanaka 16, Yoshiya, Hoffmann-Vold 17, Anna-Maria, 2, Ulrike Held, 1, Oliver Distler, 1, Mike Oliver Becker, Silvia Bellando Randone, Eustar, Lepri, Gemma, Walker, Ulrich, Iannone, Florenzo, Jordan, Suzana, Becvar, Radim, Gindzienska-Sieskiewicz, Ewa, Karaszewska, Katarzyna, Cutolo, Maurizio, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Avouac, Jérome, Desbas, Carole, Caporali, Roberto, Cavagna, Lorenzo, E Carreira, Patricia, Novak, Srdan, Czirják, László, Iudici, Michele, J Kucharz, Eugene, Zanatta, Elisabetta, Coleiro, Bernard, Moroncini, Gianluca, Farge Bancel, Dominique, Airò, Paolo, Hesselstr, Roger, Radic, Mislav, Balbir-Gurman, Alexandra, Hunzelmann, Nicola, Pellerito, Raffaele, Giollo, Alessandro, Morovic-Vergles, Jadranka, Denton, Christopher, Damjanov, Nemanja, Pecher, Ann-Christian, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Hasler, Paul, Foeldvari, Ivan, João Salvador, Maria, Stamenkovic, Bojana, Francesco Selmi, Carlo, P Ananieva, Lidia, Herrick, Ariane, Müller-Ladner, Ulf, DE PALMA, Raffaele, Engelhart, Merete, Szücs, Gabriela, de la Puente, Carlo, Midtvedt, Øyvind, Garen, Torhild, Fretheim, Håvard, Hachulla, Eric, Riccieri, Valeria, Maria Ionescu, Ruxandra, Maria Gheorghiu, Ana, Sunderkötter, Cord, Distler, Jörg, Ingegnoli, Francesca, Mouthon, Luc, Paolo Cantatore, Francesco, Ullman, Susanne, Rosa Pozzi, Maria, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Baresic, Marko, Mayer, Miroslav, Yavuz, Sule, Granel, Brigitte, de Souza Müller, Carolina, Agachi, Svetlana, Stebbings, Simon, Mathieu, D'Alessandro, Vacca, Alessandra, Solanki, Kamal, Veale, Dougla, Loyo, Esthela, Tineo, Carmen, Li, Mengtao, Rosato, Edoardo, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Foti, Rosario, Ancuta, Codrina, Maurer, Britta, van Laar, Jacob, Olesinska, Marzena, Kayser, Cristiane, Fathi, Nihal, García de la Peña Lefebvre, Paloma, Juan Gonzalez Martin, Jorge, Sibilia, Jean, Litinsky, Ira, Del Galdo, Francesco, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Bianch, Washington, Valdetaro Bianchi, Breno, Castellví, Ivan, Limonta, Massimiliano, Rimar, Doron, Couto, Maura, Spertini, Françoi, Marcoccia, Antonella, Kahl, Sarah, M Hsu, Ivien, Martin, Thierry, Moiseev, Sergey, Novikov, Pavel, S Chung, Lorinda, Schmeiser, Tim, Majewski, Dominik, Zdrojewski, Zbigniew, Martínez-Barrio, Julia, Bernardino, Vera, Riemekasten, Gabriela, Levy, Yair, Rezus, Elena, Nuri Pamuk, Omer, Sarzi Puttini, Piercarlo, Poormoghim, Hadi, Kötter, Ina, Gaches, Franci, Belloli, Laura, Sfikakis, Petro, Furst, Daniel, Ramazan, Ana-Maria, U Scherer, H, J Huizinga, Tom W, Truchetet, Marie-Elise, Lescoat, Alain, De Luca, Giacomo, Campochiaro, Corrado, M van Laar, J, Rudnicka, Lidia, Oliveira, Susana, Atzeni, Fabiola, Kuwana, Masataka, Mekinian, Arsene, L, Cédric, Puyade, Mathieu, Roblot, Pascal, Kubo, Satoshi, Todoroki, Yasuyuki, and University of Zurich

Subjects
prognostic prediction model, Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, digital ulcers, platelets inhibitors, 610 Medicine & health, Pharmacology (medical), SSc, 10060 Epidemiology, Biostatistics and Prevention Institute (EBPI)
Abstract

Objective To develop and validate the prognostic prediction model DU-VASC to assist the clinicians in decision-making regarding the use of platelet inhibitors (PIs) for the management of digital ulcers in patients with systemic sclerosis. Secondly, to assess the incremental value of PIs as predictor. Methods We analysed patient data from the European Scleroderma Trials and Research group registry (one time point assessed). Three sets of derivation/validation cohorts were obtained from the original cohort. Using logistic regression, we developed a model for prediction of digital ulcers (DUs). C-Statistics and calibration plots were calculated to evaluate the prediction performance. Variable importance plots and the decrease in C-statistics were used to address the importance of the predictors. Results Of 3710 patients in the original cohort, 487 had DUs and 90 were exposed to PIs. For the DU-VASC model, which includes 27 predictors, we observed good calibration and discrimination in all cohorts (C-statistic = 81.1% [95% CI: 78.9%, 83.4%] for the derivation and 82.3% [95% CI: 779.3%, 85.3%] for the independent temporal validation cohort). Exposure to PIs was associated with absence of DUs and was the most important therapeutic predictor. Further important factors associated with absence of DUs were lower modified Rodnan skin score, anti-Scl-70 negativity and normal CRP. Conversely, the exposure to phosphodiesterase-5 inhibitor, prostacyclin analogues or endothelin receptor antagonists seemed to be associated with the occurrence of DUs. Nonetheless, previous DUs remains the most impactful predictor of DUs. Conclusion The DU-VASC model, with good calibration and discrimination ability, revealed that PI treatment was the most important therapy-related predictor associated with reduced DU occurrence.

Published
2022

5. Differentially expressed genes in systemic sclerosis: Towards predictive medicine with new molecular tools for clinicians

Author

Shiri Keret, Doron Rimar, Pauline Lansiaux, Erik Feldman, Alain Lescoat, Neta Milman, Dominique Farge, Technion - Israel Institute of Technology [Haifa], Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Recherche clinique appliquée à l'hématologie (URP_3518), Université Paris Cité (UPCité), Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), École des Hautes Études en Santé Publique [EHESP] (EHESP), and McGill University = Université McGill [Montréal, Canada]

Subjects
[SDV]Life Sciences [q-bio], Immunology, Machine learning, Immunology and Allergy, Systemic sclerosis, Intrinsic subsets, Autologous hematopoietic stem cell transplantation, RNA gene expression
Abstract

International audience; Systemic sclerosis (SSc) is a rare and chronic autoimmune disease characterized by a pathogenic triad of immune dysregulation, vasculopathy, and progressive fibrosis. Clinical tools commonly used to assess patients, including the modified Rodnan skin score, difference between limited or diffuse forms of skin involvement, presence of lung, heart or kidney involvement, or of various autoantibodies, are important prognostic factors, but still fail to reflect the large heterogeneity of the disease. SSc treatment options are diverse, ranging from conventional drugs to autologous hematopoietic stem cell transplantation, and predicting response is challenging. Genome-wide technologies, such as high throughput microarray analyses and RNA sequencing, allow accurate, unbiased, and broad assessment of alterations in expression levels of multiple genes. In recent years, many studies have shown robust changes in the gene expression profiles of SSc patients compared to healthy controls, mainly in skin tissues and peripheral blood cells. The objective analysis of molecular patterns in SSc is a powerful tool that can further classify SSc patients with similar clinical phenotypes and help predict response to therapy. In this review, we describe the journey from the first discovery of differentially expressed genes to the identification of enriched pathways and intrinsic subsets identified in SSc, using machine learning algorithms. Finally, we discuss the use of these new tools to predict the efficacy of various treatments, including stem cell transplantation. We suggest that the use of RNA gene expression-based classifications according to molecular subsets may bring us one step closer to precision medicine in Systemic Sclerosis.

Published
2023

6. Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from the EUSTAR cohort

Author

Elisabetta Zanatta 1, Dörte Huscher 2, Augusta Ortolan 1, Jérôme Avouac 3, Paolo Airò 4, Alexandra Balbir-Gurman 5, Elise Siegert 6, Marco Matucci Cerinic 7, Franco Cozzi 8, Gabriela Riemekasten 9, Anna-Maria Hoffmann-Vold 10, Oliver Distler 11, Armando Gabrielli 12, Stefan Heitmann 13, Nicolas Hunzelmann 14, Carlomaurizio Montecucco 15, Jadranka Morovic-Vergles 16, Camillo Ribi 17, Andrea Doria 1, Yannick Allanore 3, EUSTAR collaborators, Giovanna Cuomo, Gianluca Moroncini, Jiri Stork, Fiorenzo Iannone, Ulrich Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, Eric Hachulla, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anic, Maria Üprus, Brigitte Granel, Alessandra Vacca, Cristina-Mihaela Tanaseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros Sfikakis, Maurizio Cutolo, Patricia E Carreira, Rosario Foti, Srdan Novak, Michele Iudici, Mislav Radic, Raffaele Pellerito, Carlo Francesco Selmi, Lidia P Ananieva, Gabriela Szücs, Carlos de la Puente, Ruxandra Maria Ionescu, Jörg Distler, Maria Rosa Pozzi, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz, Carolina de Souza Müller, Svetlana Agachi, Douglas Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, Ivan Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Christopher Denton, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria, Michaela Kohm, Bojana Stamenkovic, 1, Elisabetta Zanatta, 2, Dörte Huscher, 1, Augusta Ortolan, 3, Jérôme Avouac, 4, Paolo Airò, 5, Alexandra Balbir-Gurman, 6, Elise Siegert, 7, Marco Matucci Cerinic, 8, Franco Cozzi, 9, Gabriela Riemekasten, Hoffmann-Vold 10, Anna-Maria, Distler 11, Oliver, Gabrielli 12, Armando, Heitmann 13, Stefan, Hunzelmann 14, Nicola, Montecucco 15, Carlomaurizio, Morovic-Vergles 16, Jadranka, Ribi 17, Camillo, 1, Andrea Doria, 3, Yannick Allanore, Collaborators, Eustar, Cuomo, Giovanna, Moroncini, Gianluca, Stork, Jiri, Iannone, Fiorenzo, Walker, Ulrich, Bertoldo, Eugenia, Krasowska, Dorota, João Salvador, Maria, Tikly, Mohammed, Hachulla, Eric, Riccieri, Valeria, Sha, Ami, Maria Gheorghiu, Ana, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Paolo Cantatore, Francesco, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Anic, Branimir, Üprus, Maria, Granel, Brigitte, Vacca, Alessandra, Tanaseanu, Cristina-Mihaela, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Limonta, Massimiliano, Rimar, Doron, Sfikakis, Petro, Cutolo, Maurizio, E Carreira, Patricia, Foti, Rosario, Novak, Srdan, Iudici, Michele, Radic, Mislav, Pellerito, Raffaele, Francesco Selmi, Carlo, P Ananieva, Lidia, Szücs, Gabriela, de la Puente, Carlo, Maria Ionescu, Ruxandra, Distler, Jörg, Rosa Pozzi, Maria, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Yavuz, Sule, de Souza Müller, Carolina, Agachi, Svetlana, Veale, Dougla, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Maurer, Britta, Castellví, Ivan, Spertini, Françoi, Solanki, Kamal, Del Papa, Nicoletta, Espinosa, Gerard, Czirják, László, Coleiro, Bernard, Farge Bancel, Dominique, Denton, Christopher, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Kohm, Michaela, and Stamenkovic, Bojana

Subjects
interstitial lung disease, Scleroderma, Systemic, Hypertension, Pulmonary, disease subset, anti-topoisomerase I, Scleroderma, Systemic sclerosis, cutaneous form, outcome, Phenotype, Rheumatology, Scleroderma, Limited, Antibodies, Antinuclear, Scleroderma, Diffuse, Humans, Pharmacology (medical), Lung Diseases, Interstitial
Abstract

Objectives To characterize patients with positive anti-topoisomerase I (ATA) in lcSSc. Methods SSc patients enrolled in the EUSTAR cohort with a disease duration of ≤3 years at database entry were considered. We assessed the risk of major organ involvement in the following groups: ATA-lcSSc vs ACA-lcSSc and vs ANA without specificity (ANA)-lcSSc, and ATA-lcSSc vs ATA-dcSSc. Cox regression models with time-dependent covariates were performed with the following outcomes: new-onset interstitial lung disease (ILD), ILD progression [forced vital capacity (FVC) decline ≥10% and ≥5% vs values at ILD diagnosis), primary myocardial involvement (PMI), pulmonary hypertension (PH), any organ involvement and all-cause mortality. Results We included 1252 patients [194 ATA-lcSSc (15.5%)], with 7.7 years (s.d. 3.5) of follow-up. ILD risk was higher in ATA-lcSSc vs ACA- and ANA-lcSSc and similar to ATA-dcSSc, although with less frequent restrictive lung disease. The risk of FVC decline ≥10% (35% of ATA-lcSSc) was lower in ATA-lcSSc than in ATA-dcSSc, whereas FVC decline ≥5% occurs similarly between ATA-lcSSc (58% of patients) and other SSc subsets, including ATA-dcSSc. The risk of PMI was similar in ATA-lcSSc and ANA-lcSSc but lower than in ACA-lcSSc; no difference in PH and mortality risk was observed among lcSSc subsets. The risk of any organ involvement, PMI and PH was lower and the mortality tended to be lower in ATA-lcSSc vs ATA-dcSSc. Conclusion ATA-lcSSc patients have a high risk of ILD, albeit with a lower risk of progression compared with ATA-dcSSc, supporting careful screening for ILD in this subgroup.

Published
2022

7. Autoinflammatory Diseases and Vasculitis: The Missing Link

Author

Shiri Keret, Gleb Slobodin, Abid Awisat, and Doron Rimar

Subjects
Rheumatology, Immunology, Immunology and Allergy
Abstract

Familial Mediterranean fever (FMF) has been reported to be associated with vasculitis, including polyarteritis nodosa (PAN), yet vasculitis is not considered a manifestation of FMF.1 A 29-year-old male, diagnosed with FMF (compound heterozygote for V726A/E148q) at the age of 10 years, was admitted to our ward due to a recent exacerbation of FMF attacks.

Published
2022

9. Glucocorticoids prescribing practices in systemic sclerosis: an analysis of the EUSTAR database

Author

Iudici, Michele, Mongin, Denis, Siegert, Elise, Carreira, Patricia E, Distler, Jörg, Henes, Jörg, Zanatta, Elisabetta, Hachulla, Eric, De Luca, Giacomo, Müller, Carolina de Souza, Santiago, Tânia, Tandaipan, José-Luis, Bianchi, Breno Valdetaro, De Santis, Maria, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Distler, Oliver, Courvoisier, Delphine Sophie, Giovanna Cuomo, Gianluca Moroncini, Jiri Stork, Fiorenzo Iannone, Ulrich Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anic, Maria Üprus, Brigitte Granel, Alessandra Vacca, Cristina-Mihaela Tanaseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros Sfikakis, Maurizio Cutolo, Rosario Foti, Srdan Novak, Mislav Radic, Raffaele Pellerito, Carlo Francesco Selmi Rozzano, Lidia P Ananieva, Gabriela Szűcs, Carlos de la Puente, Ruxandra Maria Ionescu, Maria Rosa Pozzi, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz Altunizade, Svetlana Agachi, Douglas Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, Iván Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Christopher Denton, Nemanja Damjanov, Vera Ortiz Santamaria Granollers, Michaela Kohm, Bojana Stamenkovic, Yannick Allanore, Paolo Airo, Alexandra Balbir-Gurman, Marco Matucci Cerinic, Gabriela Riemekasten, Stefan Heitmann, Nicolas Hunzelmann, Carlomaurizio Montecucco, Jadranka Morovic-Vergles, Camillo Ribi, Michele, Iudici, Denis, Mongin, Elise, Siegert, Patricia E, Carreira, Jörg, Distler, Jörg, Hene, Elisabetta, Zanatta, Eric, Hachulla, Giacomo, De Luca, Carolina de Souza, Müller, Tânia, Santiago, José-Luis, Tandaipan, Breno Valdetaro, Bianchi, Maria, De Santi, Anna-Maria, Hoffmann-Vold, Armando, Gabrielli, Oliver, Distler, Courvoisier, Sophie, Delphine, Cuomo, Giovanna, Moroncini, Gianluca, Stork, Jiri, Iannone, Fiorenzo, Walker, Ulrich, Bertoldo, Eugenia, Krasowska, Dorota, João Salvador, Maria, Tikly, Mohammed, Riccieri, Valeria, Sha, Ami, Maria Gheorghiu, Ana, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Paolo Cantatore, Francesco, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Anic, Branimir, Üprus, Maria, Granel, Brigitte, Vacca, Alessandra, Tanaseanu, Cristina-Mihaela, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Limonta, Massimiliano, Rimar, Doron, Sfikakis, Petro, Cutolo, Maurizio, Foti, Rosario, Novak, Srdan, Radic, Mislav, Pellerito, Raffaele, Francesco Selmi Rozzano, Carlo, P Ananieva, Lidia, Szűcs, Gabriela, de la Puente, Carlo, Maria Ionescu, Ruxandra, Rosa Pozzi, Maria, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Yavuz Altunizade, Sule, Agachi, Svetlana, Veale, Dougla, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Maurer, Britta, Castellví, Iván, Spertini, Françoi, Solanki, Kamal, Del Papa, Nicoletta, Espinosa, Gerard, Czirják, László, Coleiro, Bernard, Farge Bancel, Dominique, Denton, Christopher, Damjanov, Nemanja, Ortiz Santamaria Granollers, Vera, Kohm, Michaela, Stamenkovic, Bojana, Allanore, Yannick, Airo, Paolo, Balbir-Gurman, Alexandra, Matucci Cerinic, Marco, Riemekasten, Gabriela, Heitmann, Stefan, Hunzelmann, Nicola, Montecucco, Carlomaurizio, Morovic-Vergles, Jadranka, and Ribi, Camillo

Subjects
Rheumatology, systemic sclerosis, Pharmacology (medical), epidemiology, glucocorticoid
Abstract

Objectives To estimate the prevalence of long-term exposure to glucocorticoids (GCs) and to identify factors associated with, and variations in prescribing practices over time and across recruiting countries. Methods We included patients with SSc having a visit recorded in the EUSTAR database from January 2013 onward. We analysed the prevalence and the main features of GCs users, their exposure to GCs over time, and their GCs dosages. Multivariable linear regression was used to analyse the factors identified as associated with GCs intake duration. Time trends, and variations in GCs utilization across recruiting countries were explored. Missing data were imputed using multiple imputation with chained equations. Results The 9819 patients included were mostly females (85%), the majority had lcSSc (73%), and the median age was 58 years. At baseline, 34% of patients (n = 2769/8109) (48% dcSSc vs 29% lcSSc) were on GCs, and the median dose was 7.5 mg/day. GCs users were more frequently males and anti-Scl70 positive, and more commonly had dcSSc and more severe disease. On average, GCs users spent 25% of their follow-up time (median 33.2 months) on GCs, with no significant between-subsets difference. Notably, 33% (n = 971/2959) and 22% (n = 647/2959) of patients followed up for >1 year had received GCs for >6 and >12 months, respectively. Multivariable analysis showed that patient and disease characteristics poorly explained the variability in GCs exposure (adjusted-R2 = 0.06, P Conclusions GCs are widely and long-term prescribed in SSc, with significant between-countries and within-country differences. A gradual decrease in their utilization has been observed.

Published
2022

10. 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. Nailfold video capillaroscopy as a useful diagnostic tool in systemic vasculitis

Author

Shiri, Keret, Jan, Mazzawi, Gleb, Slobodin, Ori, Rimar, Itzhak, Rosner, Michael, Rozenbaum, Lisa, Kaly, Nina, Boulman, Abid, Awisat, Aniela, Shouval, Francesca, Ingegnoli, and Doron, Rimar

Subjects
Adult, Vasculitis, Scleroderma, Systemic, Nails, Systemic Vasculitis, Humans, Raynaud Disease, Cell Biology, Cardiology and Cardiovascular Medicine, Biochemistry, Capillaries, Microscopic Angioscopy, Retrospective Studies
Abstract

Nailfold video capillaroscopy (NVC) enables us a direct view of the microvasculature. Only several capillaroscopy studies in adult patients with vasculitis have been reported.To characterize NVC changes in vasculitis.Vasculitis patients and healthy controls were evaluated by NVC. NVC changes associated with vasculitis were assessed retrospectively in a cohort of 100 patients with Raynaud's phenomenon (RP).17 patients with active vasculitis and 8 patients with vasculitis in remission were compared to 25 age and sex-matched healthy controls. Active vasculitis patients demonstrated higher rates of neoangiogenesis and capillary loss in comparison to other groups. Two novel NVC abnormalities were observed in patients with vasculitis: "Rolling" (slow capillary flow) and "peri-capillary stippling" (PCS), small deposits that may represent capillary leak. PCS was observed exclusively in 5 of 17 patients with active vasculitis. Retrospectively, we were able to detect PCS also in 14 % of 100 patients that were evaluated for RP, of whom 64 % were diagnosed with scleroderma or a related disorder.Patients with active vasculitis demonstrate frequent capillary abnormalities. Although these abnormalities are non-specific, we suggest that their combination may aid the diagnosis of vasculitis. Future studies are needed to validate our findings.

Published
2022

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