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6. Nailfold Capillaroscopy

Author

Smith, Vanessa, Hysa, Elvis, Ingegnoli, Francesca, Cutolo, Maurizio, Wigley, Fredrick M., editor, Herrick, Ariane L., editor, and Flavahan, Nicholas A., editor

Published
2024
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8. Peripheral and ocular microvascular alterations in systemic sclerosis: observations from capillaroscopic assessments, perfusion peripheral analysis, and optical coherence tomography angiography

Author

Cutolo, Carlo Alberto, Cere, Andrea, Toma, Paola, Cannavacciuolo, Tommaso, Toma, Chiara, Balito, Serena, Gerli, Veronica, Smith, Vanessa, Sulli, Alberto, Paolino, Sabrina, Gotelli, Emanuele, Traverso, Carlo Enrico, Nicolò, Massimo, Cutolo, Maurizio, and Hysa, Elvis

Published
2024
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10. Tailoring the treatment of inflammatory rheumatic diseases by a better stratification and characterization of the clinical patient heterogeneity. Findings from a systematic literature review and experts' consensus

Author

Ruscitti, Piero, Allanore, Yannick, Baldini, Chiara, Barilaro, Giuseppe, Bartoloni Bocci, Elena, Bearzi, Pietro, Bellis, Elisa, Berardicurti, Onorina, Biaggi, Alice, Bombardieri, Michele, Cantarini, Luca, Cantatore, Francesco Paolo, Caporali, Roberto, Caso, Francesco, Cervera, Ricard, Ciccia, Francesco, Cipriani, Paola, Chatzis, Loukas, Colafrancesco, Serena, Conti, Fabrizio, Corberi, Erika, Costa, Luisa, Currado, Damiano, Cutolo, Maurizio, D'Angelo, Salvatore, Del Galdo, Francesco, Di Cola, Ilenia, Di Donato, Stefano, Distler, Oliver, D'Onofrio, Bernardo, Doria, Andrea, Fautrel, Bruno, Fasano, Serena, Feist, Eugen, Fisher, Benjamin A., Gabini, Marco, Gandolfo, Saviana, Gatto, Mariele, Genovali, Irene, Gerli, Roberto, Grembiale, Rosa Daniela, Guggino, Giuliana, Hoffmann-Vold, Anna Maria, Iagnocco, Annamaria, Iaquinta, Francesco Salvatore, Liakouli, Vasiliki, Manoussakis, Menelaos N., Marino, Annalisa, Mauro, Daniele, Montecucco, Carlomaurizio, Mosca, Marta, Naty, Saverio, Navarini, Luca, Occhialini, Daniele, Orefice, Valeria, Perosa, Federico, Perricone, Carlo, Pilato, Andrea, Pitzalis, Costantino, Pontarini, Elena, Prete, Marcella, Priori, Roberta, Rivellese, Felice, Sarzi-Puttini, Piercarlo, Scarpa, Raffaele, Sebastiani, Giandomenico, Selmi, Carlo, Shoenfeld, Yehuda, Triolo, Giovanni, Trunfio, Francesca, Yan, Qingran, Tzioufas, Athanasios G., and Giacomelli, Roberto

Published
2024
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13. A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc.

Author

Saketkoo, Lesley, Frech, Tracy, Varjú, Cecília, Domsic, Robyn, Farrell, Jessica, Gordon, Jessica, Mihai, Carina, Sandorfi, Nora, Shapiro, Lee, Poole, Janet, Volkmann, Elizabeth, Lammi, Monika, McAnally, Kendra, Alexanderson, Helene, Pettersson, Henrik, Hant, Faye, Kuwana, Masataka, Shah, Ami, Smith, Vanessa, Hsu, Vivien, Kowal-Bielecka, Otylia, Assassi, Shervin, Cutolo, Maurizio, Kayser, Cristiane, Shanmugam, Victoria, Vonk, Madelon, Fligelstone, Kim, Baldwin, Nancy, Connolly, Kerri, Ronnow, Anneliese, Toth, Beata, Suave, Maureen, Farrington, Sue, Bernstein, Elana, Crofford, Leslie, Czirják, László, Jensen, Kelly, Hinchclif, Monique, Hudson, Marie, Lammi, Matthew, Mansour, Jennifer, Morgan, Nadia, Mendoza, Fabian, Nikpour, Mandana, Pauling, John, Riemekasten, Gabriela, Russell, Anne-Marie, Scholand, Mary, Seigart, Elise, Rodriguez-Reyna, Tatiana, Hummers, Laura, Walker, Ulrich, and Steen, Virginia

Subjects
Disability, Interstitial lung disease, Pulmonary fibrosis, Pulmonary hypertension, Quality of life, Renal crisis, Scleroderma, Symptom burden, Systemic sclerosis, Humans, Hypertension, Pulmonary, Lung, Lung Diseases, Interstitial, Patient Care, Scleroderma, Systemic
Abstract

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

Published
2021

15. Long-term outcomes of COVID-19 vaccination in patients with rare and complex connective tissue diseases: The ERN-ReCONNET VACCINATE study

Author

Tani, Chiara, Cardelli, Chiara, Depascale, Roberto, Gamba, Anna, Iaccarino, Luca, Doria, Andrea, Bandeira, Matilde, Dinis, Sara Paiva, Romão, Vasco C., Gotelli, Emanuele, Paolino, Sabrina, Cutolo, Maurizio, Di Giosaffatte, Niccolò, Ferraris, Alessandro, Grammatico, Paola, Cavagna, Lorenzo, Codullo, Veronica, Montecucco, Carlomaurizio, Longo, Valentina, Beretta, Lorenzo, Cavazzana, Ilaria, Fredi, Micaela, Peretti, Silvia, Guiducci, Serena, Matucci-Cerinic, Marco, Bombardieri, Stefano, Burmester, Gerd R., Fonseca, João E., Frank, Charissa, Galetti, Ilaria, Hachulla, Eric, Müller-Ladner, Ulf, Schneider, Matthias, Smith, Vanessa, Tamirou, Farah, Van Laar, Jacob M., Vieira, Ana, D'Urzo, Rossella, Cannizzo, Sara, Gaglioti, Andrea, Marinello, Diana, Talarico, Rosaria, and Mosca, Marta

Published
2023
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16. Effect of Nintedanib on Lung Function in Patients With Systemic Sclerosis-Associated Interstitial Lung Disease: Further Analyses of a Randomized, Double-Blind, Placebo-Controlled Trial.

Author

Maher, Toby, Mayes, Maureen, Kreuter, Michael, Volkmann, Elizabeth, Aringer, Martin, Castellvi, Ivan, Cutolo, Maurizio, Stock, Christian, Schoof, Nils, Alves, Margarida, and Raghu, Ganesh

Subjects
Adult, Aged, Disease Progression, Double-Blind Method, Female, Humans, Indoles, Lung, Lung Diseases, Interstitial, Male, Middle Aged, Protein Kinase Inhibitors, Scleroderma, Systemic, Treatment Outcome
Abstract

OBJECTIVE: In the SENSCIS trial in subjects with systemic sclerosis-associated interstitial lung disease (SSc-ILD), nintedanib reduced the rate of decline in forced vital capacity (FVC) over 52 weeks by 44% versus placebo. This study was undertaken to investigate the effects of nintedanib on categorical changes in FVC and other measures of ILD progression. METHODS: In post hoc analyses, we assessed the proportions of subjects with categorical changes in FVC % predicted at week 52 and the time to absolute decline in FVC of ≥5% predicted or death and absolute decline in FVC of ≥10% predicted or death. RESULTS: A total of 288 subjects received nintedanib and 288 subjects received placebo. At week 52, in subjects treated with nintedanib and placebo, respectively, 55.7% and 66.3% had any decline in FVC % predicted, 13.6% and 20.1% had a decline in FVC of >5% to ≤10% predicted, and 3.5% and 5.2% had a decline in FVC of >10% to ≤15% predicted; 34.5% and 43.8% had a decrease in FVC of ≥3.3% predicted (proposed minimal clinically important difference [MCID] for worsening of FVC), while 23.0% and 14.9% had an increase in FVC of ≥3.0% predicted (proposed MCID for improvement in FVC). Over 52 weeks, the hazard ratio (HR) for an absolute decline in FVC of ≥5% predicted or death with nintedanib versus placebo was 0.83 (95% confidence interval [95% CI] 0.66-1.06) (P = 0.14), and the HR for an absolute decline in FVC of ≥10% predicted was 0.64 (95% CI 0.43-0.95) (P = 0.029). CONCLUSION: These results suggest that nintedanib has a clinically relevant benefit on the progression of SSc-ILD.

Published
2021

18. Predictors of progression in systemic sclerosis patients with interstitial lung disease.

Author

Distler, Oliver, Assassi, Shervin, Cottin, Vincent, Cutolo, Maurizio, Danoff, Sonye, Denton, Christopher, Distler, Jörg, Hoffmann-Vold, Anna-Maria, Johnson, Sindhu, Müller Ladner, Ulf, Smith, Vanessa, Volkmann, Elizabeth, and Maher, Toby

Subjects
Biomarkers, Carbon Monoxide, Disease Progression, Humans, Lung, Lung Diseases, Interstitial, Respiratory Function Tests, Scleroderma, Systemic, Tomography, X-Ray Computed
Abstract

Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc.There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. Chest auscultation, presence of shortness of breath and pulmonary function testing are important diagnostic tools, but lack sensitivity to detect early ILD. Baseline screening with high-resolution computed tomography (HRCT) is therefore necessary to confirm an SSc-ILD diagnosis. Once diagnosed with SSc-ILD, patients clinical courses are variable and difficult to predict, although certain patient characteristics and biomarkers are associated with disease progression. It is important to monitor patients with SSc-ILD for signs of disease progression, although there is no consensus about which diagnostic tools to use or how often monitoring should occur. In this article, we review methods used to define and predict disease progression in SSc-ILD.There is no valid definition of SSc-ILD disease progression, but we suggest that either a decline in forced vital capacity (FVC) from baseline of ≥10%, or a decline in FVC of 5-9% in association with a decline in diffusing capacity of the lung for carbon monoxide of ≥15% represents progression. An increase in the radiographic extent of ILD on HRCT imaging would also signify progression. A time period of 1-2 years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression.

Published
2020

21. Does the Impact of COVID‐19 on Patients With Systemic Sclerosis Change Over Time?

Author

Deibel, Elisabeth, Carreira, Patricia E., Vonk, Madelon, del Papa, Nicoletta, Bečvář, Radim, Guillén‐Del‐Castillo, Alfredo, Campochiaro, Corrado, Poormoghim, Hadi, Liem, Sophie, Lazzaroni, Maria‐Grazia, Giollo, Alessandro, Mekinian, Arsène, de Vries‐Bouwstra, Jeska, De Santis, Maria, Balbir‐Gurman, Alexandra, Mihai, Carina, De Luca, Giacomo, Moiseev, Sergey, Zanatta, Elisabetta, Foti, Rosario, Rednic, Simona, Denton, Christopher, Cutolo, Maurizio, Belloli, Laura, Airo, Paolo, Garzanova, Liudmila, Moroncini, Gianluca, İnanç, Murat, Panopoulos, Stylianos, Tandaipan, Jose‐Luis, Chatelus, Emmanuel, Rosato, Edoardo, Kuwana, Masataka, Yavuz, Sule, Alegre‐Sancho, Juan J., Smith, Vanessa, Szűcs, Gabriella, Henes, Joerg, Rodríguez‐Pintó, Ignasi, Atzeni, Fabiola, Spierings, Julia, Truchetet, Marie‐Elise, Milchert, Marcin, Brito de Araujo, Daniel, Riemekasten, Gabriela, Bernardino, Vera, Martin, Thierry, del Galdo, Francesco, Vacca, Alessandra, Mendoza, Fabian, Midtvedt, Øyvind, Murdaca, Giuseppe, Santiago, Tânia, Codullo, Veronica, Cacciapaglia, Fabio, Walker, Ulrich, Brunborg, Cathrine, Tirelli, Francesca, Allanore, Yannick, Furst, Daniel E., Matucci, Marco, Gabrielli, Armando, Distler, Oliver, and Hoffmann‐Vold, Anna‐Maria

Published
2024
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22. Proposed Response Parameters for Twelve‐Month Drug Trial in Juvenile Systemic Sclerosis: Results of the Hamburg International Consensus Meetings

Author

Foeldvari, Ivan, Torok, Kathryn S., Anton, Jordi, Blakley, Michael, Constantin, Tamás, Curran, Megan, Cutolo, Maurizio, Denton, Christopher, Fligelstone, Kim, Ingegnoli, Francesca, Li, Suzanne C., Němcová, Dana, Orteu, Catherine, Pilkington, Clarissa, Smith, Vanessa, Stevens, Anne, Klotsche, Jens, Khanna, Dinesh, Costa‐Reis, Patrícia, Del Galdo, Francesco, Hinrichs, Bernd, Kasapcopur, Ozgur, Pain, Clare, Ruperto, Nicolino, Zheng, Alison, and Furst, Daniel E.

Published
2023
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24. Immunology of pregnancy and reproductive health in autoimmune rheumatic diseases. Update from the 11th International Conference on Reproduction, Pregnancy and Rheumatic Diseases

Author

Andreoli, Laura, Chighizola, Cecilia B., Iaccarino, Luca, Botta, Angela, Gerosa, Maria, Ramoni, Véronique, Tani, Chiara, Bermas, Bonnie, Brucato, Antonio, Buyon, Jill, Cetin, Irene, Chambers, Christina D., Clowse, Megan E.B., Costedoat-Chalumeau, Nathalie, Cutolo, Maurizio, De Carolis, Sara, Dolhain, Radboud, Fazzi, Elisa M., Förger, Frauke, Giles, Ian, Haase, Isabell, Khamashta, Munther, Levy, Roger A., Meroni, Pier Luigi, Mosca, Marta, Nelson-Piercy, Catherine, Raio, Luigi, Salmon, Jane, Villiger, Peter, Wahren-Herlenius, Marie, Wallenius, Marianne, Zanardini, Cristina, Shoenfeld, Yehuda, and Tincani, Angela

Published
2023
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26. Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis From European Scleroderma Trials and Research

Author

Vacca, Alessandra, Giollo, Alessandro, Balbir-Gurman, Alexandra, Gheorghiu, Ana Maria, Marcoccia, Antonella, Herrick, Ariane, Radic, Mislav, Stamenkovic, Bojana, Anic, Branimir, Granel, Brigitte, Ribi, Camillo, Selmi, Carlo Francesco, Carlos de la Puente, Milano, de Souza Müller, Carolina, Denton, Christopher, Kayser, Cristiane, Tanaseanu, Cristina-Mihaela, Majewski, Dominik, Rimar, Doron, Krasowska, Dorota, Veale, Douglas, Walker, Ulrich, Kerzberg, Eduardo, Rezus, Elena, Zanatta, Elisabetta, Siegert, Elise, De Langhe, Ellen, Oksel, Fahrettin, Ingegnoli, Francesca, Cantatore, Francesco Paolo, Szücs, Gabriela, Cuomo, Giovanna, Seskute, Goda, Litinsky, VilniusIra, Castellví, Ivan, Morovic-Vergles, Jadranka, Sibilia, Jean, Henes, Jörg, Solanki, Kamal, Perdan-Pirkmajer, Katja, Herrmann, Kristine, Saketkoo, Lesley Ann, Stamp, Lisa, Mouthon, Luc, Salvador, Maria João, Pozzi, Maria Rosa, Üprus, Maria, Vanthuyne, Marie, Engelhart, Merete, Köhm, Michaela, Iudici, Michele, Inanc, Murat, Fathi, Nihal, Pamuk, Nuri, García de la Peña Lefebv, Paloma, Carreira, Patricia E., Bancel, Dominique Farge, Moroncini, Luca, Montecucco, C., Ancuta, Codrina, Sunderkötter, Cord, Müller-Ladner, Ulf, Rosato, Edoardo, Kucharz, Eugene J., Iannone, Florenzo, Del Galdo, Francesco, Poormoghim, Hadi, Kötter, Ina, Distler, Jörg, Cutolo, Maurizio, Tikly, Mohammed, Damjanov, Nemanja, Hunzelmann, Nicolas, Vlachoyiannopoulos, P., Hasler, Paul, Sarzi Puttini, Piercarlo, Wiland, Piotr, Becvar, Radim, Yavuz, Sule, Zdrojewski, Zbigniew, Pellerito, Raffaele, Foti, Rosario, Ionescu, Ruxandra Maria, Adler, Sabine, Kahl, Sarah, Moiseev, Sergey, Stebbings, Simon, Rednic, Simona, Negrini, Simone, Heitmann, Stefan, Ullman, Susanne, Agachi, Svetlana, Martin, Thierry, Schmeiser, Tim, Riccieri, Valeria, Smith, Vanessa, Bernardino, Vera, Ortiz-Santamaria, Vera, Hsu, Vivien M., Abdel Atty Mohamed, Walid Ahmed, Hoffmann-Vold, Anna-Maria, Brunborg, Cathrine, Airò, Paolo, Ananyeva, Lidia P., Czirják, László, Guiducci, Serena, Hachulla, Eric, Li, Mengtao, Mihai, Carina, Riemekasten, Gabriela, Sfikakis, Petros P., Valentini, Gabriele, Kowal-Bielecka, Otylia, Allanore, Yannick, and Distler, Oliver

Published
2023
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27. Treatment with COLchicine in hospitalized patients affected by COVID-19: The COLVID-19 trial

Author

Cafaro, Giacomo, Mendeni, Monia, Colombo, Enrico, Medico, Marta Del, Cabras, Paola, Schiesaro, Mauro Giovanni, Franco, Laura, Fantoni, Massimo, Friso, Lara, Gallo, Valter, Franceschini, Franco, Paolino, Sabrina, Salaffi, Fausto, Scirè, Carlo, Zanetti, Anna, Diana, Claudia, Passaro, Angelina, Foti, Rosario, Serino, Francesco Saverio, Cassol, Maurizio, Bucaneve, Giampaolo, Rocchi, Rosalba Elisabetta, Perricone, Carlo, Scarsi, Mirko, Brucato, Antonio, Pisano, Paola, Pigatto, Erika, Becattini, Cecilia, Cingolani, Antonella, Tiso, Francesco, Prota, Roberto, Tomasoni, Lina Rachele, Cutolo, Maurizio, Tardella, Marika, Rozza, Davide, Zerbino, Carlo, Andreoni, Massimo, Poletti, Venerino, Bartoloni, Elena, and Gerli, Roberto

Published
2023
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28. Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort

Author

Matucci Cerinic, Marco, Walker, Ulrich, Iannone, Florenzo, Jordan, Suzana, Becvar, Radim, Kowal Bielecka, Otylia, Cutolo, Maurizio, Cuomo, Giovanna, Kedor, Claudia, Rednic, Simona, Avouac, Jérome, Vlachoyiannopoulos, P., Montecucco, C., Stork, Jiri, Inanc, Murat, Carreira, Patricia E., Novak, Srdan, Czirják, László, Iudici, Michele, Kucharz, Eugene J., Zanatta, Elisabetta, Perdan-Pirkmajer, Katja, Coleiro, Bernard, Moroncini, Gianluca, Farge Bancel, Dominique, Airò, Paolo, Hesselstrand, Roger, Radic, Mislav, Braun-Moscovici, Yolanda, Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Giollo, Alessandro, Morovic-Vergles, Jadranka, Denton, Christopher, Vonk, Madelon, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Hasler, Paul, Kohm, Michaela, Foeldvari, Ivan, Bajocchi, Gianluigi, Salvador, Maria João, Stamenkovic, Bojana, Selmi, Carlo Francesco, Tikly, Mohammed, Ananieva, Lidia P., Herrick, Ariane, Müller-Ladner, Ulf, De Palma, Raffaele, Engelhart, Merete, Szücs, Gabriela, Sobrino Grande, Cristina, Midtvedt, Øyvind, Launay, David, Riccieri, Valeria, Ionescu, Ruxandra Maria, Sha, Ami, Gheorghiu, Ana Maria, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Cantatore, Francesco Paolo, Ullman, Susanne, Alberto von Mühlen, Carlos, Pozzi, Maria Rosa, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Alegre-Sancho, Juan Jose, Herrmann, Kristine, De Langhe, Ellen, Anic, Branimir, Üprus, Maria, Yavuz, Sule, Granel, Brigitte, de Souza Müller, Carolina, Busquets, Joanna, Agachi, Svetlana, Stebbings, Simon, Mathieu, D'Alessandro, Sampaio-Barros, Percival D., Stamp, Lisa, Solanki, Kamal, Veale, Douglas, Loyo, Esthela, Li, Mengtao, Abdel Atty Mohamed, Walid Ahmed, Gigante, Antonietta, Oksel, Fahrettin, Tanaseanu, Cristina-Mihaela, Foti, Rosario, Ancuta, Codrina, Maurer, Britta, van Laar, Jacob, Kayser, Cristiane, Fathi, Nihal, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Abignano, Giuseppina, Seskute, Goda, Saketkoo, Lesley Ann, Kerzberg, Eduardo, Bianchi, Washington, Castellví, Ivan, Limonta, Massimiliano, Rimar, Doron, Couto, Maura, Spertini, François, Marcoccia, Antonella, Kahl, Sarah, Hsu, Ivien M., Martin, Thierry, Moiseev, Sergey, Chung, Lorinda S., Schmeiser, Tim, Majewski, Dominik, Zdrojewski, Zbigniew, Martínez-Barrio, Julia, Bernardino, Vera, Sommerlatte, Sabine, Levy, Yair, Rezus, Elena, Nuri Pamuk, Omer, Sarzi Puttini, Piercarlo, Poormoghim, Hadi, Kötter, Ina, Gaches, Francis, Belloli, Laura, Sfikakis, Petros, Markus, Juliana, Feldman, Gary R, Ramazan, Ana-Maria, Scherer, H.U., Truchetet, Marie-Elise, Lescoat, Alain, Dagna, Lorenzo, van Laar, J.M., Rudnicka, Lidia, Oliveira, Susana, Atzeni, Fabiola, Kuwana, Masataka, Mekinian, Arsene, Martin, Mickaël, Tanaka, Yoshiya, Elhai, Muriel, Sritharan, Nanthara, Boubaya, Marouane, Balbir-Gurman, Alexandra, Siegert, Elise, Hachulla, Eric, de Vries-Bouwstra, Jeska, Riemekasten, Gabriela, Distler, Jörg H W, Rosato, Edoardo, Del Galdo, Francesco, Mendoza, Fabian A, Furst, Daniel E, de la Puente, Carlos, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Distler, Oliver, Bloch-Queyrat, Coralie, and Allanore, Yannick

Published
2022
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32. Prevalence of hybrid TLR4+M2 monocytes/macrophages in peripheral blood and lung of systemic sclerosis patients with interstitial lung disease.

Author

Gotelli, Emanuele, Soldano, Stefano, Feghali-Bostwick, Carol, Montagna, Paola, Campitiello, Rosanna, Contini, Paola, Mora, Marco, Benelli, Roberto, Hysa, Elvis, Paolino, Sabrina, Pizzorni, Carmen, Sulli, Alberto, Smith, Vanessa, and Cutolo, Maurizio

Subjects
PULMONARY fibrosis, CONNECTIVE tissue diseases, ALVEOLAR macrophages, INTERSTITIAL lung diseases, SYSTEMIC scleroderma
Abstract

Introduction: Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease characterized by microvascular damage, immune system reactivity and progressive fibrosis of skin and internal organs. Interstitial lung disease is the leading cause of death for SSc patients (SSc-ILD), and the process of lung fibrosis involves also circulating monocytes and alveolar macrophages. Methods: Current study aimed to identify monocyte/macrophage phenotypes in lung and peripheral blood of SSc-ILD patients by immunostaining and flow cytometry, respectively. Single immunostaining was performed using primary antibodies against CD68 (pan-macrophage marker), CD80, CD86, TLR4 (M1 markers), CD163, CD204, and CD206 (M2 markers). Flow cytometry analysis included the evaluation of CD45, CD14, CD16 (monocyte lineage), CD1c (dendritic lineage), together with M1 and M2 activation markers on circulating monocytes. Protein synthesis of TLR4 and M2 markers was also investigated in cultured monocytes-derived macrophages (MDMs) from SSc-ILD patients by Western Blotting. Results: Lung samples were obtained from 9 SSc-ILD patients (50 ± 9 years old) and 5 control non-SSc patients without lung fibrosis (58 ± 23 years old). Alveolar macrophages (CD68+ cells) showed a significantly higher positivity of M1 and M2 markers in SSc-ILD lung samples than in controls (p<0.05 for CD80, p<0.01 for CD86, p<0.001 for CD68, p<0.0001 for TLR4, CD163, CD204 and CD206). In CD68 positive areas of SSc-ILD samples, a significantly higher percentage of TLR4, CD163, CD204, and CD206 positive cells was observed compared to CD80 and CD86 positive cells (p<0.001 in both cases), suggesting the possible presence of hybrid TLR4+M2 macrophages (CD68+CD80-CD86-TLR4+CD163+CD204+CD206+cells) in SSc-ILD samples. A second cohort of 26 SSc-ILD patients (63 ± 14 years old) and 14 SSc patients without ILD (63 ± 19 years old) was recruited for flow cytometry analysis of circulating monocytes. Again, a significantly higher percentage of hybrid TLR4+M2 monocytes (CD1c-CD80-TLR4+CD163+CD204+CD206+cells) was found in SSc-ILD positive than SSc-ILD negative patients (p<0.05). Moreover, the protein synthesis of TLR4 and M2 markers was also found higher in cultured MDMs obtained from SSc-ILD patients than in MDMs from SSc patients without ILD and this increase was significantly higher for CD163 (p<0.05) and CD206 (p<0.01). Conclusions: The presence of hybrid TLR4+M2 markers on both circulating monocytes and resident lung macrophages in SSc-ILD patients, is reported for the first time. Therefore, the detection of circulating hybrid TLR4+M2 monocytes in SSc-ILD might represent a further potential biomarker of progressive organ fibrosis, to be searched in blood samples of SSc patients. [ABSTRACT FROM AUTHOR]

Published
2024
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34. Long-term Outcome of Children Born to Women with Autoimmune Rheumatic Diseases: A Multicentre, Nationwide Study on 299 Randomly Selected Individuals

Author

Andreoli, Laura, Nalli, Cecilia, Lazzaroni, Maria Grazia, Carini, Chiara, Dall’Ara, Francesca, Reggia, Rossella, Rodrigues, Marília, Benigno, Carolina, Baldissera, Elena, Bartoloni, Elena, Basta, Fabio, Bellisai, Francesca, Bortoluzzi, Alessandra, Campochiaro, Corrado, Cantatore, Francesco Paolo, Caporali, Roberto, Ceribelli, Angela, Chighizola, Cecilia B., Conigliaro, Paola, Corrado, Addolorata, Cutolo, Maurizio, D’Angelo, Salvatore, De Stefani, Elena, Doria, Andrea, Favaro, Maria, Fischetti, Colomba, Foti, Rosario, Gabrielli, Armando, Generali, Elena, Gerli, Roberto, Gerosa, Maria, Larosa, Maddalena, Maier, Armin, Malavolta, Nazzarena, Meroni, Marianna, Meroni, Pier Luigi, Montecucco, Carlomaurizio, Mosca, Marta, Padovan, Melissa, Paolazzi, Giuseppe, Pazzola, Giulia, Peccatori, Susanna, Perricone, Roberto, Pettiti, Giorgio, Picerno, Valentina, Prevete, Immacolata, Ramoni, Véronique, Romeo, Nicoletta, Ruffatti, Amelia, Salvarani, Carlo, Sebastiani, Gian Domenico, Selmi, Carlo, Serale, Francesca, Sinigaglia, Luigi, Tani, Chiara, Trevisani, Marica, Vadacca, Marta, Valentini, Eleonora, Valesini, Guido, Visalli, Elisa, Vivaldelli, Ester, Zuliani, Lucia, and Tincani, Angela

Published
2022
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38. Progression of patients with Raynaud's phenomenon to systemic sclerosis: a five-year analysis of the European Scleroderma Trial and Research group multicentre, longitudinal registry study for Very Early Diagnosis of Systemic Sclerosis (VEDOSS)

Author

Bellando-Randone, Silvia, Del Galdo, Francesco, Lepri, Gemma, Minier, Tunde, Huscher, Dörte, Furst, Daniel E, Allanore, Yannick, Distler, Oliver, Czirják, László, Bruni, Cosimo, Guiducci, Serena, Avouac, Jerome, Cutolo, Maurizio, Smith, Vanessa, and Matucci-Cerinic, Marco

Published
2021
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42. Updating on pregnancy in rheumatoid arthritis.

Author

Schenone, Carlotta, Pacini, Greta, Gotelli, Emanuele, Hysa, Elvis, Campitiello, Rosanna, Sammorì, Silvia, Paolino, Sabrina, Sulli, Alberto, and Cutolo, Maurizio

Subjects
REPRODUCTIVE technology, PREGNANCY complications, PREGNANCY outcomes, RHEUMATOID arthritis, SYNTHETIC drugs
Abstract

Rheumatoid arthritis (RA), the most prevalent autoimmune disease in reproductive years, exhibits a higher incidence in females, suggesting involvement of estrogens, genetics and environmental factors in disease onset. Literature shows smaller families in RA patients, driving increased interest in Assisted Reproductive Techniques. This review elucidates how immunotolerance mechanisms contribute to favorable pregnancy outcomes in RA, emphasizing the need for a careful pregnancy planning to mitigate fetal complications and postnatal flares, which surpass those in the general population. A thorough medication evaluation, orchestrated by a multidisciplinary team, is imperative during pregnancy, weighing potential teratogenic effects against safer alternatives to balance medication safety with disease control. A systematic literature search on PubMed and MEDLINE, using specific terms, covered relevant academic journals up to the latest date. This narrative review comprehensively addresses pregnancy-related considerations in RA patients, prioritizing meticulous disease management with pregnancy and breastfeeding-compatible drugs in line with the latest recommendations and registry data. The focus remains on evaluating glucocorticoids, conventional, and biological disease-modifying drugs for compatibility during pregnancy and breastfeeding. Additionally, the evolving landscape of targeted synthetic drugs during pregnancy is explored, providing insights into the latest developments in rheumatological care. [ABSTRACT FROM AUTHOR]

Published
2024
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46. Standardisation of nailfold capillaroscopy for the assessment of patients with Raynaud's phenomenon and systemic sclerosis

Author

Smith, Vanessa, Herrick, Ariane L., Ingegnoli, Francesca, Damjanov, Nemanja, De Angelis, Rossella, Denton, Christopher P., Distler, Oliver, Espejo, Karinna, Foeldvari, Ivan, Frech, Tracy, Garro, Boris, Gutierrez, Marwin, Gyger, Genevieve, Hachulla, Eric, Hesselstrand, Roger, Iagnocco, Annamaria, Kayser, Cristiane, Melsens, Karin, Müller-Ladner, Ulf, Paolino, Sabrina, Pizzorni, Carmen, Radic, Mislav, Riccieri, Valeria, Snow, Marcus, Stevens, Wendy, Sulli, Alberto, van Laar, Jacob M., Vonk, Madelon C., Vanhaecke, Amber, and Cutolo, Maurizio

Published
2020
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49. The impact of COVID-19 on rare and complex connective tissue diseases: the experience of ERN ReCONNET

Author

Talarico, Rosaria, Aguilera, Silvia, Alexander, Tobias, Amoura, Zahir, Antunes, Ana M., Arnaud, Laurent, Avcin, Tadej, Beretta, Lorenzo, Bombardieri, Stefano, Burmester, Gerd R., Cannizzo, Sara, Cavagna, Lorenzo, Chaigne, Benjamin, Cornet, Alain, Costedoat-Chalumeau, Nathalie, Doria, Andrea, Ferraris, Alessandro, Fischer-Betz, Rebecca, Fonseca, João E., Frank, Charissa, Gaglioti, Andrea, Galetti, Ilaria, Grunert, Jürgen, Guimarães, Vera, Hachulla, Eric, Houssiau, Frederic, Iaccarino, Luca, Krieg, Thomas, Limper, Marteen, Malfait, Fransiska, Mariette, Xavier, Marinello, Diana, Martin, Thierry, Matthews, Lisa, Matucci-Cerinic, Marco, Meyer, Alain, Montecucco, Carlomaurizio, Mouthon, Luc, Müller-Ladner, Ulf, Rednic, Simona, Romão, Vasco C., Schneider, Matthias, Smith, Vanessa, Sulli, Alberto, Tamirou, Farah, Taruscio, Domenica, Taulaigo, Anna V., Terol, Enrique, Tincani, Angela, Ticciati, Simone, Turchetti, Giuseppe, van Hagen, P. Martin, van Laar, Jacob M., Vieira, Ana, de Vries-Bouwstra, Jeska K., Cutolo, Maurizio, and Mosca, Marta

Published
2021
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50. Defining Interstitial Lung Disease Progression: It Is Time to (Get Our) Act Together.

Author

Smith, Vanessa, Cutolo, Maurizio, and Volkmann, Elizabeth

Subjects
RAYNAUD'S disease, INTERSTITIAL lung diseases, PULMONARY arterial hypertension, DELAYED diagnosis, PULMONARY function tests, SCLERODERMA (Disease)
Abstract

The editorial discusses the challenges in defining and monitoring the progression of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). The study evaluated four different definitions of SSc-ILD progression and found that they were not interchangeable, leading to confusion in clinical practice and research. The findings suggest the need for standardized definitions to guide treatment decisions and improve outcomes for patients with SSc-ILD. The study highlights the importance of further research and initiatives like the European Reference Networks to address the complexities of rare diseases such as SSc-ILD. [Extracted from the article]

Published
2024
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