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1. POS0821 MENOPAUSE AND SYSTEMIC SCLEROSIS: THE LINK BETWEEN MENOPAUSAL AGE, DISEASE ONSET AND CLINICAL MANIFESTATIONS IN AN ANALYSIS OF THE SPRING-SIR REGISTRY

Author

Orlandi, M., primary, Giuggioli, D., additional, De Angelis, R., additional, Riccieri, V., additional, Ingegnoli, F., additional, Cacciapaglia, F., additional, Campochiaro, C., additional, Codullo, V., additional, Guiducci, S., additional, Bellando-Randone, S., additional, Ferri, C., additional, Matucci-Cerinic, M., additional, and Bruni, C., additional

Published
2024
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2. POS0044 DECIPHERING THE GUT MICROBIOTA OF VERY EARLY SYSTEMIC SCLEROSIS (VEDOSS) PATIENTS: A TAXONOMIC AND FUNCTIONAL CHARACTERIZATION

Author

Russo, E., primary, Lepri, G., additional, Baldi, S., additional, Fioretto, B. S., additional, Romano, E., additional, El Aoufy, K., additional, Ramazzotti, M., additional, Rosa, I., additional, Ghezzi, G., additional, Gloria, L. DI, additional, Orlandi, M., additional, Bertorello, S., additional, Bruni, C., additional, Guiducci, S., additional, Manetti, M., additional, Matucci-Cerinic, M., additional, Amedei, A., additional, and Bellando Randone, S., additional

Published
2024
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5. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology

Author

De Angelis R., Ferri C., Giuggioli D., Bajocchi G., Dagna L., Bellando-Randone S., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Lepri G., Girelli F., Riccieri V., Zanatta E., Bosello S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Cipolletta E., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Di Vico C., Gigante A., Pellagrino G., Pigatto E., Lazzaroni M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., 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., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., Rossella T., De Angelis, R, Ferri, C, Giuggioli, D, Bajocchi, G, Dagna, L, Bellando-Randone, S, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Lepri, G, 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, Cipolletta, E, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Di Vico, C, Gigante, A, Pellagrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, 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, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, S. L., Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, E., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Di Vico, C., Gigante, A., Pellagrino, G., Pigatto, E., Lazzaroni, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., 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., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., and Rossella, T.

Subjects
Scleroderma, Systemic, Settore MED/16 - REUMATOLOGIA, Epidemiology, Immunology, Systemic, Autoimmunity, Autoimmune Disease, Autoimmune Diseases, Scleroderma, Rheumatology, Immunology and Allergy, Seasons, Human
Abstract

ObjectiveTo describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.MethodsData involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets.ResultsAmong patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud’s phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1–16.5) than lcSSc (2 years, IQR 0–7), and dcSSc (1 year, IQR 0–3) (pConclusionThe ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.

Published
2023

7. POS0613 THE ROLE OF MICROBIOTA IN DIFFERENT DISEASE STAGES OF SYSTEMIC SCLEROSIS: A POSSIBLE IMPACT IN GASTROINTESTINAL TRACT DISEASE ACTIVITY?

Author

Russo, E., primary, Ghezzi, G., additional, Baldi, S., additional, Fioretto, B. S., additional, Romano, E., additional, El Aoufy, K., additional, Ramazzotti, M., additional, Rosa, I., additional, Lepri, G., additional, DI Gloria, L., additional, Bruni, C., additional, Melchiorre, D., additional, Guiducci, S., additional, Manetti, M., additional, Matucci-Cerinic, M., additional, Amedei, A., additional, and Bellando Randone, S., additional

Published
2023
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10. POS1313 EFFICACY OF IMMUNOGLOBULIN TREATMENT ON GASTROINTESTINAL, CUTANEOUS AND VASCULAR INVOLVEMENT IN SYSTEMIC SCLEROSIS: DATA FROM AN ITALIAN COHORT OF 65 PATIENTS

Author

Bonomi, F., primary, Damiani, A., additional, Coccia, C., additional, Levani, J., additional, Fiorentini, E., additional, Lepri, G., additional, Orlandi, M., additional, Bartoli, F., additional, Matucci-Cerinic, M., additional, Bellando-Randone, S., additional, and Guiducci, S., additional

Published
2023
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12. Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Author

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., Matucci-Cerinic, M., and EUSTAR co-workers

Published
2019
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15. POS0487 THE CROSSTALK OF THE SKIN-ORAL-GUT MICROBIOME AXIS IN LIMITED AND DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS

Author

Russo, E., primary, Carboni, D., additional, Baldi, S., additional, Fioretto, B., additional, Romano, E., additional, El Aoufy, K., additional, Ramazzotti, M., additional, Rosa, I., additional, Lepri, G., additional, DI Gloria, L., additional, Bruni, C., additional, Melchiorre, D., additional, Guiducci, S., additional, Manetti, M., additional, Matucci-Cerinic, M., additional, Amedei, A., additional, and Bellando Randone, S., additional

Published
2022
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17. AB0747 Oral microbiome in rheumatic diseases.What involvement?

Author

Ceccherini, M. T., primary, Bellando-Randone, S., additional, Guiducci, S., additional, Romano, E., additional, Carboni, D., additional, El Aoufy, K., additional, Lepri, G., additional, Cometi, L., additional, Tofani, L., additional, Matucci-Cerinic, M., additional, and Melchiorre, D., additional

Published
2022
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19. POS1267 LONG-TERM SURVEY STUDY OF THE IMPACT OF COVID-19 ON SYSTEMIC AUTOIMMUNE DISEASES. LOW DEATH RATE DESPITE THE INCREASED PREVALENCE OF SYMPTOMATIC INFECTION. ROLE OF PRE-EXISTING INTERSTITIAL LUNG DISEASE AND ONGOING TREATMENTS.

Author

Ferri, C., primary, Raimondo, V., additional, Gragnani, L., additional, Giuggioli, D., additional, Dagna, L., additional, Tavoni, A., additional, Ursini, F., additional, L’andolina, M., additional, Caso, F., additional, Ruscitti, P., additional, Caminiti, M., additional, Foti, R., additional, Riccieri, V., additional, Guiducci, S., additional, Pellegrini, R., additional, Zanatta, E., additional, Varcasia, G., additional, Olivo, D., additional, Gigliotti, P., additional, Cuomo, G., additional, Murdaca, G., additional, Cecchetti, R., additional, De Angelis, R., additional, Romeo, N., additional, Ingegnoli, F., additional, Cozzi, F., additional, Codullo, V., additional, Cavazzana, I., additional, Colaci, M., additional, Abignano, G., additional, De Santis, M., additional, Lubrano, E., additional, Fusaro, E., additional, Spinella, A., additional, Lumetti, F., additional, De Luca, G., additional, Bellando Randone, S., additional, Visalli, E., additional, Dal Bosco, Y., additional, Amato, G., additional, Giannini, D., additional, Bilia, S., additional, Masini, F., additional, Pellegrino, G., additional, Pigatto, E., additional, Generali, E., additional, Pagano Mariano, G., additional, Pettiti, G., additional, Zanframundo, G., additional, Brittelli, R., additional, Aiello, V., additional, Caminiti, R., additional, Scorpiniti, D., additional, Ferrari, T., additional, Campochiaro, C., additional, Brusi, V., additional, Fredi, M., additional, Moschetti, L., additional, Cacciapaglia, F., additional, Ferrari, S. M., additional, DI Cola, I., additional, Vadacca, M., additional, Lorusso, S., additional, Monti, M., additional, Lorini, S., additional, Paparo, S. R., additional, Ragusa, F., additional, Elia, G., additional, Mazzi, V., additional, Aprile, M. L., additional, Tasso, M., additional, Miccoli, M., additional, Bosello, S. L., additional, D’angelo, S., additional, Doria, A., additional, Franceschini, F., additional, Meliconi, R., additional, Matucci-Cerinic, M., additional, Iannone, F., additional, Giacomelli, R., additional, Salvarani, C., additional, Zignego, A. L., additional, Fallahi, P., additional, and Antonelli, A., additional

Published
2022
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20. Prognostic Value of Lung Ultrasound B-Lines in Systemic Sclerosis

Author

Gargani, L., Bruni, C., Romei, C., Frumento, P., Moreo, A., Agoston, G., Guiducci, S., Bellando-Randone, S., Lepri, G., Belloli, L., Della Rossa, A., Delle Sedie, A., Stagnaro, C., De Nes, M., Salvadori, S., Mosca, M., Falaschi, F., Epis, O., Picano, E., Matucci-Cerinic, M., and Gargani, Luna

Subjects
Pulmonary and Respiratory Medicine, medicine.medical_specialty, systemic sclerosis, Population, Critical Care and Intensive Care Medicine, Pulmonary function testing, B-lines, 03 medical and health sciences, 0302 clinical medicine, DLCO, Internal medicine, medicine, In patient, 030212 general & internal medicine, education, interstitial lung disease, education.field_of_study, ultrasound, business.industry, Ultrasound, Interstitial lung disease, prognosis, medicine.disease, Rheumatology, Lung ultrasound, 030228 respiratory system, Radiology, Cardiology and Cardiovascular Medicine, business
Abstract

Background A high percentage of systemic sclerosis (SSc) patients experience interstitial lung disease (ILD) during the disease course. Recent data have shown that lung ultrasound (LUS) can assess ILD by the evaluation of B-lines, the sonographic sign of pulmonary interstitial involvement. Research Question To establish the prognostic value of B-lines in a large number of patients with SSc. Study Design and Methods A total of 396 consecutive patients with SSc, who were enrolled at three Rheumatology Departments, underwent a comprehensive LUS examination on the anterolateral and posterior chest for a total of 58 scanning sites. All available clinical, imaging, and functional data were recorded. Patients were followed after enrolment to establish the prognostic role of LUS. Results The median number of B-lines was higher in patients with the diffuse cutaneous subset (44 vs 17 B-lines; P Interpretation Lung ultrasound B-lines are associated with worsening or development of pulmonary deterioration. In the near future, LUS might become part of the diagnostic and prognostic armamentarium in patients with SSc, which would allow a more sustainable and user-friendly approach to this very fragile population.

Published
2020

21. COVID-19 in Italian patients with rheumatic autoimmune systemic diseases

Author

Ferri, C., Giuggioli, D., Raimondo, V., Fallahi, P., Antonelli, A., Spinella, A., Brittelli, R., Aiello, V., Caminiti, R., L'Andolina, M., Tavoni, A., Bilia, S., Giannini, D., Mariano, G. P., Cecchetti, R., Guiducci, S., Bellando-Randone, S., Ursini, F., Brusi, V., Meliconi, R., Caminiti, M., Varcasia, G., Ferrari, T., Gigliotti, P., Pellegrini, R., Olivo, D., Colaci, M., Murdaca, G., Ferri C., Giuggioli D., Raimondo V., Fallahi P., Antonelli A., Spinella A., Brittelli R., Aiello V., Caminiti R., L'Andolina M., Tavoni A., Bilia S., Giannini D., Mariano G.P., Cecchetti R., Guiducci S., Bellando-Randone S., Ursini F., Brusi V., Meliconi R., Caminiti M., Varcasia G., Ferrari T., Gigliotti P., Pellegrini R., Olivo D., Colaci M., and Murdaca G.

Subjects
0301 basic medicine, Pediatrics, medicine.medical_specialty, rheumatoid, Population, Immunology, Arthritis, autoimmune disease, Disease, Scleroderma, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, Psoriatic arthritis, 0302 clinical medicine, Rheumatology, Pandemic, medicine, Immunology and Allergy, autoimmune diseases, scleroderma, education, Spondylitis, 030203 arthritis & rheumatology, education.field_of_study, business.industry, spondylitis, systemic, medicine.disease, arthriti, ankylosing, arthritis, psoriatic, 030104 developmental biology, spondyliti, business, Cohort study
Abstract

We followed with great interest the numerous reports published by the Annals 1–9 as regards the impact of the COVID-19 pandemic on different rheumatic autoimmune systemic diseases (ASD), including the survey study by Costa et al 9 that underlined the usefulness of telerheumatology in the management of patients with psoriatic arthritis in COVID-19 era. On the same topic, the world literature encompasses a large number of anecdotal observations, cohort studies and telephone surveys.1–15 Overall, the observed prevalence of symptomatic COVID-19, usually as mild-moderate disease, in patients with chronic arthritis was comparable to that found in the general population, while worse outcomes represented almost rare events.1–15 Conversely, increased percentages of symptomatic COVID-19 were observed in patient cohorts with connective tissue diseases, especially systemic lupus or systemic vasculitis.6 7 Of note, in ASD complicated by COVID-19, the baseline use of immune modifiers, namely, conventional synthetic (csDMARD), biological (bDMARD) and targeted synthetic disease-modifying antirheumatic drugs (tsDMARD), was not associated with worse COVID-19 disease outcomes.3–5 9 10 Almost invariably these monocentre studies focused on ASD patients’ populations from limited geographical areas.1–15 Given the heterogeneous distribution of pandemic infection within the same country, we investigated the impact of COVID-19 in ASD patients’ populations from three distinct regions of Italy (northern, Emilia Romagna; central, Tuscany; and southern, Calabria), characterised by different spread of the COVID-19 pandemic, prevalent in northern Italy with a marked gradient north-south.16 Our 6-week multicentre telephone survey of 1641 unselected patients with ASD confirmed the quite benign clinical course of COVID-19 in ASD, along with the safety of baseline use of either …

Published
2020
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22. A candidate gene study reveals association between a variant of the SRp55 splicing factor gene and systemic sclerosis

Author

Romano, E., Rosa, I., Fioretto, B.S., Kosałka-Węgiel, Joanna, Sticchi, E., Bellando-Randone, S., Manetti, M., and Matucci-Cerinic, M.

Subjects
Scleroderma, Systemic, Genotype, Serine-Arginine Splicing Factors, Immunology, Arginine, Phosphoproteins, Polymorphism, Single Nucleotide, Gene Frequency, Rheumatology, Case-Control Studies, Serine, Humans, Immunology and Allergy, Genetic Predisposition to Disease, RNA Splicing Factors, RNA, Messenger, Lung Diseases, Interstitial, Autoantibodies
Abstract

To examine the possible implication of the mRNA-binding protein serine/arginine protein 55 (SRp55, also known as SRSF6) rs2235611 single nucleotide polymorphism (SNP) in the genetic predisposition to systemic sclerosis (SSc) susceptibility and clinical phenotype.A total population of 872 white Italian individuals (414 SSc patients, 458 controls) was studied. SSc patients were assessed for limited and diffuse cutaneous subsets and the presence of autoantibodies, interstitial lung disease (ILD), and nailfold videocapillaroscopy (NVC) abnormalities. The SRp55 rs2235611 SNP was genotyped by TaqMan real-time PCR.SRp55 rs2235611 genotype distribution and allele frequency were similar in SSc and healthy controls, though a trend toward significance was observed for genotype distribution (p=0.07). The SRp55 rs2235611 AA genotype significantly influenced the predisposition to SSc (p= 0.03). The SRp55 rs2235611 A minor allele and AA genotype showed a significant risk association with susceptibility to SSc-related ILD (A allele: p=0.046; AA genotype: p=0.007). A significant association of the AA genotype with SSc late NVC pattern was also found (p=0.006). After Bonferroni correction for multiple comparisons, the risk association of the SRp55 rs2235611 AA genotype with SSc-related ILD and late NVC pattern remained significant (padj=0.049 and padj=0.042, respectively).The SRp55 rs2235611 AA genotype significantly influences the susceptibility to SSc, and specifically associates with the presence of SSc-related ILD and late NVC pattern. Further in-depth studies on the SRp55 gene locus will hopefully contribute to extend our knowledge of the genetic predisposition to major SSc-related manifestations such as pulmonary fibrosis and peripheral microvasculopathy.

Published
2021

25. POS0264 THE EMERGING ROLE OF MAGNETIC RESONANCE IMAGING IN INTERSTITIAL LUNG DISEASE IN SYSTEMIC SCLEROSIS: EVIDENCE FOR ULTRA SHORT TE AND COMPRESSED SENSING VIBE ACQUISITIONS AS PROMISING TOOLS FOR THE EVALUATION OF PARENCHYMAL ALTERATIONS

Author

Landini, N., primary, Orlandi, M., additional, Occhipinti, M., additional, Nardi, C., additional, Tofani, L., additional, Bellando-Randone, S., additional, Bruni, C., additional, Matucci-Cerinic, M., additional, Morana, G., additional, and Colagrande, S., additional

Published
2021
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27. POS0321 USE OF HYDROXYCHLOROQUINE AND SYSTEMIC SCLEROSIS: RESULTS FROM A PROSPECTIVE OBSERVATIONAL STUDY ON THE EUSTAR COHORT

Author

Bellando Randone, S., primary, Wilhalme, H., additional, Bruni, C., additional, Siegert, E., additional, Airò, P., additional, Irace, R., additional, Distler, O., additional, Doria, A., additional, Ananieva, L. P., additional, Czirják, L., additional, Denton, C., additional, Allanore, Y., additional, Riccieri, V., additional, Vacca, A., additional, Foeldvari, I., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Matucci-Cerinic, M., additional, and Furst, D., additional

Published
2021
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29. POS0317 THE PERFORMANCE OF DIFFUSING CAPACITY FOR MONOXIDE CARBON (DLCO) AND FORCED VITAL CAPACITY (FVC) IN PREDICTING THE ONSET OF SYSTEMIC SCLEROSIS (SSc)-INTERSTITIAL LUNG DISEASE (ILD) IN THE EUROPEAN SCLERODERMA TRIALS AND RESEARCH (EUSTAR) DATABASE

Author

Lepri, G., primary, Bruni, C., additional, Tofani, L., additional, Moggi Pignone, A., additional, Orlandi, M., additional, Sara, T., additional, Hughes, M., additional, Del Galdo, F., additional, Irace, R., additional, Distler, O., additional, Riccieri, V., additional, Allanore, Y., additional, Gheorghiu, A. M., additional, Siegert, E., additional, De Vries-Bouwstra, J., additional, Hachulla, E., additional, Tikly, M., additional, Damjanov, N., additional, Spertini, F., additional, Mouthon, L., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Guiducci, S., additional, Matucci-Cerinic, M., additional, Furst, D., additional, and Bellando Randone, S., additional

Published
2021
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32. POS1246 COVID-19 IN ITALIAN PATIENTS WITH RHEUMATIC AUTOIMMUNE SYSTEMIC DISEASES: RESULTS OF A NATIONWIDE SURVEY STUDY

Author

Ferri, C., primary, Giuggioli, D., additional, Raimondo, V., additional, Dagna, L., additional, Riccieri, V., additional, Zanatta, E., additional, Guiducci, S., additional, Tavoni, A., additional, Foti, R., additional, Cuomo, G., additional, De Angelis, R., additional, Cozzi, F., additional, Murdaca, G., additional, Cavazzana, I., additional, Romeo, N., additional, Codullo, V., additional, Ingegnoli, F., additional, Pellegrini, R., additional, Varcasia, G., additional, Della Rossa, A., additional, De Santis, M., additional, Abignano, G., additional, Colaci, M., additional, Caminiti, M., additional, L’andolina, M., additional, Lubrano, E., additional, Spinella, A., additional, Lumetti, F., additional, De Luca, G., additional, Bellando Randone, S., additional, Visalli, E., additional, Bilia, S., additional, Masini, F., additional, Pellegrino, G., additional, Pigatto, E., additional, Generali, E., additional, Franceschini, F., additional, Pagano Mariano, G., additional, Barsotti, S., additional, Pettiti, G., additional, Zanframundo, G., additional, Brittelli, R., additional, Aiello, V., additional, Scorpiniti, D., additional, Ferrari, T., additional, Caminiti, R., additional, Campochiaro, C., additional, Gigliotti, P., additional, Cecchetti, R., additional, Olivo, D., additional, Ursini, F., additional, Brusi, V., additional, Meliconi, R., additional, Caso, F., additional, Scarpa, R., additional, D’angelo, S., additional, Iannone, F., additional, Matucci-Cerinic, M., additional, Doria, A., additional, Miccoli, M., additional, Paparo, S. R., additional, Ragusa, F., additional, Elia, G., additional, Ferrari, S. M., additional, Fallahi, P., additional, and Antonelli, A., additional

Published
2021
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33. POS1495-HPR THE EXPERIENCE OF A RHEUMATOLOGY UNIT DURING THE COVID19 LOCKDOWN: TELEMEDICINE ALLOWS A SAFE FOLLOW UP OF PATIENTS WITH RHEUMATIC DISEASES

Author

El Aoufy, K., primary, Melis, M. R., additional, Bellando Randone, S., additional, Blagojevic, J., additional, Bartoli, F., additional, Fiori, G., additional, Nacci, F., additional, Conforti, M. L., additional, Cometi, L., additional, Bruni, C., additional, Moggi Pignone, A., additional, Rasero, L., additional, Guiducci, S., additional, and Matucci-Cerinic, M., additional

Published
2021
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34. POS1228 THE ROLE OF CHEST CT IN UNDERSTANDING INTERSTITIAL LUNG DISEASE (ILD): SYSTEMIC SCLEROSIS (SSc). VERSUS COVID-19

Author

Orlandi, M., primary, Landini, N., additional, Sambataro, G., additional, Nardi, C., additional, Bruni, C., additional, Bellando-Randone, S., additional, Denton, C., additional, Luppi, F., additional, Ruaro, B., additional, Tomassetti, S., additional, Cavigli, E., additional, Melchiorre, F., additional, Palmucci, S., additional, Guiducci, S., additional, Moggi Pignone, A., additional, Allanore, Y., additional, Bartoloni, A., additional, Confalonieri, M., additional, Cortese, G., additional, Dagna, L., additional, De Cobelli, F., additional, De Paulis, A., additional, Harari, S., additional, Khanna, D., additional, Kuwana, M., additional, Miele, V., additional, Taliani, G., additional, Hughes, M., additional, Vanchieri, C., additional, Colagrande, S., additional, and Matucci-Cerinic, M., additional

Published
2021
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36. Lung magnetic resonance imaging in systemic sclerosis: a new promising approach to evaluate pulmonary involvement and progression

Author

Gargani, L., Bruni, C., De Marchi, D., Romei, C., Guiducci, S., Bellando-Randone, S., Aquaro, G. D., Pepe, A., Neri, E., Colagrande, S., Falaschi, F., Moggi-Pignone, A., Pingitore, A., Matucci-Cerinic, M., and Gargani, Luna

Subjects
Lung Diseases, medicine.medical_specialty, Interstitial lung disease, Magnetic resonance imaging, Systemic sclerosis, Computed tomography, Appropriate use, Scleroderma, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, medicine, Humans, 030212 general & internal medicine, Lung, Magnetic Resonance Imaging, Respiratory Function Tests, Lung Diseases, Interstitial, Scleroderma, Systemic, 030203 arthritis & rheumatology, medicine.diagnostic_test, business.industry, Systemic, General Medicine, respiratory system, medicine.disease, Lung involvement, Hyperintensity, respiratory tract diseases, medicine.anatomical_structure, Radiology, Interstitial, business
Abstract

Interstitial lung disease (ILD) is frequent and highly disabling in systemic sclerosis (SSc). Magnetic resonance imaging (MRI) is not routinely used to evaluate the lung, due to poorer spatial resolution compared to high-resolution computed tomography (HRCT). We aimed to compare lung MRI signal with HRCT and evaluate the role of MRI in predicting ILD progression.Thirty SSc patients underwent lung MRI and HRCT. STIR and T1 mapping sequences were acquired before and after gadolinium injection. Patients were classified as normal (group 1 with normal HRCT and MRI), discordant (group 2 without ILD signs on HRCT but areas of hyperintensity on MRI), and abnormal (group 3 with ILD signs on HRCT and areas of hyperintensity on MRI). Patients were followed up for ILD progression.Mean STIR and T1 values were different between the three groups (p 0.0001). STIR values correlated with HRCT score (R = 0.79, p 0.0001), lung ultrasound B-lines (R = 0.73, p 0.0001), and %DLco (R = - 0.63, p = 0.0001). Nine events were recorded during a follow-up of 25 ± 20 months. Continuous STIR values were independently associated with events (HR 1.018; CI 1.005-1.031, p = 0.005). A STIR value90 ms discriminated patients at a higher risk of worsening pulmonary involvement (HR 8.80; CI 1.81-42.74; p 0.007).Lung MRI can detect SSc-related ILD, with good correlations with other ILD markers. STIR values, independently of HRCT appearance, may predict worsening lung involvement. Lung MRI, although very preliminary, is a promising tool that in a near future could help selecting patients for an early treatment of SSc-related ILD and a more appropriate use of HRCT. Key points • Lung MRI has the potential to differentiate inflammation-predominant versus fibrosis-predominant lesions, but it is not currently used in routine clinical practice to assess SSc-related ILD. • Lung MRI STIR and T1 values are significantly different between patients with and without SSc-related ILD. STIR values, independently of HRCT appearance, are also able to predict worsening lung involvement over time. • These preliminary data suggest that, in a near future, MRI could support the choice for an early treatment of SSc-related ILD, as well as a more appropriate use of HRCT.

Published
2020

40. THU0355 PARAMETRIC CARDIAC MAGNETIC RESONANCE IMAGING IDENTIFIES ARRHYTHMOGENIC SUBSTRATES IN SYSTEMIC SCLEROSIS PATIENTS

Author

Mavrogeni, S. I., primary, Gargani, L., additional, Pepe, A., additional, Monti, L., additional, Markousis-Mavrogenis, G., additional, De Santis, M., additional, Meloni, A., additional, Koutsogeorgopoulou, L., additional, Karabela, G., additional, Stavropoulos, E., additional, Katsifis, G., additional, Bratis, K., additional, Bellando Randone, S., additional, Guiducci, S., additional, Bruni, C., additional, Moggi-Pignone, A., additional, Dimitroulas, T., additional, Voulgari, P., additional, Kolovou, G., additional, Bournia, V. K., additional, Mukherjee, M., additional, Lima, J., additional, Kitas, G. D., additional, Sfikakis, P., additional, and Matucci-Cerinic, M., additional

Published
2020
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43. THU0360 EFFICACY OF A SELF-TREATMENT PROTOCOL FOR FACE AND TEMPOROMANDIBULAR JOINTS REHABILITATION IN SYSTEMIC SCLEROSIS (SSC)

Author

Passalacqua, M., primary, Foggi, C., additional, Mauro, N., additional, Tofani, L., additional, Guiducci, S., additional, Bruni, C., additional, Lepri, G., additional, Blagojevic, J., additional, El Aoufy, K., additional, Fiori, G., additional, Bartoli, F., additional, Maddali Bongi, S., additional, Mitola, M., additional, Gizduloch, M., additional, Matucci-Cerinic, M., additional, and Bellando Randone, S., additional

Published
2020
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44. AB0550 DIFFUSING CAPACITY OF THE LUNG FOR CARBON MONOXIDE (DLCO) VS FORCED VITAL CAPACITY (FVC): SYSTEMATIC LITERATURE REVIEW AND META-ANALYSIS TO EXAMINE THEIR ABILITY TO MEASURE CHANGE IN CLINICAL TRIALS IN SYSTEMIC SCLEROSIS (SSC)

Author

Bellando Randone, S., primary, Cappellini, E., additional, Nidiaci, L., additional, Lepri, G., additional, Lazzaroni, M. G., additional, Campochiaro, C., additional, Bagnato, G., additional, Sambataro, D., additional, Sambataro, G., additional, Matucci-Cerinic, M., additional, and Furst, D., additional

Published
2020
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47. Value of systolic pulmonary arterial pressure as a prognostic factor of death in the systemic sclerosis EUSTAR population

Author

Hachulla, Eric, Clerson, Pierre, Airò, Paolo, Cuomo, Giovanna, Allanore, Yannick, Caramaschi, Paola, Rosato, Edoardo, Carreira, Patricia E., Riccieri, Valeria, Sarraco, Marta, Denton, Christopher P., Riemekasten, Gabriela, Pozzi, Maria Rosa, Zeni, Silvana, Mihai, Carmen Marina, Ullman, Susanne, Distler, Oliver, Rednic, Simona, Smith, Vanessa, Walker, Ulrich A., Matucci-Cerinic, Marco, Müller-Ladner, Ulf, Launay, David, Bellando Randone, S, Guiducci, S, University of Zurich, Hachulla, Eric, Hachulla, E, Clerson, P, Airò, P, Cuomo, Giovanna, Allanore, Y, Caramaschi, P, Rosato, E, Carreira, Pe, Riccieri, V, Sarraco, M, Denton, Cp, Riemekasten, G, Pozzi, Mr, Zeni, S, Mihai, Cm, Ullman, S, Distler, O2, Rednic, S2, Smith, V2, Walker, Ua, Matucci Cerinic, M, Müller Ladner, U, Launay, D1, and on behalf of the EUSTAR co, Workers

Subjects
Male, European League Against Rheumatism Scleroderma Trial and Research, systemic sclerosis, 2745 Rheumatology, MULTICENTER, systolic pulmonary artery pressure, Blood Pressure, Systemic scleroderma, Scleroderma, Cohort Studies, Systemic sclerosi, DLCO, Risk Factors, Diffusing capacity, pulmonary hypertension, survival, systolic pulmonary arterial pressure, tricuspid regurgitant jet velocity, Medicine and Health Sciences, Medicine, 2736 Pharmacology (medical), Pharmacology (medical), education.field_of_study, 10051 Rheumatology Clinic and Institute of Physical Medicine, Pulmonary, Orvostudományok, Clinical Science, Middle Aged, Prognosis, Europe, Survival Rate, Echocardiography, Hypertension, Cardiology, SURVIVAL, Female, Adult, medicine.medical_specialty, DATABASE, Systole, Hypertension, Pulmonary, Population, Pulmonary hypertension, Survival, Systemic sclerosis, Systolic pulmonary arterial pressure, Tricuspid regurgitant jet velocity, Aged, Follow-Up Studies, Humans, Multivariate Analysis, Pulmonary Artery, Retrospective Studies, Scleroderma, Systemic, Rheumatology, 610 Medicine & health, Klinikai orvostudományok, DIAGNOSIS, Internal medicine, Risk factor, education, Survival rate, HYPERTENSION, business.industry, Systemic, medicine.disease, stomatognathic diseases, Blood pressure, EULAR SCLERODERMA TRIALS, business
Abstract

Objective. The aim of this study was to assess the prognostic value of systolic pulmonary artery pressure (sPAP) estimated by echocardiography in the multinational European League Against Rheumatism Scleroderma Trial and Research (EUSTAR) cohort. Methods. Data for patients with echocardiography documented between 1 January 2005 and 31 December 2011 were extracted from the EUSTAR database. Stepwise forward multivariable statistical Cox pulmonary hypertension analysis was used to examine the independent effect on survival of selected variables. Results. Based on our selection criteria, 1476 patients were included in the analysis; 87% of patients were female, with a mean age of 56.3 years (S.D. 13.5) and 31% had diffuse SSc. The mean duration of follow-up was 2.0 years (S.D. 1.2, median 1.9). Taking index sPAP of 50 mmHg. In a multivariable Cox model, sPAP and the diffusing capacity for carbon monoxide (DLCO) were independently associated with the risk of death [HR 1.833 (95% CI 1.035, 3.247) and HR 0.973 (95% CI 0.955, 0.991), respectively]. sPAP was an independent risk factor for death with a HR of 3.02 (95% CI 1.91, 4.78) for sPAP >= 36 mmHg. Conclusion. An estimated sPAP >36mmHg at baseline echocardiography was significantly and independently associated with reduced survival, regardless of the presence of pulmonary hypertension based on right heart catheterization.

Published
2015

48. Digital ulcers predict a worse disease course in patients with systemic sclerosis

Author

Mihai C, Landewé R, van der Heijde D, Walker UA, Constantin PI, Gherghe AM, Ionescu R, Rednic S, Allanore Y, Avouac J, Czirják L, Hachulla E, Riemekasten G, Cozzi F, Airò P, Cutolo M, Mueller-Ladner U1 Matucci-Cerinic M, Launay D, Dobrotă R, Sfrenţ-Cornăţeanu R, Zingarelli S, Pigatto E, Cuomo G, Caramaschi P, Ananieva L, Ullman S, Iversen L, Gurman AB, Braun-Moscovici Y, Carreira PE, Joven BE, Minier T, Guiducci S, Bellando-Randone S, Pellerito R, Hunzelmann N, Tarner IH, Radominski SC, de Souza Müller C, Iannone F, Henes J, Bancel DF, Damjanov N, Ostojić P, Pozzi MR, Hesselstrand R, Denton C, Krasowska D, Tikly M, Riccieri V, Cantatore FP, Corrado A, Da Silva JA, Salvador MJ, Tyndall A, Gabrielli A, Distler O, Jordan S, Heitmann S, Burkhardt H, Himsel A, Rozman B, Smith V, De Keyser F, Kalitena DM, Radic M, Filipescu I, Petcu A, Vlachoyiannopoulos P, Kucharz EJ, Widuchowska M, Kopec-Medrek M, Kotulska A, Szücs G, Stankovic A, Stamenkovic B, Selmi CF, De Santis M, Marasini B, Coleiro B, Santamaria VO, Westhovens R, Bečvář R, Novak S, Engelhart M, Meroni P, Ingegnoli F, Zeni S, Sulli A, Distler J, Yavuz S, Montecucco C, Eyerich K, Krummel-Lorenz B, Zenone T, Midtvedt Ø, Chizzolini C, Seidel M, Oleszowsky M, Üprus M, Opriş D, Groşeanu L, Bielecka OK, Antonio ZM, Szechinski J, Morović-Vergles J, Scorza R, Puppo F, Mathieu A, Anic B, Stork J, Stebbings S, Inanc M, Hasler P, von Mühlen CA, Aringer M, Popa S, Li M, Rosato E., Mihai, C, Landewé, R, van der Heijde, D, Walker, Ua, Constantin, Pi, Gherghe, Am, Ionescu, R, Rednic, S, Allanore, Y, Avouac, J, Czirják, L, Hachulla, E, Riemekasten, G, Cozzi, F, Airò, P, Cutolo, M, Mueller-Ladner U1 Matucci-Cerinic, M, Launay, D, Dobrotă, R, Sfrenţ-Cornăţeanu, R, Zingarelli, S, Pigatto, E, Cuomo, G, Caramaschi, P, Ananieva, L, Ullman, S, Iversen, L, Gurman, Ab, Braun-Moscovici, Y, Carreira, Pe, Joven, Be, Minier, T, Guiducci, S, Bellando-Randone, S, Pellerito, R, Hunzelmann, N, Tarner, Ih, Radominski, Sc, de Souza Müller, C, Iannone, F, Henes, J, Bancel, Df, Damjanov, N, Ostojić, P, Pozzi, Mr, Hesselstrand, R, Denton, C, Krasowska, D, Tikly, M, Riccieri, V, Cantatore, Fp, Corrado, A, Da Silva, Ja, Salvador, Mj, Tyndall, A, Gabrielli, A, Distler, O, Jordan, S, Heitmann, S, Burkhardt, H, Himsel, A, Rozman, B, Smith, V, De Keyser, F, Kalitena, Dm, Radic, M, Filipescu, I, Petcu, A, Vlachoyiannopoulos, P, Kucharz, Ej, Widuchowska, M, Kopec-Medrek, M, Kotulska, A, Szücs, G, Stankovic, A, Stamenkovic, B, Selmi, Cf, De Santis, M, Marasini, B, Coleiro, B, Santamaria, Vo, Westhovens, R, Bečvář, R, Novak, S, Engelhart, M, Meroni, P, Ingegnoli, F, Zeni, S, Sulli, A, Distler, J, Yavuz, S, Montecucco, C, Eyerich, K, Krummel-Lorenz, B, Zenone, T, Midtvedt, Ø, Chizzolini, C, Seidel, M, Oleszowsky, M, Üprus, M, Opriş, D, Groşeanu, L, Bielecka, Ok, Antonio, Zm, Szechinski, J, Morović-Vergles, J, Scorza, R, Puppo, F, Mathieu, A, Anic, B, Stork, J, Stebbings, S, Inanc, M, Hasler, P, von Mühlen, Ca, Aringer, M, Popa, S, Li, M, and Rosato, E.

Subjects
Epidemiology, Cardiovascular Disease, Systemic Sclerosis
Published
2016

49. 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

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., and Denton, C. P.

Subjects
SYSTEMIC scleroderma, ENDOTHELIN receptors, CALCIUM antagonists, ULCERS, DRUG efficacy
Abstract

Introduction: 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. Method: Baseline characteristics of patients enrolled in the OT1 and data regarding DU were analysed. Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Published
2020
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