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

Author

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

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

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

Published
2020

2. Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database

Author

Allanore Y., Bozzi S., Terlinden A., Huscher D., Amand C., Soubrane C., Siegert E., Czirjak L., Carreira P. E., Hachulla E., Zanatta E., Li M., Airo P., Mendoza F. A., Rosato E., Distler O. Svetlana Agachi, Walid Ahmed Abdel Atty Mohamed, Paolo Airò, Sabine Adler, Juan Jose Alegre-Sancho, Yannick Allanore, Lidia P. Ananieva, Codrina Ancuta, Branimir Anic, Fabiola Atzeni, Gianluigi Bajocchi, Alexandra Balbir-Gurman, Marko Baresic, Radim Becvar, Laura Belloli, Vera Bernardino, Washington Bianchi, Breno Valdetaro Bianchi, Otylia Kowal Bielecka, Francesco Paolo Cantatore, Patricia E. Carreira, Ivan Castellví, Marco Matucci Cerinic, Carlo Chizzolini, Lorinda S. Chung, Bernard Coleiro, Maura Couto, Mary Ellen Csuka, Giovanna Cuomo, Maurizio Cutolo, László Czirják, Lorenzo Dagna, Nemanja Damjanov, Ellen De Langhe, Francesco Del Galdo, Carlos de la Puente, Christopher Denton, Y., Allanore, S., Bozzi, A., Terlinden, D., Huscher, C., Amand, C., Soubrane, E., Siegert, L., Czirjak, P. E., Carreira, E., Hachulla, E., Zanatta, M., Li, P., Airo, F. A., Mendoza, E., Rosato, Svetlana Agachi, Distler O., Ahmed Abdel Atty Mohamed, Walid, Airò, Paolo, Adler, Sabine, Jose Alegre-Sancho, Juan, Allanore, Yannick, Ananieva, Lidia P., Ancuta, Codrina, Anic, Branimir, Atzeni, Fabiola, Bajocchi, Gianluigi, Balbir-Gurman, Alexandra, Baresic, Marko, Becvar, Radim, Belloli, Laura, Bernardino, Vera, Bianchi, Washington, Valdetaro Bianchi, Breno, Kowal Bielecka, Otylia, Paolo Cantatore, Francesco, Carreira, Patricia E., Castellví, Ivan, Matucci Cerinic, Marco, Chizzolini, Carlo, Chung, Lorinda S., Coleiro, Bernard, Couto, Maura, Ellen Csuka, Mary, Cuomo, Giovanna, Cutolo, Maurizio, Czirják, László, Dagna, Lorenzo, Damjanov, Nemanja, De Langhe, Ellen, Del Galdo, Francesco, de la Puente, Carlo, Denton, Christopher, Publica, and University of Zurich

Subjects
0301 basic medicine, Longitudinal study, lcsh:Diseases of the musculoskeletal system, medicine.medical_treatment, 2745 Rheumatology, HAQ-DI score, computer.software_genre, Severity of Illness Index, 0302 clinical medicine, Quality of life, Surveys and Questionnaires, Medicine and Health Sciences, Longitudinal Studies, 610 Medicine & health, skin and connective tissue diseases, Database, Mortality rate, 10051 Rheumatology Clinic and Institute of Physical Medicine, EUSTAR registry, Diffuse cutaneous systemic sclerosis, Health Assessment Questionnaire-Disability Index, HAQ-DI score, 2723 Immunology and Allergy, Disease Progression, Hemodialysis, ARTHRITIS, CLINICAL-TRIALS, Research Article, musculoskeletal diseases, medicine.medical_specialty, Diffuse cutaneous systemic sclerosis, PREDICTING MORTALITY, EUSTAR registry, CLASSIFICATION, 03 medical and health sciences, Internal medicine, SCORE, medicine, Humans, 030203 arthritis & rheumatology, 2403 Immunology, Scleroderma, Systemic, business.industry, Proportional hazards model, Diffuse cutaneous systemic sclerosi, Health Assessment Questionnaire-Disability Index, Rheumatology, Clinical trial, 030104 developmental biology, Scleroderma, Diffuse, Quality of Life, FUNCTIONAL DISABILITY, Observational study, lcsh:RC925-935, Mitoxantrone, business, computer
Abstract

BackgroundPatients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression.MethodsThis was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient’s lifetime.ResultsThe analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment, and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p p p ConclusionsHAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc.

Published
2020
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3. Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

Author

Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Lepri, G, Jaeger, Vk, Walker, Ua, Iannone, F, Cacciapaglia, F, Tomčík, M, Becvar, R, Rednic, S, Petcu, A, Szabo, I, Codullo, V, Caporali, R, Montecucco, C, Carreira, P, Ioven, B, Minier, T, Czirják, L, Chizzolini, C, Allali, D, Zanatta, E, Doria, A, Gabrielli, A, Airò, P, Lazzaroni, Mg, Radić, M, Martinovic, D, Braun-Moscovici, Y, Balbir-Gurman, A, Hunzelmann, N, Caramaschi, P, Morovic-Vergles, J, Denton, C, Santamaria, V, Heitmann, S, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Foeldvari, I, Helmus, N, Salvador, M, Stamenkovic, B, Stankovic, A, Ananieva, L, Herrick, A, Engelhart, M, De La Puente, C, Hoffmann-Vold, Am, Midtvedt, Ø, Launay, D, Sobanski, V, Riccieri, V, Opris-Belinski, D, Groseanu, L, Ionescu, R, Bojinca, M, Sunderkötter, C, Distler, J, Ingegnoli, F, van der Haecke, A, Ullman, S, Pozzi, Mr, Eyerich, K, Vanthuyne, M, Erler, A, Aringer, M, De Langhe, E, Baresic, M, Mayer, M, Anic, B, Yavuz, S, Granel, B, Popa, S, Agachi, S, Zenone, T, Mathieu, A, Vacca, A, Solanki, K, Veale, D, Loyo, E, Tineo, C, Gigante, A, Rosato, E, Oksel, F, Yagurcu, F, Tănăseanu, Cm, Visalli, E, Benenati, A, Foti, R, Ancuta, C, Dan, D, Adler, S, Villiger, P, Fathi, N, de la Peña Lefebvre PG, González Martín, J, Chatelus, E, Sibilia, J, Litinsky, I, Del Galdo, F, Ann Sakettkoo, L, Kerzberg, E, Bianchi, Wa, Bianchi, Bv, Castellví, I, Limonta, M, Rimar, D, Couto, M, Ribi, C, Spertini, F, Kahl, S, Hsu, V, Poindron, V, Meghit, K, Martin, T, Kolstad, K, Chung, L, Thiele, A, Schmeiser, T, Zdrojewski, Z, Riemekasten, G, Levy, Y, Cardoneanu, A, Burlui, A, Rezus, E, Pamuk, On, Talotta, R, Bongiovanni, S, Puttini, Ps., Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Giovanna, Cuomo, Chizzolini, Carlo, Allali, Danièle, and University of Zurich

Subjects
Adult, Male, medicine.medical_specialty, Databases, Factual, systemic sclerosis, digital ulcer, 610 Medicine & health, Disease, ddc:616.07, Logistic regression, Systemic scleroderma, Cohort Studies, Rheumatology, Risk Factors, Internal medicine, Cox proportional hazards regression, medicine, Humans, Pharmacology (medical), In patient, digital ulcers, gangrene, vasculopathy, Aged, Gangrene, Scleroderma, Systemic, business.industry, Incidence (epidemiology), Incidence, 10051 Rheumatology Clinic and Institute of Physical Medicine, food and beverages, Middle Aged, medicine.disease, Cross-Sectional Studies, Cohort, Female, business, systemic sclerosi
Abstract

Objective In patients with SSc, peripheral vasculopathy can promote critical ischaemia and gangrene. The aim of this study was to investigate the prevalence, incidence and risk factors for gangrene in the EUSTAR cohort. Methods We included patients from the EUSTAR database fulfilling the ACR 1980 or the ACR/EULAR 2013 classification criteria for SSc, with at least one visit recording data on gangrene. Centres were asked for supplementary data on traditional cardiovascular risk factors. We analysed the cross-sectional relationship between gangrene and its potential risk factors by univariable and multivariable logistic regression. Longitudinal data were analysed by Cox proportional hazards regression. Results 1757 patients were analysed (age 55.9 [14.5] years, disease duration 7.9 [10.3] years, male sex 16.7%, 24.6% diffuse cutaneous subset [dcSSc]). At inclusion, 8.9% of patients had current or previous digital gangrene, 16.1% had current digital ulcers (DUs) and 42.7% had ever had DUs (current or previous). Older age, DUs ever and dcSSc were statistically significant risk factors for gangrene in the cross-sectional multivariable model. During a median follow-up of 13.1 months, 16/771 (0.9%) patients developed gangrene. All 16 patients who developed gangrene had previously had DUs and gangrene. Further risk factors for incident gangrene were the dcSSc subset and longer disease duration. Conclusion In unselected SSc patients, gangrene occurs in about 9% of SSc patients. DUs ever and, to a lesser extent, the dcSSc subset are strongly and independently associated with gangrene, while traditional cardiovascular risk factors could not be identified as risk factors.

Published
2019

4. 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

5. 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

6. Primary systemic sclerosis heart involvement: A systematic literature review and preliminary data-driven, consensus-based WSF/HFA definition

Author

Cosimo Bruni, Maya H Buch, Daniel E Furst, Giacomo De Luca, Aleksandra Djokovic, Raluca B Dumitru, Alessandro Giollo, Marija Polovina, Alexia Steelandt, Kostantinos Bratis, Yossra Atef Suliman, Ivan Milinkovic, Anna Baritussio, Ghadeer Hasan, Anastasia Xintarakou, Yohei Isomura, George Markousis-Mavrogenis, Lorenzo Tofani, Sophie Mavrogeni, Luna Gargani, Alida LP Caforio, Carsten Tschöpe, Arsen Ristic, Karin Klingel, Sven Plein, Elijah R Behr, Yannick Allanore, Masataka Kuwana, Christopher P Denton, Dinesh Khanna, Thomas Krieg, Renzo Marcolongo, Ilaria Galetti, Elisabetta Zanatta, Francesco Tona, Petar Seferovic, Marco Matucci-Cerinic, Bruni, C., Buch, M. H., Furst, D. E., DE LUCA, Giacomo, Djokovic, A., Dumitru, R. B., Giollo, A., Polovina, M., Steelandt, A., Bratis, K., Suliman, Y. A., Milinkovic, I., Baritussio, A., Hasan, G., Xintarakou, A., Isomura, Y., Markousis-Mavrogenis, G., Tofani, L., Mavrogeni, S., Gargani, L., Caforio, A. L. P., Tschope, C., Ristic, A., Klingel, K., Plein, S., Behr, E. R., Allanore, Y., Kuwana, M., Denton, C. P., Khanna, D., Krieg, T., Marcolongo, R., Galetti, I., Zanatta, E., Tona, F., Seferovic, P., and Matucci-Cerinic, M.

Subjects
medicine.medical_specialty, Primary (chemistry), business.industry, Immunology, Original Manuscript, heart, Systematic review, Rheumatology, cardiac involvement, Immunology and Allergy, Medicine, definition, Systemic sclerosis, business, Intensive care medicine
Abstract

Introduction: Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. Methods: A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. Results: In total, 171 publications met eligibility criteria. Using the nominal group technique, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5–300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate (mKappa (95% CI) = 0.56 (0.46–1.00) for the first round and 0.55 (0.44–1.00) for the second round) and intra-rater agreement was good (mKappa (95% CI) = 0.77 (0.47–1.00)). Criterion validity showed a 78 (73–84)% correctness versus gold standard. Conclusion: A preliminary primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research.

Published
2022

7. 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

8. Screening for pulmonary arterial hypertension in systemic sclerosis: A systematic literature review

Author

Marco Matucci-Cerinic, Giacomo De Luca, Maria Grazia Lazzaroni, Cosimo Bruni, Andrea Doria, Gemma Lepri, Lorenzo Dagna, Elisabetta Zanatta, Paolo Airò, Bruni, C., De Luca, G., Lazzaroni, M. -G., Zanatta, E., Lepri, G., Airo, P., Dagna, L., Doria, A., and Matucci-Cerinic, M.

Subjects
Right heart catheterization, medicine.medical_specialty, Hypertension, Pulmonary, Algorithms, Pulmonary arterial hypertension, Screening, Systemic sclerosis, Early detection, 030204 cardiovascular system & hematology, Pulmonary arterial pressure, Pulmonary function testing, 03 medical and health sciences, 0302 clinical medicine, Serum biomarkers, Internal medicine, Internal Medicine, medicine, Humans, Mass Screening, 030212 general & internal medicine, Scleroderma, Systemic, business.industry, Gold standard (test), Predictive value, Systematic review, Echocardiography, Cardiology, business
Abstract

Pulmonary arterial hypertension (PAH) carries a high morbidity and mortality burden in Systemic Sclerosis (SSc). Therefore, PAH screening and early detection are pivotal. A systematic literature review (SLR) to search for all screening tools and modalities for SSc-PAH was performed in reference to right heart catheterization as diagnostic gold standard. Papers from 2 previously published SLRs and derived from a systematic search on Pubmed, EMBASE, Web of Science for papers published from 03/10/2017 to 31/12/2018 were manually included. A total of 199 papers were reviewed and 32 were extracted, with a low bias risk according to QUADAS2. Echocardiography, pulmonary function tests, clinical features and serum biomarkers were the most frequently tools used for screening, with different parameters combined in a variable fashion, as single item or as part of composite algorithms. Among the composite algorithms, the DETECT score, ESC/ERS 2009 or 2015 guidelines, ASIG and ITINER-air algorithms were the most commonly used in a wide range of patients. In different cohorts, DETECT and ASIG showed higher sensitivity and negative predictive value than ESC/ERS 2009. In conclusion, the literature shows echocardiography as the leading screening tool for SSc-PAH. In particular, systolic pulmonary arterial pressure (sPAP) and tricuspid regurgitation velocity (TRV), both as single items or part of composite algorithms, including also serum biomarkers, clinical and functional items, are the most frequent parameters evaluated.

Published
2020

9. Revised European Scleroderma Trials and Research Group Activity Index is the best predictor of short-term severity accrual

Author

Elisabetta Zanatta, Gabriele Valentini, Jérôme Avouac, Ulrich A. Walker, Valeria Riccieri, Florenzo Iannone, Ellen De Langhe, Paola Caramaschi, Carina Mihai, Valentina Messiniti, Otylia Kowal-Bielecka, Edoardo Rosato, Jörg H W Distler, Alessandra Vacca, Yannick Allanore, Serena Fasano, Vanessa Smith, Antonella Riccardi, Britta Maurer, Oliver Distler, Elise Siegert, Paolo Airò, Paloma García de la Peña Lefebvre, Fasano, S., Riccardi, A., Messiniti, V., Caramaschi, P., Rosato, E., Maurer, B., Smith, V., Siegert, E., De Langhe, E., Riccieri, V., Airo, P., Mihai, C., Avouac, J., Zanatta, E., Walker, U. A., Iannone, F., De La Pena Lefebvre, P. G., Distler, J. H. W., Vacca, A., Distler, O., Kowal-Bielecka, O., Allanore, Y., Valentini, G., University of Zurich, and Kowal-Bielecka, Otylia

Subjects
Male, medicine.medical_specialty, Index (economics), Multivariate analysis, Time Factors, Accrual, systemic sclerosis, 2745 Rheumatology, Immunology, 610 Medicine & health, autoimmune disease, Logistic regression, Severity of Illness Index, General Biochemistry, Genetics and Molecular Biology, Scleroderma, outcomes research, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, Predictive Value of Tests, Internal medicine, Immunology and Allergy, Medicine, Humans, autoimmune diseases, Prospective Studies, 2403 Immunology, Clinical Trials as Topic, Scleroderma, Systemic, business.industry, Vascular disease, 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, medicine.disease, Prognosis, Europe, 2723 Immunology and Allergy, Disease Progression, Female, Outcomes research, Group activity, business, Follow-Up Studies
Abstract

BackgroundThe European Scleroderma Trials and Research Group (EUSTAR) recently developed a preliminarily revised activity index (AI) that performed better than the European Scleroderma Study Group Activity Index (EScSG-AI) in systemic sclerosis (SSc).ObjectiveTo assess the predictive value for short-term disease severity accrual of the EUSTAR-AI, as compared with those of the EScSG-AI and of known adverse prognostic factors.MethodsPatients with SSc from the EUSTAR database with a disease duration from the onset of the first non-Raynaud sign/symptom ≤5 years and a baseline visit between 2003 and 2014 were first extracted. To capture the disease activity variations over time, EUSTAR-AI and EScSG-AI adjusted means were calculated. The primary outcome was disease progression defined as a Δ≥1 in the Medsger’s severity score and in distinct items at the 2-year follow-up visit. Logistic regression analysis was carried out to identify predictive factors.Results549 patients were enrolled. At multivariate analysis, the EUSTAR-AI adjusted mean was the only predictor of any severity accrual and of that of lung and heart, skin and peripheral vascular disease over 2 years.ConclusionThe adjusted mean EUSTAR-AI has the best predictive value for disease progression and development of severe organ involvement over time in SSc.

Published
2019

10. Evaluation of right ventricular function performed by 3d-echocardiography in scleroderma patients

Author

E. Pigatto, Diletta Peluso, P. Miatton, Franco Cozzi, Elisabetta Zanatta, Luigi P. Badano, Leonardo Punzi, K. Bourji, Pamela Polito, Pigatto, E, Peluso, D, Zanatta, E, Polito, P, Miatton, P, Bourji, K, Badano, L, Punzi, L, and Cozzi, F

Subjects
Adult, Male, 3d-echocardiography, Right ventricular function, Systemic sclerosis, Rheumatology, medicine.medical_specialty, lcsh:Internal medicine, Heart disease, Ventricular Dysfunction, Right, Echocardiography, Three-Dimensional, lcsh:Medicine, Logistic regression, Sensitivity and Specificity, Scleroderma, Systemic sclerosi, Predictive Value of Tests, Internal medicine, Medicine, Humans, skin and connective tissue diseases, lcsh:RC31-1245, Pathological, Aged, Pressure overload, Scleroderma, Systemic, integumentary system, business.industry, lcsh:R, Reproducibility of Results, Right ventricular function, 3d-echocardiography, Systemic sclerosis, Middle Aged, medicine.disease, medicine.anatomical_structure, Italy, Ventricle, Echocardiography, Case-Control Studies, Cardiology, Vascular resistance, Female, business
Abstract

The impairment of the right ventricle (RV) in systemic sclerosis (SSc) is usually related to pulmonary arterial hypertension (PAH). New echocardiographic techniques, such as 3-dimensional echocardiography (3DE) and 2-dimensional speckle tracking (2DSTE), allow an accurate evaluation of the RV function. The aim of this study was to evaluate the RV function using 3DE and 2DSTE in SSc patients with no history of heart disease and no PAH. Forty-five SSc patients, 42 females and 3 males, 28 with limited cutaneous SSc (lcSSc) and 17 with diffuse cutaneous SSc (dcSSc), were studied. Forty-three age- and gender-matched healthy subjects were enrolled as controls. All of them underwent a 3DE and 2DSTE ecocardiographic evaluation of the RV function. Systolic pulmonary arterial pressure (sPAP) and total pulmonary vascular resistance (tPVR) were also estimated by power doppler. RV echocardiographic parameters were compared in the different subsets of SSc patients. A statistical analysis was performed by t-test, ANOVA and multiple logistic regression. RV areas in 2DSTE and volumes in 3DE were higher and RV function parameters were reduced in SSc patients compared with controls. Also sPAP and tVPR were higher, but they did not reach pathological values. Echocardiographic alterations were more pronounced in patients with lcSSc. 3DE and 2DSTE echocardiography allowed us to detect morphological and functional alterations of the RV in a group of SSc patients with no clinical signs of heart disease and no PAH. These patients had significantly higher sPAP and tPVR than healthy controls without reporting values compatible with PAH. These data suggest that RV alterations are related to a pressure overload rather than to an intrinsic myocardial involvement in SSc.

Published
2015

11. Systemic sclerosis: state of the art on clinical practice guidelines

Author

Jeska K de Vries-Bouwstra, Jacob M van Laar, Ronald F van Vollenhoven, Els Vandecasteele, Alexandre E. Voskuyl, Charissa Frank, Tobias Alexander, Gemma Lepri, Fonseca João Eurico, Eric Hachulla, Angela Tincani, Alexis Mathian, Elisabetta Zanatta, Veronica Codullo, Alberto Sulli, Luc Mouthon, Marco Matucci-Cerinic, Vanessa Smith, Amber Vanhaecke, Gerd R Burmester, Marie Vanthuyne, Frank J. A. van den Hoogen, D. Launay, Matthias Schneider, Maurizio Cutolo, Rosaria Talarico, Yannick Allanore, Ilaria Galetti, Frédéric Houssiau, Virgil A. S. H. Dalm, Alessandra Della Rossa, Cosimo Bruni, Carlo Alberto Scirè, Ulf Mueller-Ladner, Oliver Distler, Stefano Bombardieri, Paolo Airò, Barbara Ruaro, Marta Mosca, Ana Rita Vieira, Immunology, Internal Medicine, Smith, V, Scire, C, Talarico, R, Airo, P, Alexander, T, Allanore, Y, Bruni, C, Codullo, V, Dalm, V, De Vries-Bouwstra, J, Della Rossa, A, Distler, O, Galetti, I, Launay, D, Lepri, G, Mathian, A, Mouthon, L, Ruaro, B, Sulli, A, Tincani, A, Vandecasteele, E, Vanhaecke, A, Vanthuyne, M, Van Den Hoogen, F, Van Vollenhoven, R, Voskuyl, A, Zanatta, E, Bombardieri, S, Burmester, G, Eurico, F, Frank, C, Hachulla, E, Houssiau, F, Mueller-Ladner, U, Schneider, M, Van Laar, J, Vieira, A, Cutolo, M, Mosca, M, Matucci-Cerinic, M, Scirè, Ca, Van den Hoogen, F, Voskuyl, Ae, Eurico, Fj, van Laar, Jm, and Matucci-Cerinic, M.

Subjects
ERN ReCONNET, European reference networks, clinical practice guidelines, nailfold videocapillaroscopy, systemic sclerosis, unmet needs, European reference network, Disease, INTERSTITIAL LUNG-DISEASE, RECOMMENDATIONS, DEVELOPING CRITERIA, PRACTICE PATHWAY, High morbidity, 0302 clinical medicine, Fibrosis, Medicine and Health Sciences, EXPERT CONSENSUS, Immunology and Allergy, 030212 general & internal medicine, skin and connective tissue diseases, integumentary system, Interstitial lung disease, unmet need, Clinical Practice, medicine.symptom, systemic sclerosi, clinical practice guideline, medicine.medical_specialty, Immunology, Systemic Sclerosis, NO, Pharmacological treatment, Unmet needs, 03 medical and health sciences, Rheumatology, medicine, SIMPLE CAPILLAROSCOPIC DEFINITIONS, Intensive care medicine, 030203 arthritis & rheumatology, business.industry, STEM-CELL TRANSPLANTATION, medicine.disease, Sexual dysfunction, SKIN ULCERS, FUNCTIONAL DISABILITY, clinical practice guidelines, ERN ReCONNET, European reference networks, nailfold videocapillaroscopy, systemic sclerosis, unmet needs, POINTS, business
Abstract

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains ‘Vascular & Ulcers’ (ie, non-pharmacological approach to digital ulcer), ‘PAH’ (ie, screening and treatment), ‘Treatment’ and ‘Juveniles’ (ie, evaluation of juveniles with Raynaud’s phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and non-pharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.

Published
2018

12. Dental enamel defects and screening for coeliac disease

Author

E Del Santo, P Clarich, E Zanatta, P Radovich, Alessandro Ventura, Stefano Martelossi, Martelossi, S, Zanatta, E, DEL SANTO, E, Clarich, P, Radovich, P, and Ventura, Alessandro

Subjects
medicine.medical_specialty, Adolescent, Intestinal biopsy, Population, Dentistry, Sensitivity and Specificity, Asymptomatic, Short stature, Gastroenterology, Gliadin, Coeliac disease, Cohort Studies, Internal medicine, medicine, Humans, Mass Screening, Serologic Tests, Child, Dental Enamel, education, Permanent teeth, education.field_of_study, business.industry, Incidence (epidemiology), Dental enamel, General Medicine, medicine.disease, Immunoglobulin A, Celiac Disease, stomatognathic diseases, Pediatrics, Perinatology and Child Health, medicine.symptom, business
Abstract

Specific dental enamel defects (DEDs) in permanent teeth are frequently observed in coeliac patients. We examined the permanent teeth in 6949 secondary school children living in Trieste (78% of 8724 children born between 1978 and 1982). Children with DEDs were tested for serum antigliadin antibodies (AGAs) and antiendomysium antibodies (AEAs), and those positive for serum AGAs and/or AEAs underwent intestinal biopsy. Specific DEDs were observed in 52 children (0.59% of the total population examined). Serum AGAs and/or AEAs were positive in 10 cases. Nine patients underwent intestinal biopsy (one refused) and in four cases a flat mucosa was documented (one with short stature, three completely asymptomatic). The known incidence of CD in the study area was 1:1000 before the study programme and 1:670 (an increase of 44%) after it. Dental enamel inspection may be utilized for detecting undiagnosed coeliac disease in symptom-free schoolchildren. This clinical test is probably less sensitive than serum AGA screening test, but deserves some consideration because it is cheap, easy to perform and well accepted by the population.

Published
1996
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