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108. Preliminary criteria for the very early diagnosis of systemic sclerosis: Results of a Delphi consensus study from EULAR scleroderma trials and research group

Author

UCL - (MGD) Service de rhumatologie, Avouac, J., Fransen, J., Walker, U.A., Riccieri, V., Smith, V., Muller, C., Miniati, I., Tarner, I.H., Bellando Randone, S., Cutolo, M., Allanore, Y., Distler, O., Valentini, G., Czirjak, L., Müller-Ladner, U., Furst, D.E., Tyndall, A., Matucci-Cerinic, M., De Keyser, F., Sulli, A., Pizzorni, C., Maurer, B., Sierakowsky, S., Kowal-Bielecka, O., Coelho, P., Riemekasten, G., Rednic, S., Nicoara, I., Caporali, R., Štork, J., Inanc, M., Carreira, P., Novak, S., Varju, C., Chizzolini, C., Ribi, C., Kucharz, E.J., Kotulska, A., Widuchowska, M., Richter, J., Sipek-Dolnicar, A., Rozman, B., Gabrielli, A., Moroncini, G., Farge, D., Durant, C., Kiener, H.P., Rath, E., Airo, P., Wollheim, F.A., Hunzelmann, N., Bombardieri, S., Della Rossa, A., Bazzichi, L., Pellerito, R., Saracco, M., Denton, C., Vonk, M., Van Den Hoogen, F., Damjanov, N., Kötter, I., Heitmann, S., Seidel, M., Hasler, P., Van Laar, J.M., Salvador, M.J., Pereira Da Silva, J.A., Jacobsen, S., Worm, M., Kuhn, A., Nevskaya, T., Nasonov, E.L., Scorza, R., Nielsen, H., Silver, R.M., Hachulla, E., Launay, D., Valesini, G., Ionescu, R., Opris, D., Del Papa, N., Maglione, W., Comina, D., Udrea, G., Ciurtin, C., Ionitescu, R., Mihai, C., Sunderkötter, C., Jun, J.-B., Derk, C., Alhasani, S., Alhajjar, L., Ton, E., Seibold, J., Nash, P., Mouthon, L., Von Mühlen, C.A., Krummel-Lorenz, B., Eilbacher, P., Westhovens, R., De Langhe, E., Mayer, M., Anic, B., Baresic, M., Stoeckl, F., Üprus, M., Popa, S., Buslau, M., Granel, B., Zenone, T., Mathieu, A., Vacca, A., Amerio, P., Tourinho, T., Lonzetti, L., Lemos Lopes, M., De Souza, R., Vealex, D., Caramaschi, P., Balbir-Gurman, A., Braun, Y., Ullman, S., Szmyrka-Kaczmarek, M., Morgiel, E., Vanthuyne, Marie, Meurer, M., Rehberger, P., Müller, C., Sampaio-Barros, P., UCL - (MGD) Service de rhumatologie, Avouac, J., Fransen, J., Walker, U.A., Riccieri, V., Smith, V., Muller, C., Miniati, I., Tarner, I.H., Bellando Randone, S., Cutolo, M., Allanore, Y., Distler, O., Valentini, G., Czirjak, L., Müller-Ladner, U., Furst, D.E., Tyndall, A., Matucci-Cerinic, M., De Keyser, F., Sulli, A., Pizzorni, C., Maurer, B., Sierakowsky, S., Kowal-Bielecka, O., Coelho, P., Riemekasten, G., Rednic, S., Nicoara, I., Caporali, R., Štork, J., Inanc, M., Carreira, P., Novak, S., Varju, C., Chizzolini, C., Ribi, C., Kucharz, E.J., Kotulska, A., Widuchowska, M., Richter, J., Sipek-Dolnicar, A., Rozman, B., Gabrielli, A., Moroncini, G., Farge, D., Durant, C., Kiener, H.P., Rath, E., Airo, P., Wollheim, F.A., Hunzelmann, N., Bombardieri, S., Della Rossa, A., Bazzichi, L., Pellerito, R., Saracco, M., Denton, C., Vonk, M., Van Den Hoogen, F., Damjanov, N., Kötter, I., Heitmann, S., Seidel, M., Hasler, P., Van Laar, J.M., Salvador, M.J., Pereira Da Silva, J.A., Jacobsen, S., Worm, M., Kuhn, A., Nevskaya, T., Nasonov, E.L., Scorza, R., Nielsen, H., Silver, R.M., Hachulla, E., Launay, D., Valesini, G., Ionescu, R., Opris, D., Del Papa, N., Maglione, W., Comina, D., Udrea, G., Ciurtin, C., Ionitescu, R., Mihai, C., Sunderkötter, C., Jun, J.-B., Derk, C., Alhasani, S., Alhajjar, L., Ton, E., Seibold, J., Nash, P., Mouthon, L., Von Mühlen, C.A., Krummel-Lorenz, B., Eilbacher, P., Westhovens, R., De Langhe, E., Mayer, M., Anic, B., Baresic, M., Stoeckl, F., Üprus, M., Popa, S., Buslau, M., Granel, B., Zenone, T., Mathieu, A., Vacca, A., Amerio, P., Tourinho, T., Lonzetti, L., Lemos Lopes, M., De Souza, R., Vealex, D., Caramaschi, P., Balbir-Gurman, A., Braun, Y., Ullman, S., Szmyrka-Kaczmarek, M., Morgiel, E., Vanthuyne, Marie, Meurer, M., Rehberger, P., Müller, C., and Sampaio-Barros, P.

Abstract

Objective: To identify a core set of preliminary items considered as important for the very early diagnosis of systemic sclerosis (SSc). Methods: A list of items provided by European League Against Rheumatism (EULAR) Scleroderma Trial and Research(EUSTAR) centres were subjected to a Delphi exercise among 110 experts in the field of SSc. In round 1, experts were asked to choose the items they considered as the most important for the very early diagnosis of SSc. In round 2, experts were asked to reconsider the items accepted after the first stage. In round 3, the clinical relevance of selected items and their importance as measures that would lead to an early referral process were rated using appropriateness scores. Results: Physicians from 85 EUSTAR centres participated in the study and provided an initial list of 121 items. After three Delphi rounds, the steering committee, with input from external experts, collapsed the 121 items into three domains containing seven items, developed as follows: skin domain (puffy fingers/puffy swollen digits turning into sclerodactily);vascular domain (Raynaud's phenomenon, abnormal capillaroscopy with scleroderma pattern) and laboratory domain (antinuclear, anticentromere and antitopoisomerase-I antibodies). Finally, the whole assembly of EUSTAR centres ratified with a majority vote the results in a final face-to-face meeting. Conclusion: The three Delphi rounds allowed us to identify the items considered by experts as necessary for the very early diagnosis of SSc. The validation of these items to establish diagnostic criteria is currently ongoing in a prospective observational cohort.

Published
2011

109. Agonistic anti-human Fas monoclonal antibody induces fibroblast-like synoviocyte apoptosis in haemophilic arthropathy: potential therapeutic implications

Author

Romano, E., primary, Manetti, M., additional, Peruzzi, F., additional, Melchiorre, D., additional, Milia, A. F., additional, Bellando-Randone, S., additional, Nishioka, K., additional, Innocenti, M., additional, Carulli, C., additional, Linari, S., additional, Morfini, M., additional, Ibba-Manneschi, L., additional, Matucci-Cerinic, M., additional, and Guiducci, S., additional

Published
2013
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117. S.9.1 Lung ultrasound for the screening of interstitial lung disease in SSc

Author

Barskova, T., primary, Gargani, L., additional, Conforti, M. L., additional, Guiducci, S., additional, Bruni, C., additional, Moggi Pignone, A., additional, Picano, E., additional, Matucci Cerinic, M., additional, Doveri, M., additional, Agoston, G., additional, Moreo, A., additional, Musca, F., additional, Bruschi, E., additional, Epis, O., additional, Bellando Randone, S., additional, Bazzichi, L., additional, Bombardieri, S., additional, Varga, A., additional, Sicari, R., additional, Akbayrak, E., additional, Tarner, I. H., additional, Muller-Ladner, U., additional, Dinser, R., additional, Stamenkovic, B., additional, Stankovic, A., additional, Dimic, A., additional, Damjanov, N., additional, Nedovic, J., additional, Menkovic, D., additional, Stojanovic, S., additional, and Milenkovic, S., additional

Published
2012
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118. Bone marrow-derived mesenchymal stem cells from early diffuse systemic sclerosis exhibit a paracrine machinery and stimulate angiogenesis in vitro

Author

Guiducci, S., primary, Manetti, M., additional, Romano, E., additional, Mazzanti, B., additional, Ceccarelli, C., additional, Dal Pozzo, S., additional, Milia, A. F., additional, Bellando-Randone, S., additional, Fiori, G., additional, Conforti, M. L., additional, Saccardi, R., additional, Ibba-Manneschi, L., additional, and Matucci-Cerinic, M., additional

Published
2011
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119. Autologous mesenchymal stem cells foster revascularization of ischemic limbs in systemic sclerosis: a case report.

Author

Guiducci S, Porta F, Saccardi R, Guidi S, Ibba-Manneschi L, Manetti M, Mazzanti B, Dal Pozzo S, Milia AF, Bellando-Randone S, Miniati I, Fiori G, Fontana R, Amanzi L, Braschi F, Bosi A, and Matucci-Cerinic M

Abstract

Background: Mesenchymal stem cells can differentiate into endothelial cells and participate in angiogenesis in adults. In experimental models of acute myocardial infarction, mesenchymal stem cells led to the recovery of cardiac function through the formation of a new vascular network. Objective: To describe treatment with intravenous infusions of expanded autologous mesenchymal stem cells in 1 patient with critical limb ischemia due to systemic sclerosis. Design: Case report. Setting: The rheumatology unit at the University of Florence, Florence, Italy. Patient: A woman, aged 34 years, with systemic sclerosis who developed acute gangrene of the upper and lower limbs. Intervention: 3 intravenous pulses of expanded autologous mesenchymal stem cells. Measurements: Angiography, skin histopathology, and immunohistochemistry. Results: Areas of necrotic skin were reduced after the first mesenchymal stem-cell infusion. After the third infusion, angiography showed revascularization of the patient's extremities. Skin section analysis revealed cell clusters with tubelike structures, and angiogenic factors were strongly expressed. Limitation: Causality cannot be established by a single case. Conclusion: In patients with systemic sclerosis who have severe peripheral ischemia, intravenous infusion of expanded autologous mesenchymal stem cells may foster the recovery of the vascular network, restore blood flow, and reduce skin necrosis. Primary Funding Source: Fondazione Cassa di Risparmio di Pistoia e Pescia (partial funding). [ABSTRACT FROM AUTHOR]

Published
2010
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121. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology

Author

Rossella, De Angelis, Dilia, Giuggioli, Gianluigi, Bajocchi, Lorenzo, Dagna, Giovanni, Zanframundo, Rosario, Foti, Fabio, Cacciapaglia, Giovanna, Cuomo, Alarico, Ariani, Edoardo, Rosato, Serena, Guiducci, Francesco, Girelli, Valeria, Riccieri, Elisabetta, Zanatta, Silvia, Bosello, Ilaria, Cavazzana, Francesca, Ingegnoli, Maria De, Santis, Giuseppe, Murdaca, Giuseppina, Abignano, Nicoletta, Romeo, Alessandra, Della Rossa, Maurizio, Caminiti, Annamaria, Iuliano, Giovanni, Ciano, Lorenzo, Beretta, Gianluca, Bagnato, Ennio, Lubrano, Ilenia, De Andres, Alessandro, Giollo, Marta, Saracco, Cecilia, Agnes, Federica, Lumetti, Amelia, Spinella, Luca, Magnani, Corrado, Campochiaro, Giacomo, De Luca, Veronica, Codullo, Elisa, Visalli, Francesco, Masini, Antonietta, Gigante, Silvia, Bellando-Randone, Greta, Pellegrino, Erika, Pigatto, Francesca, Dall'Ara, Maria Grazia, Lazzaroni, Elena, Generali, Gianna, Mennillo, Simone, Barsotti, Giuseppa Pagano, Mariano, Francesca, Calabrese, Federica, Furini, Licia, Vultaggio, Simone, Parisi, Clara Lisa, Peroni, Anna Maria, Risa, Davide, Rozza, Anna, Zanetti, Greta, Carrara, Giampiero, Landolfi, Carlo Alberto, Scirè, Gerolamo, Bianchi, Enrico, Fusaro, Gian Domenico, Sebastiani, Marcello, Govoni, Salvatore, D'Angelo, Franco, Cozzi, Andrea, Doria, Florenzo, Iannone, Carlo, Salvarani, Marco, Matucci-Cerinic, Clodoveo, Ferri, De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Dall'Ara, F, Lazzaroni, M, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Risa, A, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri, C, de Angelis, R., Giuggioli, D., Bajocchi, G., Dagna, L., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Guiducci, S., Girelli, F., Riccieri, V., Zanatta, E., Bosello, S., Cavazzana, I., Ingegnoli, F., de Santis, M., Murdaca, G., Abignano, G., Romeo, N., Rossa, A. D., Caminiti, M., Iuliano, A., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., de Andres, I., Giollo, A., Saracco, M., Agnes, C., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., de Luca, Giacomo., Codullo, V., Visalli, E., Masini, F., Gigante, A., Bellando-Randone, S., Pellegrino, G., Pigatto, E., Dall'Ara, F., Lazzaroni, M. G., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Calabrese, F., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Risa, A. M., Rozza, D., Zanetti, A., Carrara, G., Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, E., Sebastiani, G. D., Govoni, M., D'Angelo, S., Cozzi, F., Doria, A., Iannone, F., Salvarani, C., Matucci-Cerinic, M., Ferri, C., De Angelis, Rossella, Giuggioli, Dilia, Bajocchi, Gianluigi, Dagna, Lorenzo, Zanframundo, Giovanni, Foti, Rosario, Cacciapaglia, Fabio, Cuomo, Giovanna, Ariani, Alarico, Rosato, Edoardo, Guiducci, Serena, Girelli, Francesco, Riccieri, Valeria, Zanatta, Elisabetta, Bosello, Silvia, Cavazzana, Ilaria, Ingegnoli, Francesca, De Santis, Maria, Murdaca, Giuseppe, Abignano, Giuseppina, Romeo, Nicoletta, Della Rossa, Alessandra, Caminiti, Maurizio, Iuliano, Annamaria, Ciano, Giovanni, Beretta, Lorenzo, Bagnato, Gianluca, Lubrano, Ennio, De Andres, Ilenia, Giollo, Alessandro, Saracco, Marta, Agnes, Cecilia, Lumetti, Federica, Spinella, Amelia, Magnani, Luca, Campochiaro, Corrado, De Luca, Giacomo, Codullo, Veronica, Visalli, Elisa, Masini, Francesco, Gigante, Antonietta, Bellando-Randone, Silvia, Pellegrino, Greta, Pigatto, Erika, Dall'Ara, Francesca, Lazzaroni, Maria Grazia, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Pagano Mariano, Giuseppa, Calabrese, Francesca, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Risa, Anna Maria, Rozza, Davide, Zanetti, Anna, Carrara, Greta, Landolfi, Giampiero, Scirè, Carlo Alberto, Bianchi, Gerolamo, Fusaro, Enrico, Sebastiani, Gian Domenico, Govoni, Marcello, D'Angelo, Salvatore, Cozzi, Franco, Doria, Andrea, Iannone, Florenzo, Salvarani, Carlo, Matucci-Cerinic, Marco, and Ferri, Clodoveo

Subjects
Male, medicine.medical_specialty, Vital capacity, Settore MED/16 - REUMATOLOGIA, Cross-sectional study, Immunology, Left, Socio-culturale, scleroderma, sex, systemic sclerosis, Disease, Ventricular Function, Left, Scleroderma, Systemic sclerosi, Rheumatology, Internal medicine, Sicca syndrome, Sex, Systemic sclerosis, Cross-Sectional Studies, Female, Humans, Italy, Registries, Sex Characteristics, Stroke Volume, Scleroderma, Systemic, Sjogren's Syndrome, medicine, LS8_2, Immunology and Allergy, Ventricular Function, Honeycombing, skin and connective tissue diseases, Ejection fraction, integumentary system, business.industry, Systemic, medicine.disease, Cohort, business
Abstract

ObjectiveThere is still a great deal to learn about the influence of sex in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying sex differences in disease expression, with a special focus on demographic, clinical, and serological characteristics.MethodsA multicenter SSc cohort of 2281 patients, including 247 men, was recruited in the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry. Demographic data, disease manifestations, serological profile, and internal organ involvement were compared.ResultsThe overall female/male ratio was 8.2:1. Female/male ratios for limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma subsets were 8.7:1, 4.9:1, and 10.7:1, respectively. A shorter time from onset of Raynaud phenomenon to SSc diagnosis, an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, whereas a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein B was detected in the female group. Males exhibited lower left ventricular ejection fraction, as well as higher prevalence of conduction blocks, arrhythmias, ground glass, and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs.ConclusionOur study further supports the presence of several sex-related differences in patients with SSc. These differences were pronounced in the severity of cutaneous, peripheral vascular, and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterized the female sex.

Published
2022

122. The role of chest CT in deciphering interstitial lung involvement: systemic sclerosis versus COVID-19

Author

Jelena Blagojevic, Francesca Wanda Rossi, Alessandro Bartoloni, Cosimo Nardi, S. Tomassetti, Martina Orlandi, Alberto Moggi-Pignone, Yannick Allanore, L. Dagna, Stefano Palmucci, Carlo Vancheri, Marco Matucci-Cerinic, Francesca Della Casa, Marco Confalonieri, Federico Lavorini, Amato de Paulis, Lorenzo Tofani, Gloria Taliani, Virginia Vegni, Dinesh Khanna, Vittorio Miele, Alberto Pesci, Barbara Ruaro, C. Campochiaro, Lorenzo Zammarchi, Giovanni Morana, Michele Spinicci, Gianluca Sambataro, Antonella Caminati, Silvia Bellando-Randone, Daniela Melchiorre, Cosimo Bruni, Nicholas Landini, Francesco De Cobelli, Masataka Kuwana, Giacomo De Luca, Sergio Harari, Stefano Colagrande, Fabio Melchiorre, Edoardo Cavigli, Serena Guiducci, Christopher P. Denton, Fabrizio Luppi, Michael Hughes, Marco Albanesi, Orlandi, Martina, Landini, Nichola, Sambataro, Gianluca, Nardi, Cosimo, Tofani, Lorenzo, Bruni, Cosimo, Bellando-Randone, Silvia, Blagojevic, Jelena, Melchiorre, Daniela, Hughes, Michael, Denton, Christopher P, Luppi, Fabrizio, Ruaro, Barbara, Della Casa, Francesca, Rossi, Francesca W, De Luca, Giacomo, Campochiaro, Corrado, Spinicci, Michele, Zammarchi, Lorenzo, Tomassetti, Sara, Caminati, Antonella, Cavigli, Edoardo, Albanesi, Marco, Melchiorre, Fabio, Palmucci, Stefano, Vegni, Virginia, Guiducci, Serena, Moggi-Pignone, Alberto, Allanore, Yannick, Bartoloni, Alessandro, Confalonieri, Marco, Dagna, Lorenzo, De Cobelli, Francesco, De Paulis, Amato, Harari, Sergio, Khanna, Dinesh, Kuwana, Masataka, Taliani, Gloria, Lavorini, Federico, Miele, Vittorio, Morana, Giovanni, Pesci, Alberto, Vancheri, Carlo, Colagrande, Stefano, Matucci-Cerinic, Marco, Denton, Christopher P., Rossi, Francesca W., Decobelli, Francesco, Depaulis, Amato, Orlandi, M, Landini, N, Sambataro, G, Nardi, C, Tofani, L, Bruni, C, Bellando-Randone, S, Blagojevic, J, Melchiorre, D, Hughes, M, Denton, C, Luppi, F, Ruaro, B, Della Casa, F, Rossi, F, De Luca, G, Campochiaro, C, Spinicci, M, Zammarchi, L, Tomassetti, S, Caminati, A, Cavigli, E, Albanesi, M, Melchiorre, F, Palmucci, S, Vegni, V, Guiducci, S, Moggi-Pignone, A, Allanore, Y, Bartoloni, A, Confalonieri, M, Dagna, L, De Cobelli, F, De Paulis, A, Harari, S, Khanna, D, Kuwana, M, Taliani, G, Lavorini, F, Miele, V, Morana, G, Pesci, A, Vancheri, C, Colagrande, S, Matucci-Cerinic, M, Orlandi, M., Landini, N., Sambataro, G., Nardi, C., Tofani, L., Bruni, C., Bellando-Randone, S., Blagojevic, J., Melchiorre, D., Hughes, M., Denton, C. P., Luppi, F., Ruaro, B., Della Casa, F., Rossi, F. W., De Luca, G., Campochiaro, C., Spinicci, M., Zammarchi, L., Tomassetti, S., Caminati, A., Cavigli, E., Albanesi, M., Melchiorre, F., Palmucci, S., Vegni, V., Guiducci, S., Moggi-Pignone, A., Allanore, Y., Bartoloni, A., Confalonieri, M., Dagna, L., Decobelli, F., de Paulis, A., Harari, S., Khanna, D., Kuwana, M., Taliani, G., Lavorini, F., Miele, V., Morana, G., Pesci, A., Vancheri, C., Colagrande, S., and Matucci-Cerinic, M.

Subjects
medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), Fibrosi, systemic sclerosis, education, Chest ct, Stock options, COVID-19, COVID-19 pneumonia, interstitial lung disease, lung CT scan, Computed tomography, Institutional ethics, COVID-19 Testing, Rheumatology, Fibrosis, Medicine, Humans, Pharmacology (medical), Lung, health care economics and organizations, Scleroderma, Systemic, Competing interests, medicine.diagnostic_test, business.industry, Interstitial lung disease, medicine.disease, Lung involvement, Peripheral, Clinical Practice, Pneumonia, Family medicine, Radiology, Differential diagnosis, business, Lung Diseases, Interstitial, Tomography, X-Ray Computed, systemic sclerosi, Human
Abstract

Background: In clinical practice, the striking similarities observed at computed tomography (CT) between the diseases make it difficult to distinguish a COVID-19 pneumonia from a progression of interstitial lung disease (ILD) secondary to Systemic sclerosis (SSc). The aim of the present study was to identify the main CT features that may help distinguishing SSc-ILD from COVID-19 pneumonia. Methods: This multicentric study included 22 international readers divided in the radiologist group (RAD) and non-radiologist group (nRAD). A total of 99 patients, 52 with COVID-19 and 47 with SSc-ILD, were included in the study. Findings: Fibrosis inside focal ground glass opacities (GGO) in the upper lobes; fibrosis in the lower lobe GGO; reticulations in lower lobes (especially if bilateral and symmetrical or associated with signs of fibrosis) were the CT features most frequently associated with SSc-ILD. The CT features most frequently associated with COVID- 19 pneumonia were: consolidation (CONS) in the lower lobes, CONS with peripheral (both central/peripheral or patchy distributions), anterior and posterior CONS and rounded-shaped GGOs in the lower lobes. After multivariate analysis, the presence of CONS in the lower lobes (p

Published
2022

123. COVID-19 and systemic sclerosis: clinicopathological implications from Italian nationwide survey study

Author

Clodoveo Ferri, Dilia Giuggioli, Vincenzo Raimondo, Lorenzo Dagna, Valeria Riccieri, Elisabetta Zanatta, Serena Guiducci, Antonio Tavoni, Rosario Foti, Giovanna Cuomo, Rossella De Angelis, Franco Cozzi, Giuseppe Murdaca, Ilaria Cavazzana, Nicoletta Romeo, Veronica Codullo, Francesca Ingegnoli, Roberta Pellegrini, Giuseppe Varcasia, Alessandra Della Rossa, Maria De Santis, Giuseppina Abignano, Michele Colaci, Maurizio Caminiti, Massimo L'Andolina, Ennio Lubrano, Amelia Spinella, Federica Lumetti, Giacomo De Luca, Silvia Bellando-Randone, Elisa Visalli, Silvia Bilia, Daiana Giannini, Francesco Masini, Greta Pellegrino, Erika Pigatto, Elena Generali, Francesca Dall'Ara, Giuseppa Pagano Mariano, Simone Barsotti, Giorgio Pettiti, Giovanni Zanframundo, Raffaele Brittelli, Vincenzo Aiello, Daniela Scorpiniti, Tommaso Ferrari, Rodolfo Caminiti, Corrado Campochiaro, Salvatore D'Angelo, Florenzo Iannone, Marco Matucci-Cerinic, Andrea Doria, Mario Miccoli, Poupak Fallahi, Alessandro Antonelli, Riccardo Cecchetti, Pietro Gigliotti, Domenico Olivo, Francesco Ursini, Veronica Brusi, Riccardo Meliconi, Raffaele Scarpa, Enrico Fusaro, Anna Linda Zignego, Sabrina Rosaria Paparo, Francesca Ragusa, Giusy Elia, Silvia Martina Ferrari, Ferri, C., Giuggioli, D., Raimondo, V., Dagna, L., Riccieri, V., Zanatta, E., Guiducci, S., Tavoni, A., Foti, R., Cuomo, G., De Angelis, R., Cozzi, F., Murdaca, G., Cavazzana, I., Romeo, N., Codullo, V., Ingegnoli, F., Pellegrini, R., Varcasia, G., Rossa, A. D., De Santis, M., Abignano, G., Colaci, M., Caminiti, M., L'Andolina, M., Lubrano, E., Spinella, A., Lumetti, F., De Luca, G., Bellando-Randone, S., Visalli, E., Bilia, S., Giannini, D., Masini, F., Pellegrino, G., Pigatto, E., Generali, E., Dall'Ara, F., Mariano, G. P., Barsotti, S., Pettiti, G., Zanframundo, G., Brittelli, R., Aiello, V., Scorpiniti, D., Ferrari, T., Caminiti, R., Campochiaro, C., D'Angelo, S., Iannone, F., Matucci-Cerinic, M., Doria, A., Miccoli, M., Fallahi, P., Antonelli, A., Della Rossa, A., Pagano Mariano, G., Cecchetti, R., Gigliotti, P., Olivo, D., Ursini, F., Brusi, V., Meliconi, R., Scarpa, R., Fusaro, E., Zignego, A. L., Paparo, S. R., Ragusa, F., Elia, G., Ferrari, S. M., Ferri C., Giuggioli D., Raimondo V., Dagna L., Riccieri V., Zanatta E., Guiducci S., Tavoni A., Foti R., Cuomo G., De Angelis R., Cozzi F., Murdaca G., Cavazzana I., Romeo N., Codullo V., Ingegnoli F., Pellegrini R., Varcasia G., Rossa A.D., De Santis M., Abignano G., Colaci M., Caminiti M., L'Andolina M., Lubrano E., Spinella A., Lumetti F., De Luca G., Bellando-Randone S., Visalli E., Bilia S., Giannini D., Masini F., Pellegrino G., Pigatto E., Generali E., Dall'Ara F., Mariano G.P., Barsotti S., Pettiti G., Zanframundo G., Brittelli R., Aiello V., Scorpiniti D., Ferrari T., Caminiti R., Campochiaro C., D'Angelo S., Iannone F., Matucci-Cerinic M., Doria A., Miccoli M., Fallahi P., Antonelli A., Della Rossa A., Pagano Mariano G., Cecchetti R., Gigliotti P., Olivo D., Ursini F., Brusi V., Meliconi R., Scarpa R., Fusaro E., Zignego A.L., Paparo S.R., Ragusa F., Elia G., Ferrari S.M., and De Luca, Giacomo.

Subjects
medicine.medical_specialty, 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), business.industry, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Immunology, MEDLINE, COVID-19, Nationwide survey, Rheumatology, Family medicine, Systemic sclerosis, Immunology and Allergy, Medicine, business
Abstract

None

Published
2021

124. Systemic sclerosis and primary biliary cholangitis: Longitudinal data to determine the outcomes

Author

Gemma Lepri, Paolo Airò, Oliver Distler, Kristofer Andréasson, Yolanda Braun-Moscovici, Eric Hachulla, Alexandra Balbir-Gurman, Ellen De Langhe, Simona Rednic, Francesca Ingegnoli, Edoardo Rosato, Laura Groseanu, Ruxandra Ionescu, Silvia Bellando-Randone, Liudmila Garzanova, Lorenzo Beretta, Chiara Bellocchi, Sergey Moiseev, Pavel Novikov, Iulia Szabo, Dorota Krasowska, Veronica Codullo, Ulrich A. Walker, Chrysoula Manolaraki, Serena Guiducci, Marie-Elise Truchetet, Florenzo Iannone, Lorenzo Tofani, Cosimo Bruni, Vanessa Smith, Giovanna Cuomo, Martin Krusche, Marco Matucci-Cerinic, Yannick Allanore, Lepri, G., Airo, P., Distler, O., Andreasson, K., Braun-Moscovici, Y., Hachulla, E., Balbir-Gurman, A., De Langhe, E., Rednic, S., Ingegnoli, F., Rosato, E., Groseanu, L., Ionescu, R., Bellando-Randone, S., Garzanova, L., Beretta, L., Bellocchi, C., Moiseev, S., Novikov, P., Szabo, I., Krasowska, D., Codullo, V., Walker, U. A., Manolaraki, C., Guiducci, S., Truchetet, M. -E., Iannone, F., Tofani, L., Bruni, C., Smith, V., Cuomo, G., Krusche, M., Matucci-Cerinic, M., and Allanore, Y.

Subjects
fibrotic disease, Rheumatology, autoimmunity, Immunology, outcome, Systemic sclerosis, Immunology and Allergy, overlap syndrome, primary biliary cholangiti
Abstract

Background: Several studies described the cross-sectional characteristics of systemic sclerosis patients and coexisting primary biliary cholangitis, but longitudinal prognostic data are lacking. Aims: To describe the systemic sclerosis–primary biliary cholangitis phenotype, including baseline characteristics and outcomes. Methods: We performed a multicentre the European Scleroderma Trials and Research Group study of systemic sclerosis patients with primary biliary cholangitis or with primary biliary cholangitis–specific antibodies, matched with systemic sclerosis controls free from hepatobiliary involvement matched for disease duration and cutaneous subset. Data were recorded at baseline and at the last available visit. Results: A total of 261 patients were enrolled (115 primary biliary cholangitis–systemic sclerosis, 161 systemic sclerosis). At baseline, systemic sclerosis–primary biliary cholangitis patients had a higher prevalence of anti-centromere antibodies ( p = 0.0023) and a lower prevalence of complete absence of digital ulcers. The milder vascular involvement was confirmed at follow-up when crucial differences emerged in the percentage of patients experiencing digital ulcers; a significantly higher number of patients who never experienced digital ulcers were observed among primary biliary cholangitis–systemic sclerosis patients ( p = 0.0015). Moreover, a greater incidence of pulmonary arterial hypertension ( p Conclusion: Our data show that systemic sclerosis–primary biliary cholangitis exhibit a mild systemic sclerosis and primary biliary cholangitis phenotype with outcomes being in general favourable.

Published
2023

125. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author

Lorenzo, Cavagna, Federica, Meloni, Alain, Meyer, Gianluca, Sambataro, Mirko, Belliato, Ellen De Langhe, Cavazzana, Ilaria, Nicolò, Pipitone, Konstantinos, Triantafyllias, Marta, Mosca, Simone, Barsotti, Giuseppe, Zampogna, Alessandro, Biglia, Giacomo, Emmi, Marianne De Visser, Anneke Van Der Kooi, Paola, Parronchi, Sandrine, Hirschi, Jose Antonio Pereira da Silva, Carlo Alberto Scirè, Federica, Furini, Margherita, Giannini, Olga Martinez Gonzalez, Laura, Damian, Yves, Piette, Vanessa, Smith, Antonio, Mera-Valera, Javier, Bachiller-Corral, Ivan Cabezas Rodriguez, Anahy, M Brandy-Garcia, François, Maurier, Julie, Perrin, Juan, Gonzalez-Moreno, Ulrich, Drott, Christiane, Delbruck, Andreas, Schwarting, Eugenio, Arrigoni, Gian Domenico Sebastiani, Annamaria, Iuliano, Carlotta, Nannini, Luca, Quartuccio, Ana, B Rodriguez Cambron, Maria, Á Blázquez Cañamero, Ignacio Villa Blanco, Giovanni, Cagnotto, Alberto, Pesci, Francesco, Luppi, Giulia, Dei, Fredeswinda Isabel Romero Bueno, Franceschini, Franco, Ilaria, Chiapparoli, Giovanni, Zanframundo, Sara, Lettieri, Ludovico De Stefano, Maurizio, Cutolo, Alessandro, Mathieu, Matteo, Piga, Sergio, Prieto-González, Maria Francisca Moraes-Fontes, Joao Eurico Fonseca, Vega, Jovani, Valeria, Riccieri, Alessandro, Santaniello, Stephen, Montfort, David, Bilocca, Gian Luca Erre, Elena, Bartoloni, Roberto, Gerli, M Cristina Monti, Hanns, M Lorenz, Domenico, Sambataro, Silvia Bellando Randone, Udo, Schneider, Claudia, Valenzuela, Raquel, Lopez-Mejias, Jose, Cifrian, Mayra, Mejia, Monserrat-Ixchel Gonzalez Perez, Sarah, Wendel, Marco, Fornaro, Giacomo De Luca, Giovanni, Orsolini, Maurizio, Rossini, Philippe, Dieude, Johannes, Knitza, Santos, Castañeda, Reinhard, E Voll, Jorge, Rojas-Serrano, Adele, Valentini, Carlo, Vancheri, Marco, Matucci-Cerinic, Eugen, Feist, Veronica, Codullo, Florenzo, Iannone, Jorg, H Distler, Carlomaurizio, Montecucco, Miguel, A Gonzalez-Gay, AENEAS collaborative group, Neurology, ANS - Neuroinfection & -inflammation, AII - Inflammatory diseases, EURO-NMD, Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Repositório da Universidade de Lisboa, Cavagna, Lorenzo, Meloni, Federica, Meyer, Alain, Sambataro, Gianluca, Belliato, Mirko, De Langhe, Ellen, Cavazzana, Ilaria, Pipitone, Nicolò, Triantafyllias, Konstantino, Mosca, Marta, Barsotti, Simone, Zampogna, Giuseppe, Biglia, Alessandro, Emmi, Giacomo, De Visser, Marianne, Van Der Kooi, Anneke, Parronchi, Paola, Hirschi, Sandrine, da Silva, Jose Antonio Pereira, Scirè, Carlo Alberto, Furini, Federica, Giannini, Margherita, Martinez Gonzalez, Olga, Damian, Laura, Piette, Yve, Smith, Vanessa, Mera-Valera, Antonio, Bachiller-Corral, Javier, Cabezas Rodriguez, Ivan, Brandy-Garcia, Anahy M, Maurier, Françoi, Perrin, Julie, Gonzalez-Moreno, Juan, Drott, Ulrich, Delbruck, Christiane, Schwarting, Andrea, Arrigoni, Eugenio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Nannini, Carlotta, Quartuccio, Luca, Rodriguez Cambron, Ana B, Blázquez Cañamero, Maria Á, Villa Blanco, Ignacio, Cagnotto, Giovanni, Pesci, Alberto, Luppi, Francesco, Dei, Giulia, Romero Bueno, Fredeswinda Isabel, Franceschini, Franco, Chiapparoli, Ilaria, Zanframundo, Giovanni, Lettieri, Sara, De Stefano, Ludovico, Cutolo, Maurizio, Mathieu, Alessandro, Piga, Matteo, Prieto-González, Sergio, Moraes-Fontes, Maria Francisca, Fonseca, Joao Eurico, Jovani, Vega, Riccieri, Valeria, Santaniello, Alessandro, Montfort, Stephen, Bilocca, David, Erre, Gian Luca, Bartoloni, Elena, Gerli, Roberto, Monti, M Cristina, Lorenz, Hanns M, Sambataro, Domenico, Bellando Randone, Silvia, Schneider, Udo, Valenzuela, Claudia, Lopez-Mejias, Raquel, Cifrian, Jose, Mejia, Mayra, Gonzalez Perez, Monserrat-Ixchel, Wendel, Sarah, Fornaro, Marco, De Luca, Giacomo, Orsolini, Giovanni, Rossini, Maurizio, Dieude, Philippe, Knitza, Johanne, Castañeda, Santo, Voll, Reinhard E, Rojas-Serrano, Jorge, Valentini, Adele, Vancheri, Carlo, Matucci-Cerinic, Marco, Feist, Eugen, Codullo, Veronica, Iannone, Florenzo, Distler, Jorg H, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel A

Subjects
Lung Diseases, Interferon-Induced Helicase, IFIH1, rapidly progressive interstitial lung diseases, idiopathic inflammatory myopathies, idiopathic inflammatory myopathie, Immunology, Middle Aged, Prognosis, Dermatomyositis, rapidly progressive interstitial lung disease, Rheumatology, melanoma differentiation-associated protein 5 antibody, rapidly progressive interstitial lung diseases, idiopathic inflammatory myopathies, Humans, Immunology and Allergy, Female, Lung Diseases, Interstitial, Interferon-Induced Helicase, Interstitial, melanoma differentiation-associated protein 5 antibody, Autoantibodies, Retrospective Studies, IFIH1
Abstract

© Copyright Clinical and Experimental Rheumatology 2022., Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022

126. Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. the experience of the Italian SIR-SPRING registry and review of the world literature

Author

Clodoveo Ferri, Rossella De Angelis, Dilia Giuggioli, Gianluigi Bajocchi, Lorenzo Dagna, Giovanni Zanframundo, Rosario Foti, Fabio Cacciapaglia, Giovanna Cuomo, Alarico Ariani, Edoardo Rosato, Serena Guiducci, Francesco Girelli, Valeria Riccieri, Elisabetta Zanatta, Silvia Bosello, Ilaria Cavazzana, Francesca Ingegnoli, Maria De Santis, Giuseppe Murdaca, Giuseppina Abignano, Nicoletta Romeo, Alessandra Della Rossa, Maurizio Caminiti, Annamaria Iuliano, Giovanni Ciano, Lorenzo Beretta, Gianluca Bagnato, Ennio Lubrano, Ilenia De Andres, Alessandro Giollo, Marta Saracco, Cecilia Agnes, Federica Lumetti, Amelia Spinella, Luca Magnani, Corrado Campochiaro, Giacomo De Luca, Veronica Codullo, Elisa Visalli, Francesco Masini, Antonietta Gigante, Silvia Bellando-Randone, Greta Pellegrino, Erika Pigatto, Maria Grazia Lazzaroni, Franco Franceschini, Elena Generali, Gianna Mennillo, Simone Barsotti, Giuseppa Pagano Mariano, Francesca Calabrese, Federica Furini, Licia Vultaggio, Simone Parisi, Clara Lisa Peroni, Davide Rozza, Anna Zanetti, Greta Carrara, Giampiero Landolfi, Carlo Alberto Scirè, Gerolamo Bianchi, Enrico Fusaro, Gian Domenico Sebastiani, Marcello Govoni, Salvatore D'Angelo, Franco Cozzi, Andrea Doria, Florenzo Iannone, Carlo Salvarani, Marco Matucci-Cerinic, Ferri, C, De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, and Matucci-Cerinic, M

Subjects
Settore MED/16 - REUMATOLOGIA, systemic sclerosis, Macro-areas, Immunology, Geographical areas, macro-areas, Antibodies, environmental, Environmental, Scleroderma, Tertiary Care Centers, Systemic sclerosi, Rheumatology, Antinuclear, Humans, Immunology and Allergy, scleroderma, Registries, Keywords: Environmental, Referral, Systemic sclerosis, Geographical area, Scleroderma, Systemic, geographical areas, Systemic, referral, Phenotype, Italy, Antibodies, Antinuclear, Macro-area
Abstract

Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature. Materials and methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 ± 26.9 yrs.; mean disease duration 8.9 ± 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas. Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches. Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities.

Published
2022

127. Use of vasoactive/vasodilating drugs for systemic sclerosis (SSc)-related digital ulcers (DUs) in expert tertiary centres: results from the analysis of the observational real-life DeSScipher study

Author

Silvia Bellando-Randone, Ulrich A. Walker, Marc Frerix, Svetlana I. Nihtyanova, Veronika Lóránd, Marco Matucci-Cerinic, Ingo H. Tarner, Serena Vettori, Veronika K. Jaeger, Ulf Müller-Ladner, Giuseppina Abignano, Jérôme Avouac, G. Riemekasten, Cosimo Bruni, Oliver Distler, L. Czirják, Yannick Allanore, Alberto Moggi-Pignone, F. Del Galdo, Jelena Blagojevic, Laura Cometi, Dörte Huscher, Christopher P. Denton, Britta Maurer, Serena Guiducci, Elise Siegert, Blagojevic, Jelena, Abignano, G, Avouac, J, Cometi, L, Frerix, M, Bellando-Randone, S, Guiducci, S, Bruni, C, Huscher, D, Jaeger, V K, Lóránd, V, Maurer, B, Nihtyanova, S, Riemekasten, G, Siegert, E, Tarner, I H, Vettori, S, Walker, U A, Czirják, L, Denton, C P, Distler, O, Allanore, Y, Müller-Ladner, U, Moggi-Pignone, A, Matucci-Cerinic, M, and Del Galdo, F

Subjects
Adult, Male, Drug, medicine.medical_specialty, Combination therapy, Sildenafil, Vasodilator Agents, media_common.quotation_subject, Digital ulcer, Sildenafil Citrate, Fingers, chemistry.chemical_compound, Rheumatology, Internal medicine, Skin Ulcer, medicine, Humans, Iloprost, Prospective Studies, Aged, media_common, Wound Healing, Scleroderma, Systemic, business.industry, Bosentan, General Medicine, Management, Systemic sclerosis, Middle Aged, Europe, Treatment Outcome, chemistry, cGMP-specific phosphodiesterase type 5, Drug Therapy, Combination, Female, Observational study, business, medicine.drug
Abstract

DeSScipher is the first European multicentre study on management of systemic sclerosis (SSc), and its observational trial 1 (OT1) evaluated the efficacy of different drugs for digital ulcer (DU) prevention and healing. The aim of this study was to assess current use of vasoactive/vasodilating agents for SSc-related DU in the expert centres by analysing the baseline data of the DeSScipher OT1.Baseline characteristics of patients enrolled in the OT1 and data regarding DU were analysed.The most commonly used drugs, in both patients with and without DU, were calcium channel blockers (CCBs) (71.6%), followed by intravenous iloprost (20.8%), endothelin receptor antagonists (ERAs) (20.4%) and phosphodiesterase 5 (PDE-5) inhibitors (16.5%). Of patients, 32.6% with DU and 12.8% without DU received two drugs (p 0.001), while 11.5% with DU and 1.9% without DU were treated with a combination of three or more agents (p 0.001). Sixty-five percent of the patients with recurrent DU were treated with bosentan and/or sildenafil. However, 64 out of 277 patients with current DU (23.1%) and 101 (23.6%) patients with recurrent DU were on CCBs alone.Our study shows that CCBs are still the most commonly used agents for DU management in SSc. The proportion of patients on combination therapy was low, even in patients with recurrent DU: almost one out of four patients with current and recurrent DU was on CCBs alone. Prospective analysis is planned to investigate the efficacy of different drugs/drug combinations on DU healing and prevention. Key Points • The analysis of DeSScipher, the first European multicentre study on management of SSc, has shown that the most commonly used vasoactive/vasodilating drugs for DU were CCBs, followed by intravenous Iloprost, ERAs and PDE-5 inhibitors. • More than half of the patients with recurrent DU received bosentan and/or sildenafil. • However, the proportion of patients on combination therapy of more than one vasoactive/vasodilating drug was low and almost one out of four patients with current and recurrent DU was on CCBs alone.

Published
2019

128. Covid-19 And Rheumatic Autoimmune Systemic Diseases: Role of Pre-Existing Lung Involvement and Ongoing Treatments

Author

M. L. Aprile, Rosario Foti, Ruscitti Piero, Giuseppe Varcasia, Ilaria Cavazzana, Nicoletta Romeo, Riccardo Meliconi, Serena Lorini, Serena Guiducci, Amelia Spinella, Roberto Giacomelli, Vincenzo Aiello, Alessandra Della Rossa, Giorgio Amato, Daiana Giannini, Vincenzo Raimondo, Francesco Caso, Ennio Lubrano, Silvia Bosello, Maurizio Caminiti, Monica Monti, Tasso Marco, Francesca Ingegnoli, Giorgio Pettiti, Massimo L'Andolina, Salvatore D'Angelo, Francesca Ragusa, Elisabetta Zanatta, Giacomo De Luca, Riccardo Cecchetti, Franco Franceschini, Greta Pellegrino, Silvia Bellando-Randone, Silvia Martina Ferrari, Micaela Fredi, Veronica Brusi, Lorenzo Dagna, Giusy Elia, Fabio Cacciapaglia, Francesco Ursini, Giuseppina Abignano, Sebastiano Lorusso, Clodoveo Ferri, Anna Linda Zignego, Rodolfo Caminiti, Sabrina Rosaria Paparo, Raffaele Brittelli, Liala Moschetti, Elena Generali, Marco Matucci-Cerinic, Roberta Pellegrini, Ylenia Dal Bosco, Giovanni Zanframundo, Domenico Olivo, Tommaso Ferrari, Alessandro Antonelli, M. Vadacca, Andrea Doria, Pietro Gigliotti, Poupak Varcasia, Enrico Fusaro, Elisa Visalli, Simone Barsotti, Giuseppa Pagano Mariano, Giovanna Cuomo, Florenzo Iannone, Maria De Santis, Valeria Mazzi, Giuseppe Murdaca, Michele Colaci, Silvia Bilia, Franco Cozzi, Dilia Giuggioli, Corrado Campochiaro, Valeria Riccieri, Erika Pigatto, Laura Gragnani, Ilenia Di Cola, Daniela Scorpiniti, Mario Miccoli, Francesco Masini, Veronica Codullo, Rossella De Angelis, Federica Lumetti, Antonio Tavoni, Ferri, Clodoveo, Giuggioli, Dilia, Raimondo, Vincenzo, L’Andolina, Massimo, Dagna, Lorenzo, Tavoni, Antonio, Caso, Francesco, Ursini, Francesco, Piero, Ruscitti, Caminiti, Maurizio, Foti, Rosario, Riccieri, Valeria, Guiducci, Serena, Pellegrini, Roberta, Zanatta, Elisabetta, Varcasia, Giuseppe, Olivo, Domenico, Gigliotti, Pietro, Cuomo, Giovanna, Murdaca, Giuseppe, Cecchetti, Riccardo, De Angelis, Rossella, Romeo, Nicoletta, Ingegnoli, Francesca, Cozzi, Franco, Codullo, Veronica, Cavazzana, Ilaria, Colaci, Michele, Abignano, Giuseppina, De Santis, Maria, Lubrano, Ennio, Fusaro, Enrico, Rossa, Alessandra Della, Spinella, Amelia, Lumetti, Federica, De Luca, Giacomo, Bellando-Randone, Silvia, Visalli, Elisa, Dal Bosco, Ylenia, Amato, Giorgio, Giannini, Daiana, Bilia, Silvia, Masini, Francesco, Pellegrino, Greta, Pigatto, Erika, Generali, Elena, Mariano, Giuseppa Pagano, Pettiti, Giorgio, Zanframundo, Giovanni, Brittelli, Raffaele, Aiello, Vincenzo, Caminiti, Rodolfo, Scorpiniti, Daniela, Ferrari, Tommaso, Campochiaro, Corrado, Brusi, Veronica, Fredi, Micaela, Moschetti, Liala, Cacciapaglia, Fabio, Gragnani, Laura, Monti, Monica, Lorini, Serena, Paparo, Sabrina Rosaria, Ragusa, Francesca, Mazzi, Valeria, Elia, Giusy, Ferrari, Silvia Martina, Di Cola, Ilenia, Vadacca, Marta, Lorusso, Sebastiano, Barsotti, Simone, Aprile, Maria Letizia, Marco, Tasso, Miccoli, Mario, Bosello, Silvia, Matucci-Cerinic, Marco, D'Angelo, Salvatore, Doria, Andrea, Franceschini, Franco, Meliconi, Riccardo, Iannone, Florenzo, Giacomelli, Roberto, Zignego, Anna Linda, Varcasia, Poupak, Antonelli, Alessandro, L'Andolina, Massimo, Della Rossa, Alessandra, Pagano Mariano, Giuseppa, Rosaria Paparo, Sabrina, Martina Ferrari, Silvia, Letizia Aprile, Maria, Linda Zignego, Anna, Ferri C., Giuggioli D., Raimondo V., L'andolina M., Dagna L., Tavoni A., Caso F., Ursini F., Ruscitti P., Caminiti M., Foti R., Riccieri V., Guiducci S., Pellegrini R., Zanatta E., Varcasia G., Olivo D., Gigliotti P., Cuomo G., Murdaca G., Cecchetti R., De Angelis R., Romeo N., Ingegnoli F., Cozzi F., Codullo V., Cavazzana I., Colaci M., Abignano G., De Santis M., Lubrano E., Fusaro E., Della Rossa A., Spinella A., Lumetti F., De Luca G., Bellando-Randone S., Visalli E., Dal Bosco Y., Amato G., Giannini D., Bilia S., Masini F., Pellegrino G., Pigatto E., Generali E., Mariano G.P., Pettiti G., Zanframundo G., Brittelli R., Aiello V., Caminiti R., Scorpiniti D., Ferrari T., Campochiaro C., Brusi V., Fredi M., Moschetti L., Cacciapaglia F., Gragnani L., Monti M., Lorini S., Paparo S.R., Ragusa F., Mazzi V., Elia G., Ferrari S.M., Di Cola I., Vadacca M., Lorusso S., Barsotti S., Aprile M.L., Marco T., Miccoli M., Bosello S., Matucci-Cerinic M., D'angelo S., Doria A., Franceschini F., Meliconi R., Iannone F., Giacomelli R., Zignego A.L., Fallahi P., and Antonelli A.

Subjects
medicine.medical_specialty, Settore MED/16 - REUMATOLOGIA, Referral, Coronavirus disease 2019 (COVID-19), autoimmune systemic disease, systemic sclerosis, Population, COVID-19, SARS-CoV-2, arthritis, autoimmune systemic diseases, connective tissue diseases, interstitial lung disease, rheumatic diseases, Arthritis, Keywords: COVID-19, Scleroderma, Autoimmune Diseases, Covid-19, Autoimmune systemic diseases, Connective tissue diseases, Interstitial lung disease, Rheumatic diseases, Systemic sclerosis, Internal medicine, Rheumatic Diseases, Drug Discovery, Pandemic, medicine, Humans, education, rheumatic disease, Lung, Pandemics, Pharmacology, education.field_of_study, Aspirin, business.industry, covid 19, medicine.disease, arthriti, connective tissue disease, business, medicine.drug
Abstract

Background: The Covid-19 pandemic may have a deleterious impact on patients with autoimmune systemic diseases (ASD) due to their deep immune-system alterations. Objective: This study aims to investigate the prevalence of symptomatic Covid-19 and its correlations with both organ involvement and ongoing treatments in a large series of Italian ASD patients during the first wave of pandemic. Methods: Our multicenter telephone 6-week survey included 3,029 unselected ASD patients enrolled at 36 tertiary referral centers of northern, central, and southern Italian macro-areas with different diffusion of the pandemic. Symptomatic SARS-CoV-2 infection was classified as definite Covid-19 (presence of symptoms plus positive oral/nasopharyngeal swabs) or highly suspected Covid-19 (highly suggestive symptoms, in the absence of a swab testing). Results: A significantly higher prevalence of definite plus highly suspected Covid-19 compared to the Italian general population was detected in the whole ASD series (p=.000), as well as in patients from the three macro-areas (p=.000 in all). Statistically higher prevalence of Covid-19 was also found in connective tissue diseases compared to chronic arthritis subgroup (p=.000) and in ASD patients with pre-existing interstitial lung involvement (p=.000). Patients treated with either conventional disease-modifying anti-rheumatic drugs (DMARDs) and/or biological DMARDs showed a significantly lower prevalence of Covid-19 (p=.000 in both). Finally, scleroderma patients undergoing low-dose aspirin showed a significantly lower rate of Covid-19 compared to those without (p=0.003). Conclusion: The higher prevalence of Covid-19 in ASD patients, along with the significant correlations with important clinical features and therapeutic regimens, suggests the need to develop targeted prevention/management strategies during the current pandemic wave.

Published
2021

129. The role of ultrasound in systemic sclerosis: On the cutting edge to foster clinical and research advancement

Author

Yossra A. Suliman, Tania Santiago, Shinji Watanabe, Michael Hughes, Annamaria Iagnocco, Gemma Lepri, Luna Gargani, Silvia Bellando-Randone, Barbara Ruaro, Daniel E. Furst, Andrea Delle Sedie, Cosimo Bruni, Giovanna Cuomo, Marwin Gutierrez, Hughes, Michael, Bruni, Cosimo, Cuomo, Giovanna, Delle Sedie, Andrea, Gargani, Luna, Gutierrez, Marwin, Lepri, Gemma, Ruaro, Barbara, Santiago, Tania, Suliman, Yossra, Watanabe, Shinji, Iagnocco, Annamaria, Furst, Daniel, Bellando-Randone, Silvia, Hughes, M., Bruni, C., Cuomo, G., Delle Sedie, A., Gargani, L., Gutierrez, M., Lepri, G., Ruaro, B., Santiago, T., Suliman, Y., Watanabe, S., Iagnocco, A., Furst, D., and Bellando-Randone, S.

Subjects
skin, medicine.medical_specialty, Immunology, Complex disease, digital ulcer, Reviews, 030204 cardiovascular system & hematology, Scleroderma, lung, Systemic sclerosi, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Immunology and Allergy, Medicine, scleroderma, musculoskeletal, Systemic sclerosis, ultrasound, 030203 arthritis & rheumatology, business.industry, Ultrasound, medicine.disease, Systemic sclerosis, scleroderma, ultrasound, musculoskeletal, digital ulcer, lung, skin, Radiology, business
Abstract

Ultrasound has been widely explored in systemic sclerosis in the clinical and research settings. Ultrasound allows a non-invasive and ionising radiation-free ‘window’ into this complex disease and is well-suited to repeated examinations. Ultrasound provides novel insights into the pathogenesis and measurement of disease in systemic sclerosis, including early (preclinical) internal organ involvement. The purpose of this review is to describe the role of ultrasound to foster clinical and research advancements in systemic sclerosis relating to (1) musculoskeletal, (2) digital ulcer, (3) lung disease and (4) skin disease. We also highlight unmet needs which much be addressed for ultrasound to assume a central role in systemic sclerosis clinical care and research.

Published
2020

130. The Role of Endogenous Eicosapentaenoic Acid and Docosahexaenoic Acid-Derived Resolvins in Systemic Sclerosis

Author

Francesca Wanda Rossi, Abdurrahman Tufan, Nella Prevete, Marco Matucci-Cerinic, Silvia Bellando-Randone, Aslıhan Avanoǧlu Güler, Amato de Paulis, Mirko Manetti, Avanoglu Guler, A., Rossi, F. W., Bellando-Randone, S., Prevete, N., Tufan, A., Manetti, M., de Paulis, A., and Matucci-Cerinic, M.

Subjects
lcsh:Immunologic diseases. Allergy, 0301 basic medicine, Docosahexaenoic Acids, systemic sclerosis, Immunology, Inflammation, Review, Proinflammatory cytokine, resolvins, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Fibrosis, Immunity, Immunology and Allergy, Medicine, Animals, Humans, adaptive immunity, fibrosis, innate immunity, resolution of inflammation, Efferocytosis, resolvin, Innate immune system, Scleroderma, Systemic, business.industry, Acquired immune system, medicine.disease, 030104 developmental biology, chemistry, Eicosapentaenoic Acid, medicine.symptom, Inflammation Mediators, lcsh:RC581-607, fibrosi, business, Resolvin, 030215 immunology
Abstract

Resolvins, the member of specialized pro-resolving mediators, are produced from omega-3 polyunsaturated fatty acids as a response to an acute inflammatory process in that termination and resolution of inflammation. In the acute inflammation, these lipid mediators limit polymorphonuclear cells infiltration, proinflammatory cytokine production; promote efferocytosis, and regulate several cell types being important roles in innate and adaptive immunity. Any dysregulation or defect of the resolution phase result in prolonged, persistent inflammation and eventually fibrosis. Resolvins are implicated in the development of various chronic autoimmune diseases. Systemic sclerosis (SSc) is a very complicated, chronic autoimmune disorder proceeding with vasculopathy, inflammation, and fibrosis. Dysregulation of innate and adaptive immunity is another important contributing factor in the pathogenesis of SSc. In this review, we will focus on the different roles of this new family of lipid mediators, characterized by the ability to prevent the spread of inflammation and its chronicity in various ways and how they can control the development of fibrotic diseases like SSc.

Published
2020

131. Systemic sclerosis Progression INvestiGation (SPRING) Italian registry: demographic and clinico-serological features of scleroderma spectrum

Author

C. Ferri, D. Giuggioli, S. Guiducci, F. Lumetti, G. Bajocchi, L. Magnani, V. Codullo, A. Ariani, F. Girelli, V. Riccieri, G. Pellegrino, S. Bosello, R. Foti, E. Visalli, G. Amato, A. Benenati, Giovanna Cuomo, F. Iannone, F. Cacciapaglia, R. De Angelis, F. Ingegnoli, R. Talotta, C. Campochiaro, L. Dagna, G. De Luca, S. Bellando-Randone, A. Spinella, G. Murdaca, N. Romeo, M. De Santis, E. Generali, S. Barsotti, A. Della Rossa, I. Cavazzana20, F. Dall’Ara20, M. G. Lazzaroni20, F. Cozzi22, A. Doria22, E. Pigatto22, E. Zanatta22, G. Ciano23, L. Beretta24, G. Abignano25, S. D’Angelo25, G. Mennillo25, G. Bagnato26, F. Calabrese27, M. Caminiti27, G. Pagano Mariano27, E. Battaglia28, E. Lubrano29, G. Zanframundo4, A. Iuliano30, F. Furini31, A. Zanetti32, G. Carrara32, F. Rumi32, C. A. Scirè31, M. Matucci-Cerinic2, on behalf of the Italian Society of Rheumatology (SIR), Ferri, C., Giuggioli, D., Guiducci, S., Lumetti, F., Bajocchi, G., Magnani, L., Codullo, V., Ariani, A., Girelli, F., Riccieri, V., Pellegrino, G., Bosello, S., Foti, R., Visalli, E., Amato, G., Benenati, A., Cuomo, Giovanna, Iannone, F., Cacciapaglia, F., De Angelis, R., Ingegnoli, F., Talotta, R., Campochiaro, C., Dagna, L., De Luca, G., Bellando-Randone, S., Spinella, A., Murdaca, G., Romeo, N., De Santis, M., Generali, E., Barsotti, S., Della Rossa, A., Cavazzana20, I., Dall’Ara20, F., Lazzaroni20, M. G., Cozzi22, F., Doria22, A., Pigatto22, E., Zanatta22, E., Ciano23, G., Beretta24, L., Abignano25, G., D’Angelo25, S., Mennillo25, G., Bagnato26, G., Calabrese27, F., Caminiti27, M., Pagano Mariano27, G., Battaglia28, E., Lubrano29, E., Zanframundo4, G., Iuliano30, A., Furini31, F., Zanetti32, A., Carrara32, G., Rumi32, F., Scirè31, C. A., Matucci-Cerinic2, M., and behalf of the Italian Society of Rheumatology (SIR), On

Subjects
systemic sclerosis, VEDOSS, Raynaud’s phenomenon
Abstract

Objective. Systemic sclerosis (SSc) is a severe multiple-organ disease charac- terised by unpredictable clinical course, inadequate response to treatment, and poor prognosis. National SSc registries may provide large and representative patients cohorts required for descriptive and prognostic studies. Therefore, the Italian Society of Rheumatology pro- moted the registry SPRING (Systemic sclerosis Progression INvestiGation). Methods. The SPRING is a multi- centre rheumatological cohort study encompassing the wide scleroder- ma spectrum, namely the primary Raynaud’s phenomenon (pRP), sus- pected secondary RP, Very Early Diag- nosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteris- tics of 2,028 Italian patients’ popula- tion at the initial phase of enrollment, mainly focusing on the cohort of 1,538 patients with definite SSc. Results. Definite SSc showed signifi- cantly higher prevalence of digital ul- cers, capillaroscopic ‘late’ pattern, oesophageal and cardio-pulmonary involvement compared to VEDOSS, as expected on the basis of the followed classification criteria. The in-depth analysis of definite SSc revealed that male gender, diffuse cu- taneous subset, and anti-Scl70 sero- positivity were significantly associated with increased prevalence of the most harmful disease manifestations. Similarly, patients with very short RP duration (≤1 year) at SSc diagnosis showed a statistically increased preva- lence of unfavorable clinico-serologi- cal features. Conclusion. Nationwide registries with suitable patients’ subsetting and follow-up studies since the prodromal phase of the disease may give us valu- able insights on the SSc natural history and main prognostic factors.

Published
2020

132. Systemic sclerosis Progression INvestiGation (SPRING) Italian registry: demographic and clinico-serological features of scleroderma spectrum

Author

Ferri, Clodoveo, Giuggioli, Dilia, Guiducci, Serena, Lumetti, Federica, Bajocchi, Gianluigi, Magnani, Luca, Codullo, Veronica, Ariani, Alarico, Girelli, Francesco, Riccieri, Valeria, Pellegrino, Greta, Bosello, Silvia, Foti, Rosario, Visalli, Elisa, Amato, Giorgio, Benenati, Alessia, Cuomo, Giovanna, Iannone, Florenzo, Cacciapaglia, Fabio, Angelis, Rossella, Ingegnoli, Francesca, ROSSELLA TALOTTA, Campochiaro, Corrado, Dagna, Lorenzo, Luca, Giacomo, Bellando-Randone, Silvia, Spinella, Amelia, Murdaca, Giuseppe, Romeo, Nicoletta, Santis, Maria, Generali, Elena, Barsotti, Simone, Della Rossa, Alessandra, Cavazzana, Ilaria, Dall Ara, Francesca, Lazzaroni, Maria G., Cozzi, Franco, Andrea Doria, Pigatto, Erika, ELISABETTA ZANATTA, Ciano, Giovanni, Beretta, Lorenzo, Abignano, Giuseppina, D Angelo, Salvatore, Mennillo, Gianna, Bagnato, Gianluca, Calabrese, Francesca, Caminiti, Maurizio, Pagano Mariano, Giuseppa, Battaglia, Elisabetta, Lubrano, Ennio, Zanframundo, Giovanni, Iuliano, Annamaria, Furini, Federica, Zanetti, Anna, Carrara, Greta, Rumi, Federica, Scirè, Carlo Alberto, Matucci-Cerinic, Marco, Ferri, C, Giuggioli, D, Guiducci, S, Lumetti, F, Bajocchi, G, Magnani, L, Codullo, V, Ariani, A, Girelli, F, Riccieri, V, Pellegrino, G, Bosello, S, Foti, R, Visalli, E, Amato, G, Benenati, A, Cuomo, G, Iannone, F, Cacciapaglia, F, De Angelis, R, Ingegnoli, F, Talotta, R, Campochiaro, C, Dagna, L, De Luca, G, Bellando-Randone, S, Spinella, A, Murdaca, G, Romeo, N, De Santis, M, Generali, E, Barsotti, S, Della Rossa, A, Cavazzana, I, Dall'Ara, F, Lazzaroni, M, Cozzi, F, Doria, A, Pigatto, E, Zanatta, E, Ciano, G, Beretta, L, Abignano, G, D'Angelo, S, Mennillo, G, Bagnato, G, Calabrese, F, Caminiti, M, Pagano Mariano, G, Battaglia, E, Lubrano, E, Zanframundo, G, Iuliano, A, Furini, F, Zanetti, A, Carrara, G, Rumi, F, Scirè, C, Matucci-Cerinic, M, Ferri, Clodoveo, Giuggioli, Dilia, Guiducci, Serena, Lumetti, Federica, Bajocchi, Gianluigi, Magnani, Luca, Codullo, Veronica, Ariani, Alarico, Girelli, Francesco, Riccieri, Valeria, Pellegrino, Greta, Bosello, Silvia, Foti, Rosario, Visalli, Elisa, Amato, Giorgio, Benenati, Alessia, Cuomo, Giovanna, Iannone, Florenzo, Cacciapaglia, Fabio, De Angelis, Rossella, Ingegnoli, Francesca, Talotta, Rossella, Campochiaro, Corrado, Dagna, Lorenzo, De Luca, Giacomo, Bellando-Randone, Silvia, Spinella, Amelia, Murdaca, Giuseppe, Romeo, Nicoletta, De Santis, Maria, Generali, Elena, Barsotti, Simone, Della Rossa, Alessandra, Cavazzana, Ilaria, Dall'Ara, Francesca, Lazzaroni, Maria G, Cozzi, Franco, Doria, Andrea, Pigatto, Erika, Zanatta, Elisabetta, Ciano, Giovanni, Beretta, Lorenzo, Abignano, Giuseppina, D'Angelo, Salvatore, Mennillo, Gianna, Bagnato, Gianluca, Calabrese, Francesca, Caminiti, Maurizio, Pagano Mariano, Giuseppa, Battaglia, Elisabetta, Lubrano, Ennio, Zanframundo, Giovanni, Iuliano, Annamaria, Furini, Federica, Zanetti, Anna, Carrara, Greta, Rumi, Federica, Scirè, Carlo Alberto, and Matucci-Cerinic, Marco

Subjects
Male, Scleroderma, Systemic, Settore MED/16 - REUMATOLOGIA, systemic sclerosis, Raynaud’s phenomenon, VEDOSS, Raynaud Disease, Microscopic Angioscopy, Cohort Studies, Systemic sclerosi, Italy, Humans, Registries, Systemic sclerosis
Abstract

Objectives: Systemic sclerosis (SSc) is a severe multiple-organ disease characterised by unpredictable clinical course, inadequate response to treatment, and poor prognosis. National SSc registries may provide large and representative patients cohorts required for descriptive and prognostic studies. Therefore, the Italian Society for Rheumatology promoted the registry SPRING (Systemic sclerosis Progression INvestiGation). Methods: The SPRING is a multi-centre rheumatological cohort study encompassing the wide scleroderma spectrum, namely the primary Raynaud's phenomenon (pRP), suspected secondary RP, Very Early Diagnosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteristics of a population of 2,028 Italian patients at the initial phase of enrolment, mainly focusing on the cohort of 1,538 patients with definite SSc. Results: Definite SSc showed a significantly higher prevalence of digital ulcers, capillaroscopic 'late' pattern, oesophageal and cardio-pulmonary involvement compared to VEDOSS, as expected on the basis of the followed classification criteria. The in-depth analysis of definite SSc revealed that male gender, diffuse cutaneous subset, and anti-Scl70 seropositivity were significantly associated with increased prevalence of the most harmful disease manifestations. Similarly, patients with very short RP duration (≤1 year) at SSc diagnosis showed a statistically increased prevalence of unfavourable clinico-serological features. Conclusions: Nationwide registries with suitable subsetting of patients and follow-up studies since the prodromal phase of the disease may give us valuable insights into the SSc natural history and main prognostic factors.

Published
2020

133. Quantitative analysis of pulmonary vasculature in systemic sclerosis at spirometry-gated chest CT

Author

Silvia Bellando-Randone, Federico Lavorini, Anna Bassetto, Giovanna Cuomo, Stefano Colagrande, Marco Matucci-Cerinic, Dilia Giuggioli, S. Tomassetti, Massimo Pistolesi, Cosimo Bruni, Gianna Camiciottoli, Maurizio Bartolucci, Alessio Fabbrizzi, Giulia Ciardi, Mariaelena Occhipinti, Occhipinti, M., Bruni, C., Camiciottoli, G., Bartolucci, M., Bellando-Randone, S., Bassetto, A., Cuomo, G., Giuggioli, D., Ciardi, G., Fabbrizzi, A., Tomassetti, S., Lavorini, F., Pistolesi, M., Colagrande, S., and Matucci-Cerinic, M.

Subjects
Lung Diseases, pulmonary fibrosi, Male, systemic sclerosis, Vital Capacity, 030204 cardiovascular system & hematology, Scleroderma, Pulmonary function testing, 0302 clinical medicine, arterial hypertension, cardiovascular disease, disease activity, pulmonary fibrosis, Adult, Female, Humans, Logistic Models, Lung, Lung Diseases, Interstitial, Middle Aged, Prospective Studies, Respiratory Function Tests, Scleroderma, Systemic, Spirometry, Statistics, Nonparametric, Tomography, X-Ray Computed, DLCO, Pulmonary fibrosis, Immunology and Allergy, Medicine, Lung volumes, Tomography, Respiratory Function Test, medicine.diagnostic_test, Statistics, Interstitial lung disease, respiratory system, X-Ray Computed, medicine.anatomical_structure, Cardiology, systemic sclerosi, Human, medicine.medical_specialty, Logistic Model, Immunology, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, FEV1/FVC ratio, Rheumatology, Internal medicine, Nonparametric, business.industry, Systemic, medicine.disease, Prospective Studie, 030228 respiratory system, Interstitial, business
Abstract

ObjectiveTo prospectively investigate whether differences in pulmonary vasculature exist in systemic sclerosis (SSc) and how they are distributed in patients with different pulmonary function.MethodsSeventy-four patients with SSc undergoing chest CT scan for interstitial lung disease (ILD) screening or follow-up were prospectively enrolled. A thorough clinical, laboratory and functional evaluation was performed the same day. Chest CT was spirometry gated at total lung capacity and images were analysed by two automated software programs to quantify emphysema, ILD patterns (ground-glass, reticular, honeycombing), and pulmonary vascular volume (PVV). Patients were divided in restricted (FVC% ResultsAbsolute and lung volume normalised PVV (PVV/LV) correlated inversely with functional parameters and positively with all ILD patterns (ρ=0.75 with ground glass, ρ=0.68 with reticular). PVV/LV was the only predictor of DLco at multivariate analysis (p=0.007). Meanwhile, the reticular pattern prevailed in peripheral regions and lower lung thirds, PVV/LV prevailed in central regions and middle lung thirds. iDLco group had a significantly higher PVV/LV (2.2%) than normal (1.6%), but lower than restricted ones (3.8%).ConclusionsChest CT in SSc detects a progressive increase in PVV/LV as DLco decreases. Redistribution of perfusion to less affected lung regions rather than angiogenesis nearby fibrotic lung may explain the results. Further studies to ascertain whether the increase in PVV/LV reflects a real increase in blood volume are needed.

Published
2020

134. COVID-19 and rheumatic autoimmune systemic diseases: report of a large Italian patients series

Author

Giuseppa Pagano Mariano, Maurizio Caminiti, Alessandro Antonelli, Vincenzo Aiello, Dilia Giuggioli, Tommaso Ferrari, Massimo L'Andolina, Michele Colaci, Rodolfo Caminiti, Serena Guiducci, Silvia Bilia, Raffaele Brittelli, Daiana Giannini, Domenico Olivo, Giuseppe Varcasia, Veronica Brusi, Riccardo Meliconi, Vincenzo Raimondo, Amelia Spinella, Poupak Fallahi, Pietro Gigliotti, Clodoveo Ferri, Roberta Pellegrini, Giuseppe Murdaca, Silvia Bellando-Randone, R. Cecchetti, Antonio Tavoni, Francesco Ursini, Ferri C., Giuggioli D., Raimondo V., L'Andolina M., Tavoni A., Cecchetti R., Guiducci S., Ursini F., Caminiti M., Varcasia G., Gigliotti P., Pellegrini R., Olivo D., Colaci M., Murdaca G., Brittelli R., Mariano G.P., Spinella A., Bellando-Randone S., Aiello V., Bilia S., Giannini D., Ferrari T., Caminiti R., Brusi V., Meliconi R., Fallahi P., and Antonelli A.

Subjects
0301 basic medicine, Male, Systemic disease, Arthritis, Autoimmune systemic diseases, Connective tissue diseases, COVID-19, Rheumatic diseases, SARS-CoV-2, Inflammatory arthritis, Psoriatic, Disease, Scleroderma, Arthritis, Rheumatoid, 0302 clinical medicine, Glucocorticoid, Rheumatoid, 80 and over, Medicine, Lupus Erythematosus, Systemic, Viral, Aged, 80 and over, education.field_of_study, Undifferentiated connective tissue disease, Antirheumatic Agent, General Medicine, Middle Aged, Sjogren's Syndrome, Italy, Adult, Aged, Antirheumatic Agents, Arthritis, Psoriatic, Autoimmune Diseases, Betacoronavirus, Coronavirus Infections, Dermatomyositis, Female, Glucocorticoids, Humans, Pandemics, Pneumonia, Viral, Rheumatic Diseases, Scleroderma, Systemic, Spondylitis, Ankylosing, Undifferentiated Connective Tissue Diseases, Original Article, Arthriti, Human, Ankylosing, medicine.medical_specialty, Population, Autoimmune systemic disease, Autoimmune Disease, Rheumatic Disease, 03 medical and health sciences, Rheumatology, Internal medicine, education, Connective tissue disease, 030203 arthritis & rheumatology, Dermatomyositi, Betacoronaviru, Pandemic, Lupus Erythematosus, business.industry, Coronavirus Infection, Systemic, Pneumonia, medicine.disease, 030104 developmental biology, business, Spondylitis
Abstract

IntroductionCovid-19 infection poses a serious challenge for immune-compromised patients with inflammatory autoimmune systemic diseases. We investigated the clinical-epidemiological findings of 1641 autoimmune systemic disease Italian patients during the Covid-19 pandemic.MethodThis observational multicenter study included 1641 unselected patients with autoimmune systemic diseases from three Italian geographical areas with different prevalence of Covid-19 [high in north (Emilia Romagna), medium in central (Tuscany), and low in south (Calabria)] by means of telephone 6-week survey. Covid-19 was classified as (1)definitediagnosis of Covid-19 disease: presence of symptomatic Covid-19 infection, confirmed by positive oral/nasopharyngeal swabs; (2)highly suspectedCovid-19 disease: presence of highly suggestive symptoms, in absence of a swab test.ResultsA significantly higher prevalence of patients withdefinitediagnosis of Covid-19 disease,or withhighly suspectedCovid-19 disease, or both the conditions together, was observed in the whole autoimmune systemic disease series, compared to “Italian general population” (p = .030,p = .001,p = .000, respectively); and fordefinite + highly suspecteddiagnosis of Covid-19 disease, in patients with autoimmune systemic diseases of the three regions (p = .000, for all comparisons with the respective regional general population).Moreover, significantly higher prevalence ofdefinite + highly suspecteddiagnosis of Covid-19 disease was found either in patients with various “connective tissue diseases” compared to “inflammatory arthritis group” (p p = .011).ConclusionsThe finding of a higher prevalence of Covid-19 in patients with autoimmune systemic diseases is particularly important, suggesting the need to develop valuable prevention/management strategies, and stimulates in-depth investigations to verify the possible interactions between Covid-19 infection and impaired immune-system of autoimmune systemic diseases.Key Points• Significantly higher prevalence of Covid-19 is observed in a large series of patients with autoimmune systemic diseases compared to the Italian general population, mainly due to patients’ increased susceptibility to infections and favored by the high exposure to the virus at medical facilities before the restriction measures on individual movement.• The actual prevalence of Covid-19 in autoimmune systemic diseases may be underestimated, possibly due to the wide clinical overlapping between the two conditions, the generally mild Covid-19 disease manifestations, and the limited availability of virological testing.• Patients with “connective tissue diseases” show a significantly higher prevalence of Covid-19, possibly due to deeper immune-system impairment, with respect to “inflammatory arthritis group”.• Covid-19 is more frequent in the subgroup of autoimmune systemic diseases patients without ongoing conventional synthetic disease-modifying anti-rheumatic drugs, mainly hydroxyl-chloroquine and methotrexate, which might play some protective role against the most harmful manifestations of Covid-19.

Published
2020

135. Alveolar haemorrhage in ANCA-associated vasculitis: Long-term outcome and mortality predictors

Author

Francesco Cianci, Francesco Ferro, Viviana Ravagnani, Sara Monti, Angela Padula, Silvia Balduzzi, Alvise Berti, Roberto Caporali, Paolo Stobbione, Marcello Govoni, Silvano Bettio, I. Leccese, M. C. Ditto, Stefano Murgia, Marco Matucci Cerinic, Lorenzo Dagna, Franco Schiavon, Federica Furini, Alessandra Bortoluzzi, Michele Colaci, Silvia Bellando Randone, Bernd Raffeiner, P.P. Sainaghi, Gian Luca Erre, Francesco Carubbi, Dario Roccatello, Milena Bond, Giulia Pazzola, Giuseppe Paolazzi, Mara Felicetti, Carlo Salvarani, Giuseppina Alfieri, Aurora Ianniello, Elena Silvestri, Roberto Padoan, Alessandro Giollo, Luca Quartuccio, Roberto Bortolotti, Claudia Lomater, Simone Parisi, Adriana Cariddi, Enrica Bozzolo, Salvatore D'Angelo, Giacomo Emmi, Salvatore De Vita, Gerardo Di Scala, Miriam Isola, Pietro Leccese, Elisa Gremese, Nicoletta Franzolini, Fabrizio Conti, Paola Faggioli, Quartuccio, L., Bond, M., Isola, M., Monti, S., Felicetti, M., Furini, F., Murgia, S., Berti, A., Silvestri, E., Pazzola, G., Bozzolo, E., Leccese, P., Raffeiner, B., Parisi, S., Leccese, I., Cianci, F., Bettio, S., Sainaghi, P., Ianniello, A., Ravagnani, V., Bellando Randone, S., Faggioli, P., Lomater, C., Stobbione, P., Ferro, F., Colaci, M., Alfieri, G., Carubbi, F., Erre, G. L., Giollo, A., Franzolini, N., Ditto, M. C., Balduzzi, S., Padoan, R., Bortolotti, R., Bortoluzzi, A., Cariddi, A., Padula, A., Di Scala, G., Gremese, E., Conti, F., D'Angelo, S., Matucci Cerinic, M., Dagna, L., Emmi, G., Salvarani, C., Paolazzi, G., Roccatello, D., Govoni, M., Schiavon, F., Caporali, R., and De Vita, S.

Subjects
0301 basic medicine, Vasculitis, Adult, Male, medicine.medical_specialty, Immunology, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Hemorrhage, NO, Alveolar haemorrhage, Mortality, Outcome, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Immunology and Allergy, Medicine, Humans, Public Health Surveillance, Cause of death, Aged, Retrospective Studies, 030203 arthritis & rheumatology, Performance status, business.industry, Hazard ratio, Retrospective cohort study, Middle Aged, medicine.disease, Prognosis, Confidence interval, Pulmonary Alveoli, 030104 developmental biology, Respiratory failure, Italy, Female, business, Cohort study
Abstract

Introduction Alveolar haemorrhage (AH) is considered an important cause of morbidity and early mortality in anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV). Objectives The aim of this study was to identify predictors of outcome in patients with AH-AAV and to evaluate outcome and causes of death in this subset. Materials and methods A multicenter retrospective study was conducted in 29 Italian Centers. Clinicians were asked to recruit all patients diagnosed with AAV-associated AH during the last 10 years, from 2007 to 2016. Univariate and multivariable analysis were performed. Results One-hundred and six patients were included (median age at onset of 55 years [IQR 42–67]). The majority were ANCA-positive (PR3 57.1%, MPO 33.7%) and 72.6% had also renal involvement. At presentation, anaemia was shown in 97 (92.4%) patients, hemoptysis in 54 (51.9%), respiratory failure in 68 (66.7%), of whom 48 (70.6%), requiring respiratory support. At the end of the 37 months [IQR 13–77] follow-up, 19/106 (17.9%) patients were dead. The main causes of death were active disease and infections. By stepwise regression analysis, age >65 years (HR 3.66 [95% CI 1.4–9.51], p = 0.008) and the need for respiratory support (HR 4.58 [95% CI 1.51–13.87], p = 0.007) at AH onset were confirmed to be predictive of mortality. Conclusions Predictors of outcome in AAV-AH were determined. Factors related to the patient's performance status and the severity of the lung involvement strongly influenced the outcome. Balancing harms and benefits for the individual patient in induction and maintenance treatment strategies is crucial.

Published
2020

136. Kidney involvement in systemic sclerosis: From pathogenesis to treatment

Author

Francesca Wanda Rossi, Silvia Bellando-Randone, Emanuela Praino, Cosimo Bruni, Giovanna Cuomo, Bruni, C., Cuomo, G., Rossi, F. W., Praino, E., Bellando-Randone, S., Bruni, Cosimo, Cuomo, Giovanna, Rossi, Francesca W., Praino, Emanuela, and Bellando-Randone, Silvia

Subjects
030203 arthritis & rheumatology, 0301 basic medicine, medicine.medical_specialty, Kidney, business.industry, Immunology, Scleroderma Renal Crisis, Reviews, Renal function, Scleroderma renal crisi, Gastroenterology, Pathogenesis, Systemic sclerosi, 03 medical and health sciences, 030104 developmental biology, 0302 clinical medicine, medicine.anatomical_structure, Systemic sclerosis, Kidney, Scleroderma renal crisis, Renal function, Rheumatology, Internal medicine, medicine, Immunology and Allergy, business
Abstract

Among all possible systemic sclerosis internal organ complications, kidney involvement is frequently neglected or underestimated, except for the life-threatening scleroderma renal crisis. Fortunately, this severe clinical presentation is nowadays better controlled with available treatments, in particular angiotensin-converting enzyme inhibitors, and this has led to a reduction in its short- and longer-term mortality. Pathogenetic determinants are not well understood and many different other kidney involvements are possible in systemic sclerosis, including proteinuria, albuminuria, reduction of renal filtration, autoantibodies-related glomerulonephritis, and drug-related side effects. Different serological and radiological methods of evaluations are nowadays available, some representing promising diagnostic tool and prognostic outcome measure. Except for angiotensin-converting enzyme inhibitors in scleroderma renal crisis, no other treatment is currently recommended for treatment of kidney involvement in systemic sclerosis. For this reason, further studies are necessary to investigate its prognostic impact, in particular in combination with other systemic sclerosis–related internal organ manifestations. This review summarizes current available literature on kidney involvement in systemic sclerosis.

Published
2018

137. The Renal Resistive Index in systemic sclerosis: Determinants, prognostic implication and proposal for specific age-adjusted cut-offs

Author

Cosimo Bruni, Edoardo Rosato, Maria Boddi, Jelena Blagojevic, Marco Chiostri, Marco Matucci-Cerinic, Khadija El Aoufy, Silvia Bellando-Randone, Giulia Tesei, Antonietta Gigante, Vanessa Maestripieri, Daniel E. Furst, Serena Guiducci, Amato de Paulis, Alberto Moggi-Pignone, Bruni, C., Rosato, E., Maestripieri, V., Gigante, A., Tesei, G., Bellando-Randone, S., Guiducci, S., Chiostri, M., El Aoufy, K., Blagojevic, J., Moggi-Pignone, A., De Paulis, A., Furst, D. E., Boddi, M., and Matucci-Cerinic, M.

Subjects
Male, Percentile, Fibrosi, Blood Pressure, Vasculopathy, Disease, 030204 cardiovascular system & hematology, Renal artery Doppler ultrasound, Kidney, Kidney Function Tests, Severity of Illness Index, Systemic sclerosi, Renal Artery, 0302 clinical medicine, Fibrosis, 030212 general & internal medicine, education.field_of_study, integumentary system, Interstitial lung disease, Middle Aged, Prognosis, Renal Resistive Index, Systemic sclerosis, medicine.anatomical_structure, Italy, Quartile, Cardiology, Female, Adult, medicine.medical_specialty, Prognosi, Population, Age adjustment, 03 medical and health sciences, Internal medicine, Internal Medicine, medicine, Humans, education, Aged, Retrospective Studies, Scleroderma, Systemic, Lung, business.industry, Ultrasonography, Doppler, medicine.disease, Survival Analysis, Vascular Resistance, business, human activities
Abstract

Background Renal Resistive Index (RRI), reflects changes in both renal vascular and tubular-interstitial compartments and in systemic vascular compliance related to age and comorbidities. Objectives a) To investigate determinants of RRI in SSc population, b) its association with SSc-related features and c) to test its prognostic impact on organ specific worsening or death. Methods 380 SSc patients ≥18 years were enrolled after giving informed consent. Baseline data on RRI, laboratory, instrumental and therapeutic features were retrospectively collected. Age-SSc adjusted cut-offs were created by dividing the population in age quartiles and considering RRI values >75th percentile as pathologic. Clinical follow-up was performed until last available visit or the development/worsening of specific internal organ involvement or death. Results RRI was independently predicted by age and systolic pulmonary arterial pressure on Echo. Therefore, we created Age-SSc adjusted pathologic RRI cut-offs, which were significantly associated with various disease related skin and lung fibrotic manifestations, as well as vasculopathic complications. After a mean follow-up of 3.6 ± 2.6 years, RRI was one of the independent predictors (together with modified Rodnan skin score, interstitial lung disease, presence of dyspnoea and late nailfold-videocapillaroscopy pattern) for mortality, with 0.68 as best cut-off (sensitivity 88.5%, specificity 50.9%). Conclusion If corroborated, Renal Resistive Index cut-offs might be used to evaluate renal and extrarenal involvement in SSc and could serve as predictors of mortality.

Published
2019

138. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author

Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrián, José Manuel, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I., Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, González-Gay, Miguel A., Universitat Autònoma de Barcelona, Universidad de Cantabria, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, and Gonzalez-Gay, M

Subjects
medicine.medical_specialty, antisynthetase antibodies, antisynthetase syndrome, arthritis, interstitial lung disease, myositis, Medizin, Arthritis, lcsh:Medicine, Antisynthetase syndrome, Interstitial lung disease, Article, NO, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, antisynthetase antibodies, antisynthetase syndrome, arthritis, interstitial lung disease, myositis, ddc:610, Myositis, 030203 arthritis & rheumatology, Antisynthetase antibodies, biology, business.industry, lcsh:R, Autoantibody, General Medicine, medicine.disease, arthriti, 030228 respiratory system, Time course, Cohort, biology.protein, Antibody, business, antisynthetase antibodie
Abstract

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition. ispartof: JOURNAL OF CLINICAL MEDICINE vol:8 issue:11 ispartof: location:Switzerland status: published

Published
2019

139. Disease activity assessment of rheumatic diseases during pregnancy: a comprehensive review of indices used in clinical studies

Author

Pier Luigi Meroni, Mauro Galeazzi, Maria Sole Chimenti, Marco Matucci-Cerinic, Marta Mosca, Carlo Salvarani, Micaela Fredi, Gian Domenico Sebastiani, Maria Gerosa, Salvatore D'Angelo, Antonio Brucato, Giulia Pazzola, Véronique Ramoni, Alessandra Bortoluzzi, Angela Tincani, Laura Andreoli, Andrea Doria, Maria Chiara Gerardi, Paola Conigliaro, Roberto Perricone, Maria Stefania Cutro, Massimo Patanè, Maurizio Cutolo, Marcello Govoni, Cecilia Beatrice Chighizola, Carlo Alberto Scirè, M. Pendolino, M. Meroni, Roberto Caporali, Guido Valesini, Annamaria Iuliano, Elena Elefante, Silvia Bellando-Randone, Francesca Romana Spinelli, Maddalena Larosa, Melissa Alexandre Fernandes, Carlo Selmi, Maria Grazia Lazzaroni, Andreoli, L, Gerardi, M, Fernandes, M, Bortoluzzi, A, Bellando-Randone, S, Brucato, A, Caporali, R, Chighizola, C, Chimenti, M, Conigliaro, P, Cutolo, M, Cutro, M, D'Angelo, S, Doria, A, Elefante, E, Fredi, M, Galeazzi, M, Gerosa, M, Govoni, M, Iuliano, A, Larosa, M, Lazzaroni, M, Matucci-Cerinic, M, Meroni, M, Meroni, P, Mosca, M, Patane, M, Pazzola, G, Pendolino, M, Perricone, R, Ramoni, V, Salvarani, C, Sebastiani, G, Selmi, C, Spinelli, F, Valesini, G, Scire, C, and Tincani, A

Subjects
0301 basic medicine, Vasculitis, medicine.medical_specialty, Vasculiti, Pregnancy, Activity indices, Rheumatoid arthritis, Spondyloarthritis, Systemic lupus erythematosus, Autoimmune diseases, Vasculitis, Autoimmune diseases, Immunology, Disease, Systemic lupus erythematosu, NO, Activity indices, Pregnancy, Rheumatoid arthritis, Spondyloarthritis, Systemic lupus erythematosus, Disease activity, 03 medical and health sciences, 0302 clinical medicine, Rheumatic Diseases, Autoimmune disease, medicine, Immunology and Allergy, Humans, Intensive care medicine, Rheumatoid arthriti, 030203 arthritis & rheumatology, Fetus, business.industry, Activity indice, Maternal disease, medicine.disease, HCC MED, Clinical Practice, Pregnancy Complications, Settore MED/16 - Reumatologia, 030104 developmental biology, Female, Spondyloarthriti, business, Active inflammation
Abstract

Pregnancy requires a special management in women with inflammatory rheumatic diseases (RDs), with the aim of controlling maternal disease activity and avoiding fetal complications. Despite the heterogeneous course of RDs during pregnancy, their impact on pregnancy largely relates to the extent of active inflammation at the time of conception. Therefore, accurate evaluation of disease activity is crucial for the best management of pregnant patients. Nevertheless, there are limitations in using conventional measures of disease activity in pregnancy, as some items included in these instruments can be biased by symptoms or by physiological changes related to pregnancy and the pregnancy itself may influence laboratory parameters used to assess disease activity. This article aims to summarize the current literature about the available instruments to measure disease activity during pregnancy in RDs. Systemic lupus erythematosus is the only disease with instruments that have been modified to account for several adaptations which might interfere with the attribution of signs or symptoms to disease activity during pregnancy. No modified-pregnancy indices exist for women affected by other RDs, but standard indices have been applied to pregnant patients. The current body of knowledge shows that the physiologic changes that occur during pregnancy need to be either adapted from existing instruments or developed to improve the management of pregnant women with RDs. Standardized instruments to assess disease activity during pregnancy would be helpful not only for clinical practice but also for research purposes. info:eu-repo/semantics/publishedVersion

Published
2019

140. Combination therapy with Bosentan and Sildenafil improves Raynaud’s phenomenon and fosters the recovery of microvascular involvement in systemic sclerosis

Author

Laura Cometi, Serena Guiducci, Jelena Blagojevic, A de Paulis, Marco Matucci-Cerinic, A. Radicati, Gemma Lepri, Silvia Bellando-Randone, Cosimo Bruni, Bellando Randone, S, Lepri, G., Bruni, C., Blagojevic, J., Radicati, A., Cometi, L., DE PAULIS, Amato, Matucci Cerinic, M., and Guiducci, S.

Subjects
Adult, Male, 0301 basic medicine, medicine.medical_specialty, Combination therapy, Sildenafil, Vasodilator Agents, Condition score, Gastroenterology, Sildenafil Citrate, Scleroderma, Microscopic Angioscopy, Systemic sclerosi, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Microvasculature, Rheumatology, Internal medicine, Humans, Medicine, Aged, Retrospective Studies, 030203 arthritis & rheumatology, Sulfonamides, Scleroderma, Systemic, business.industry, Raynaud Disease, Bosentan, Retrospective cohort study, General Medicine, Middle Aged, medicine.disease, Capillaries, Surgery, Treatment Outcome, 030104 developmental biology, Nails, chemistry, Microvessels, Drug Therapy, Combination, Female, business, Rheumatism, medicine.drug
Abstract

The aim of this study was to evaluate in systemic sclerosis (SSc) retrospectively the effect of Bosentan and Sildenafil and their combination on Raynaud’s phenomenon (RP), function, and capillaroscopic patterns. One hundred and twenty-three SSc patients (mean age ± sd, 57.69 ± 14.07 years) were retrospectively evaluated and divided into two groups according to American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification score: group 1 score < 10, group 2 score > 10. Each group was divided into three subgroups according to treatment: Bosentan, Sildenafil, and Bosentan + Sildenafil. Nailfold videocapillaroscopy (NVC), Scleroderma Health Assessment Questionnaire (SHAQ) and Raynaud Condition Score (RCS) were performed at baseline and after 3 and 6 months. In Bosentan (29 patients: 12, group 1; 17, group 2), NVC changed significantly in both groups, after 3 and 6 months (p = 0.00439, group 1; p = 0.00035, group 2). In group 1, the “active” and the “late” patterns reduced, and the “aspecific” increased. In group 2, there was a reduction of late patterns, a worsening of SHAQ (p < 0.005) and an improvement of RCS (p = 0.00014). In Sildenafil (63 patients: 35, group 1; 28, group 2), after 3 months, NVC patterns changed significantly in both groups(p = 0.042 group 1, p = 0.00089 group 2). In group 1, the late and early patterns increased, and the aspecific decreased. In group 2, a significant change of NVC pattern was observed also after 6 months (p = 0.00089): the late pattern increased while the active one reduced. After 6 months, SHAQ was significantly reduced in group 1 (p = 0.00027) and in group 2 (p = 0.0043). RCS improved in both groups (p = 0.0042, group 1; p = 0.0016, group 2). Combination therapy (Bosentan + Sildenafil) (31 patients: 14, group 1; 17, group 2) induced significant changes on NVC only in group 1 after 3 (p = 0.00256) and 6 months (p = 0.000349) with a reduction of the late and active patterns and an increase of the early pattern. In both groups, after 6 months, SHAQ (p < 0.05, group 1; p = 0.00049, group 2) and RCS significantly reduced (group 1, p = 0.00024; group 2, p = 0.0021). Patients treated with Bosentan + Sildenafil show a significant improvement of RCS and NVC. This combination therapy may exert a vascular activity achieving an amelioration of the structure of microvasculature in SSc.

Published
2015

141. Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Author

Blagojevic, Jelena, Bellando-Randone, Silvia, Abignano, Giuseppina, Avouac, Jérôme, Cometi, L, Czirják, László, Denton, Christopher P, Distler, Oliver, Frerix, Marc, Guiducci, Serena, Huscher, Dörte, Jaeger, Veronika K, Lóránd, Veronika, Maurer, Britta, Nihtyanova, Svetlana, Riemekasten, Gabriela, Siegert, Elise, Tarner, Ingo H, Vettori, Serena, Walker, Ulrich A, Allanore, Yannick, Müller-Ladner, Ulf, Del Galdo, Francesco, Matucci-Cerinic, Marco, EUSTAR co-workers, University of Zurich, Blagojevic, Jelena, Blagojevic, J., Bellando-Randone, S., Abignano, G., Avouac, J., Cometi, L., Czirják, L., Denton, C. P., Distler, O., Frerix, M., Guiducci, S., Huscher, D., Jaeger, V. K., Lóránd, V., Maurer, B., Nihtyanova, S., Riemekasten, G., Siegert, E., Tarner, I. H., Vettori, S., Walker, U. A., Allanore, Y., Müller-Ladner, U., Del Galdo, F., and Matucci-Cerinic, M.

Subjects
0301 basic medicine, Male, lcsh:Diseases of the musculoskeletal system, Observational Trial, 2745 Rheumatology, Digital ulcer, Categorisation, Scleroderma, Systemic sclerosi, 0302 clinical medicine, Surveys and Questionnaires, Immunology and Allergy, Prospective Studies, 10051 Rheumatology Clinic and Institute of Physical Medicine, Digital ulcers, Middle Aged, Calcium Channel Blockers, Classification, 3. Good health, Clinical Practice, Categorization, 2723 Immunology and Allergy, Systemic sclerosis, Drug Therapy, Combination, Female, Research Article, Adult, medicine.medical_specialty, Immunology, 610 Medicine & health, Sildenafil Citrate, Fingers, 03 medical and health sciences, Rheumatology, Skin Ulcer, medicine, Humans, In patient, European Union, Iloprost, 030203 arthritis & rheumatology, 2403 Immunology, Scleroderma, Systemic, business.industry, Bosentan, Essential item, medicine.disease, 030104 developmental biology, Essential items, Physical therapy, Observational study, lcsh:RC925-935, business
Abstract

Background: A consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al., J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing. The aims of this study were to identify a set of essential items for digital ulcer (DU) evaluation, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate if the new categorisation was preferred to the simple distinction of DU in recurrent and not recurrent, in patients with systemic sclerosis (SSc).Methods: DeSScipher is the largest European multicentre study on SSc. It consists of five observational trials (OTs), and one of them, OT1, is focused on DU management. The DeSScipher OT1 items on DU that reached ≥ 60% of completion rate were administered to EUSTAR (European Scleroderma Trials and Research group) centres via online survey. Questions about feasibility and usefulness of the existing DU classification (DU due to digital pitting scars, to loss of tissue, derived from calcinosis and gangrene) and newly proposed categorisation (episodic, recurrent and chronic) were also asked.Results: A total of 84/148 (56.8%) EUSTAR centres completed the questionnaire. DeSScipher items scored by ≥ 70% of the participants as essential and feasible for DU evaluation were the number of DU defined as a loss of tissue (level of agreement 92%), recurrent DU (84%) and number of new DU (74%). For 65% of the centres, the proposed classification of DU was considered useful and feasible in clinical practice. Moreover, 80% of the centres preferred the categorisation of DU in episodic, recurrent and chronic to simple distinction in recurrent/not recurrent DU.Conclusions: For clinical practice, EUSTAR centres identified only three essential items for DU evaluation and considered the proposed classification and categorisation as useful and feasible. The set of items needs to be validated while further implementation of DU classification and categorisation is warranted.Trial registration: Observational trial on DU (OT1) is one of the five trials of the DeSScipher project (ClinicalTrials.gov; OT1 Identifier: NCT01836263, posted on April 19, 2013).

Published
2018

142. 'To Be or Not To Be,' Ten Years After: Evidence for Mixed Connective Tissue Disease as a Distinct Entity

Author

Simona Rednic, L. P. Ananyeva, Olga Koneva, Katarina Simic Pasalic, Marta Mosca, László Czirják, Valeria Riccieri, Guido Valesini, Stefano Bombardieri, Susanna Cappelli, Csaba György Kiss, Maria Magdalena Tamas, Ruxandra Ionescu, Mike O Becker, Marco Matucci Cerinic, S. Cardarelli, Gabriela Riemekasten, Franco Cozzi, Nemanja Damjanov, Yannick Allanore, Rosaria Talarico, Gabriele Valentini, Silvia Bellando Randone, Giovanna Cuomo, Martin Aringer, Mislav Radić, Anne Kathrin Tausche, Alberto Sulli, Serena Guiducci, Dušanka Martinović, Maurizio Cutolo, Daniela Opris, Cappelli, S, Bellando Randone, S, Martinović, D, Tamas, Mm, Pasalić, K, Allanore, Y, Mosca, M, Talarico, R, Opris, D, Kiss, Cg, Tausche, Ak, Cardarelli, S, Riccieri, V, Koneva, O, Cuomo, Giovanna, Becker, Mo, Sulli, A, Guiducci, S, Radić, M, Bombardieri, S, Aringer, M, Cozzi, F, Valesini, G, Ananyeva, L, Valentini, Gabriele, Riemekasten, G, Cutolo, M, Ionescu, R, Czirják, L, Damjanov, N, Rednic, S, and Matucci Cerinic, M.

Subjects
Adult, Male, rheumatoid arthritis, medicine.medical_specialty, Pathology, undifferentiated connective tissue disease, systemic sclerosis, autoantibodies, overlap syndrome, systemic lupus erythematosus, mixed connective tissue disease, Connective tissue, Disease, Mixed connective tissue disease, Rheumatology, medicine, Humans, Retrospective Studies, business.industry, Autoantibody, Undifferentiated connective tissue disease, Retrospective cohort study, Overlap syndrome, medicine.disease, Dermatology, Anesthesiology and Pain Medicine, medicine.anatomical_structure, Rheumatoid arthritis, Disease Progression, Female, business, Follow-Up Studies
Abstract

OBJECTIVES: To determine if mixed connective tissue disease (MCTD) can be considered an independent clinical entity, to compare 3 different classification criteria for MCTD (Kasukawa, Alarcón-Segovia, and Sharp), and to define predictors (clinical features and autoantibodies) of potential evolution toward other connective tissue diseases (CTDs). METHODS: One hundred sixty-one MCTD patients were evaluated retrospectively at the diagnosis and in 2008. They were classified, at the diagnosis, according to the 3 classification criteria of MCTD (Sharp, Alarcón-Segovia, and Kasukawa) and reclassified in 2008 according to their evolution. Statistical analyses were performed to find out predictors (clinical features and autoantibodies) of evolution into other CTDs. RESULTS: After a mean of 7.9 years of disease, 57.9% of patients still satisfied MCTD classification criteria of Kasukawa; 17.3% evolved into systemic sclerosis, 9.1% into systemic lupus erythematosus, 2.5% into rheumatoid arthritis, 11.5% was reclassified as affected by undifferentiated connective tissue disease, and 1.7% as suffering from overlap syndrome. Kasukawa's criteria were more sensitive (75%) in comparison to those of Alarcón-Segovia (73%) and Sharp (42%). The presence of anti-DNA antibodies (P = 0.012) was associated with evolution into systemic lupus erythematosus; hypomotility or dilation of esophagus (P < 0.001); and sclerodactyly (P = 0.034) with evolution into systemic sclerosis. CONCLUSIONS: MCTD is a distinct clinical entity but it is evident that a subgroup of patients may evolve into another CTD during disease progression. Initial clinical features and autoantibodies can be useful to predict disease evolution

Published
2012

143. The B-cells paradigm in Systemic Sclerosis: an update on pathophysiology and B-cell targeted therapies.

Author

Scaletti C, Pratesi S, Bellando Randone S, Di Pietro L, Campochiaro C, Annunziato F, and Matucci Cerinic M

Abstract

Systemic sclerosis is considered a rare autoimmune disease in which there are alterations of both the innate and adaptive immune response resulting in the production of autoantibodies. Abnormalities of the immune system compromise the normal function of blood vessels leading to a vasculopathy manifested by Raynaud's phenomenon, an early sign of systemic sclerosis. As a consequence of this reactive picture, the disease can evolve leading to tissue fibrosis. Several systemic sclerosis-specific autoantibodies are currently known and are associated with specific clinical manifestations and prognosis. Although the pathogenetic role of these autoantibodies is still unclear, their production by B cells and plasma cells suggests the importance of these cells in the development of systemic sclerosis. This review narratively examines B cell dysfunctions and their role in the pathogenesis of systemic sclerosis and discusses B cell-targeted therapies currently used or potentially useful for the management of end-organ complications., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Immunology.)

Published
2024
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144. Doppler ultrasound, a noninvasive tool for the study of mesenteric arterial flow in systemic sclerosis: a cross-sectional study of a patient cohort with review and meta-analysis of the literature.

Author

Bandini G, Monami M, Ciuti G, Mercatelli P, Lo Cricchio A, De Santis MC, Bonomi F, Bellando Randone S, Campochiaro C, El Aoufy K, Ruaro B, Giuggioli D, Hughes M, McMahan ZH, Benfaremo D, Moroncini G, Maconi G, Accogli E, Dagna L, Matucci Cerinic M, and Moggi Pignone A

Abstract

Gastrointestinal involvement (GI) is a frequent and troublesome complication of systemic sclerosis (SSc), whose etiology is poorly understood, though it is hypothesized that autoimmunity and progressive vasculopathy may play a role. Vasculopathy is considered one of the main pathogenetic pathways responsible for many of the clinical manifestations of SSc, and, therefore, studying the principal splanchnic vessels (i.e., superior mesenteric artery-SMA and inferior mesenteric artery-IMA) with Doppler Ultrasound (DUS) may provide further insights into measuring the progression of vasculopathy, evaluating its possible association with SSc GI symptoms, and determining whether it plays a role in the development or severity of SSc GI disease. A cohort of SSc patients consecutively recruited underwent DUS examination, and associations with GI (UCLA-GIT 2.0 questionnaire) and extraintestinal SSc characteristics were evaluated. Semiquantitative DUS parameters (resistive index-RI and pulsatility index-PI), were applied for splanchnic vessel assessment in SSc patients and healthy subjects (HS). Moreover, a review and meta-analysis of the literature to understand which the values of the main semiquantitative DUS parameters (RI and PI) are both in SSc patients and HS has been conducted. Seventy-eight patients completed DUS examinations and clinical assessments. 30 (39%) were classified as diffuse cutaneous SSc (dcSSC), 35 (45%) as limited cutaneous SSc (lcSSc) and 13 (17%) as sine scleroderma. A significant difference was found both for SMA RI (p for trend = 0.032) and SMA PI (p for trend = 0.004) between patients with sine scleroderma, lcSSc and dcSSc, with lower values observed in the sine scleroderma and lcSSc groups. IMA RI and PI were significantly correlated with GI symptoms such as fecal incontinence (ῥ - 0.33, p = 0.008 and ῥ - 0.30, p = 0.021, respectively). By multivariate analysis, significant associations were confirmed between SMA RI and SMA PI and mRSS (β 0.248, p = 0.030 and β 2.995, p = 0.004, respectively) and with bosentan (β 0.400, p = 0.003 and β 3.508, p = 0.001, respectively), but not with anticentromere antibody (ACA). No significant differences were found between the weighted median values of SMA RI and SMA PI of SSc patients compared to those of HS that were derived from the meta-analysis of the literature (p = 0.72 and p = 0.64, respectively). This cross-sectional study confirms that the splanchnic vasculature of SSc patients can noninvasively been studied with DUS. Vascular splanchnic involvement correlates with the presence and/or severity of specific clinical features in SSc, including GI. Larger and prospective studies are needed to confirm these preliminary observations and to examine the role of DUS in SSc-risk stratification and GI progression and to obtain definitive data regarding both HS and SSc patients splanchnic DUS parameters., (© 2024. The Author(s).)

Published
2024
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145. The Role of Adipokines between Genders in the Pathogenesis of Osteoarthritis.

Author

Economou A, Mallia I, Fioravanti A, Gentileschi S, Nacci F, Bellando Randone S, Lepri G, and Guiducci S

Subjects
Humans, Male, Female, Sex Factors, Leptin metabolism, Leptin blood, Sex Characteristics, Resistin blood, Resistin metabolism, Osteoarthritis metabolism, Osteoarthritis blood, Adipokines metabolism, Adipokines blood
Abstract

Osteoarthritis (OA) is a chronic, progressive, degenerative joint disease characterized by joint pain, stiffness, and limited movement. It presents significant intra- and inter-individual variability-in particular, between genders. Recent research has increasingly focused on the role of adipokines-especially leptin, adiponectin, and resistin-in the development of OA. Adipokines, peptide hormones primarily secreted by adipose tissue, are involved in crucial physiological processes related to metabolism and immunity. They can also impact bone and cartilage turnover by interacting with joint cells such as osteoblasts, osteoclasts, chondrocytes, and mesenchymal stem cells, thereby linking inflammation with bone cartilage homeostasis. This review aims to elucidate the structure and functions of various adipokines, their serum and synovial levels, and their association with clinical presentation and radiographic progression in OA patients, with a focus on differences between sexes. A narrative literature review was conducted using three databases specifically analyzing sex differences. OA patients generally show elevated serum and synovial levels of leptin, chemerin, and visfatin, as well as high plasma levels of resistin and visfatin. In contrast, synovial levels of adiponectin and omentin are reduced in OA patients compared to healthy individuals, with an inverse relationship to disease severity, suggesting a potential protective role. Resistin and leptin were positively correlated with pain severity and radiographic progression, while adiponectin's role in OA remains controversial. Regarding sex differences, male OA patients exhibited higher serum levels of leptin, chemerin, and omentin compared to healthy controls, with a positive correlation to the BMI and estrogen levels, potentially explaining the sexual dimorphism observed in this condition. Studies on visfatin and lipocalin did not reveal significant differences in synovial or serum levels between the sexes. The role of resistin remains controversial. Adipokines influence the joint microenvironment and contribute to the progression of osteoarthritis (OA). However, the precise biological mechanisms are not yet fully understood due to the complex interactions between the metabolic, mechanical, and immune systems. Further research is needed to clarify their roles in OA and to identify targeted therapies for managing this degenerative disease.

Published
2024
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146. Evidence for telemedicine heterogeneity in rheumatic and musculoskeletal diseases care: a scoping review.

Author

El Aoufy K, Melis MR, Magi CE, Bellando-Randone S, Tamburini M, Bandini G, Moggi-Pignone A, Matucci-Cerinic M, Bambi S, and Rasero L

Subjects
Humans, Rheumatology methods, Telemedicine, Rheumatic Diseases therapy, Musculoskeletal Diseases therapy
Abstract

Telemedicine and digital health represent alternative approaches for clinical practice; indeed, its potential in healthcare services for prevention, diagnosis, treatment, rehabilitation, and disease monitoring is widely acknowledged. These are all crucial issues to consider when dealing with chronic Rheumatic and Musculoskeletal Diseases (RMDs). The aim was to determine the current state of telemedicine in the field of rheumatology, considering the tools and devices in use as well as the Patient Reported Outcomes. A scoping review was performed following the PRISMA-ScR, retrieving articles through five databases from 1990 to 2022. Inclusion criteria were as follows: (I) adult patients with RMDs, (II) original research papers in the English language with available abstracts, and (III) telehealth and telemedicine are provided as healthcare services. Within the 62 included studies, multiple tools of telemedicine were used: 21/62 websites/online platforms, 18/62 mobile applications, 16/62 telephone contacts, 5/62 video-consultations, and 1/62 wearable devices. Outcomes were classified based on the economic, clinical, and humanistic framework. Clinical outcomes assessed through digital tools were pain, disease activity, and serum uric acid levels. Humanistic outcomes have been grouped according to four categories (e.g., mental and physical function, health management, and health perception). The heterogeneity of digital tools in the field of rheumatology highlights the challenge of implementing reliable research into clinical practice. Effective telerehabilitation models have been presented, and the use of a tight control strategy has also been mentioned. Future research should focus on establishing studies on other RMDs as well as summarizing and formulating clinical guidelines for RMDs. Key Points • Evidence for the usefulness of telemedicine and digital health for managing and monitoring rheumatic and musculoskeletal diseases is progressively increasing. • Several digital tools effectively measure clinical and humanistic and patient reported outcomes in rheumatic and musculoskeletal diseases. • Integrating diverse digital tools in rheumatology is challenging yet promising. • Future research should focus on developing standardized recommendations for practical use of telemedicine in daily practice., (© 2024. The Author(s).)

Published
2024
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147. Accuracy of lung ultrasound performed with handheld ultrasound device in internal medicine: an observational study.

Author

Lo Cricchio A, Storelli A, Bertoletti I, Ciuti G, Fabbri A, Martinelli E, De Santis MC, Mercatelli P, El Aoufy K, Bellando Randone S, Moggi Pignone A, Accogli E, and Bandini G

Subjects
Humans, Male, Female, Aged, Middle Aged, Heart Failure diagnostic imaging, Reproducibility of Results, Pneumonia diagnostic imaging, Aged, 80 and over, Ultrasonography methods, Ultrasonography instrumentation, Internal Medicine, Lung diagnostic imaging
Abstract

Aims: Lung ultrasound (LUS) is increasingly used in Internal Medicine to complement medical examination, documenting pleural and lung conditions. This study aimed to compare the accuracy of handheld ultrasound device (HHUSD) with high-end ultrasound device (HEUSD) in patients with heart failure or pneumonia, also including the assessment of costs and time-savings., Methods: In this observational study 72 patients (aged ≥ 18) admitted to Internal Medicine Unit for heart failure or pneumonia underwent LUS plus evaluation of inferior cava vein (ICV) when indicated, using both HHUSD and HEUSD. Each evaluation, independently performed by 2 different experienced operators, included B-lines number, pleural effusion, lung consolidations, ICV ectasia and its respiratory excursions., Results: Concordance between HHUSD and HEUSD findings was 79.3% ± 17.7 (mean ± SD) for B-lines, 88.6% for pleural effusion, 82.3% for consolidations and 88.7% and 84.9% for ICV ectasia and its respiratory excursions respectively. BMI didn't significantly influence concordance between the two methods. Moreover, examination time (as mean ± SD) was shorter with HHUSD (8 ± 1.5 min) compared to HEUSD (10 ± 2.5 min)., Conclusions: HHUSD demonstrated high accuracy in detecting B-lines, pleural effusions, lung consolidations and ICV evaluation when compared to HEUSD. Thus, HHUSD, not only is characterized by accessibility, portability, and easy handling due to its small size, but it also offers advantages in terms of saving costs and time, ultimately contributing to faster patient assessment compared to HEUSD., (© 2024. The Author(s).)

Published
2024
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148. Towards a comprehensive approach to the management and prognosis of systemic sclerosis's patients: The role of comorbidities in the SPRING-SIR registry.

Author

Orlandi M, Bellando-Randone S, De Angelis R, Ferri C, Giuggioli D, Cacciapaglia F, Magnani L, Cuomo G, Gigante A, Codullo V, Campochiaro C, Ariani A, Foti R, Guiducci S, Matucci-Cerinic M, and Bruni C

Abstract

Objectives: The current knowledge about the role of comorbidities in systemic sclerosis (SSc) is limited. Therefore, the aim of this study was to evaluate the prevalence of comorbidities and their impact on disease activity and prognosis in the Systemic sclerosis PRogression INvestiGation (SPRING) registry., Methods: SSc patients from the SPRING registry, fulfilling the ACR/EULAR 2013 classification criteria, with complete data on baseline comorbidities were enrolled. The Charlson comorbidity index (CCI) was used to quantify the overall comorbidity burden. The disease activity was calculated using the revised EUSTAR activity index (AI). The impact of SSc features on CCI, the effect of CCI on SSc disease activity and mortality were tested with multivariable regression models., Results: Among 1910 SSc patients enrolled, 67.3 % had at least one comorbidity at baseline. The most frequent comorbidities were systemic arterial hypertension (23.7 %), osteoporosis (12.9 %) and dyslipidemia (11 %). The mean value of CCI score was 2.0 ± 1.8. When patients were grouped according to increasing levels of CCI, a clear separation in the distribution of SSc-related clinical features could be observed. Among over 900 patients with available follow-up, no association between baseline CCI and changes in disease activity was observed. Conversely, the risk of death over time was independently predicted by both CCI and AI., Conclusions: Comorbidities and disease activity independently impact on the prognosis of SSc patients. This suggests that the management of comorbidities, together with the reduction of disease activity, is fundamental to improve patient survival., Competing Interests: Declaration of competing interest Orlandi M, Bellando-Randone S, Ferri C, Foti R, Codullo V, De Angelis R, Cuomo G, Campochiaro C, Luca Magnani, Cacciapaglia F, Ariani A, Giuggioli D, and Guiducci S have no conflict of interest to declare. Matucci-Cerinic M received fees from Actelion, Janssen, Inventiva, Bayer, Biogen, Boehringer, CSL Behring, Corbus, Galapagos, Mitsubishi, Samsung, Regeneron, Acceleron, MSD, Chemomab, Lilly, Pfizer, Roche. Bruni C received consulting fees and/or honoraria from Eli-Lilly, Boehringer Ingelheim, Research grants from Gruppo Italiano Lotta alla Sclerodermia (GILS), European Scleroderma Trials and Research Group (EUSTAR), Scleroderma Clinical Trials Consortium (SCTC), Scleroderma Research Foundation (SRF), Novartis foundation for biomedical research. Congress supports from Boehringer-Ingelheim. Educational grants from AbbVie and Wellcome Trust., (Copyright © 2024. Published by Elsevier B.V.)

Published
2024
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149. The Potential Role of Butyrate in the Pathogenesis and Treatment of Autoimmune Rheumatic Diseases.

Author

Coccia C, Bonomi F, Lo Cricchio A, Russo E, Peretti S, Bandini G, Lepri G, Bartoli F, Moggi-Pignone A, Guiducci S, Del Galdo F, Furst DE, Matucci Cerinic M, and Bellando-Randone S

Abstract

The gut microbiota is a complex ecosystem of microorganisms residing in the human gastrointestinal tract, playing a crucial role in various biological processes and overall health maintenance. Dysbiosis, an imbalance in the composition and function of the gut microbiota, is linked to systemic autoimmune diseases (SAD). Short-chain fatty acids (SCFAs), especially butyrate, produced by the gut microbiota through the fermentation of dietary fibers, play a significant role in immunomodulation and maintaining intestinal homeostasis. Butyrate is essential for colonocyte energy, anti-inflammatory responses, and maintaining intestinal barrier integrity. Studies show reduced butyrate-producing bacteria in SAD patients, suggesting that increasing butyrate levels could have therapeutic benefits. Butyrate's anti-inflammatory effects and its potential therapeutic role have been studied in rheumatoid arthritis, Sjogren's syndrome, systemic lupus erythematosus, systemic sclerosis, and Behçet's disease. Despite promising in vitro and animal model results, human studies are limited, and the optimal strategies for modulating dysbiosis in SADs remain elusive. This review explores the current evidence on the immunoregulatory role of butyrate and its potential therapeutic effects in SAD.

Published
2024
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150. Systemic sclerosis and environment: an intriguing and still debated association.

Author

Lepri G, Bellando Randone S, Damiani A, Blagojevic J, and Guiducci S

Subjects
Humans, Gene-Environment Interaction, Genetic Predisposition to Disease, Risk Factors, Environmental Exposure adverse effects, Environment, Fibrosis, Scleroderma, Systemic immunology
Abstract

Systemic sclerosis (SSc) is characterised by a heterogeneous clinical expression probably reflecting the different genetic background of each patient. Progress has been made in the definition of the principal pathogenetic events of the disease that can be summarised in endothelial damage and dysfunction, inflammation with activation of immune system and fibrosis. The aetiology of the disease still remains to be clarified and probably the first events are attributable to the repeated action of environmental stimuli in genetically predisposed subjects.The aim of the present manuscript is to review the most recent and relevant data regarding the association of SSc with environmental factors.

Published
2024
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