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1. RESULTS OF VERA APPLICATION TO THE AP1000® PWR STARTUP

Author

Franceschini F., Salazar D., and Godfrey A.

Subjects
ap1000, casl, vera, Physics, QC1-999
Abstract

This paper describes the application of the Virtual Environment for Reactor Applications, VERA, under development by the Consortium for Advanced Simulation of LWRs (CASL) to the core physics analysis of the AP1000 PWR. The AP1000 PWR features an advanced first core with radial and axial heterogeneities which provides significant enhancements compared to traditional first cores, allowing best fuel usage and short transition to the equilibrium cycle with subsequent fuel reloads. These core advanced features can pose some challenges to the core physics tools making application of VERA to the AP1000 PWR first core especially relevant to qualify VERA performance. This paper focuses on the qualification efforts at hot zero power conditions, where Monte-Carlo reference solutions have also been established. In particular, the paper focuses on the comparison of the predictions obtained with VERA for the four AP1000 units that recently started up with the measured values. It is shown that there is excellent agreement between VERA and the key reactor physics parameters measured during the AP1000 startup.

Published
2021
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2. Evaluation of a large set of patients with Autoimmune Polyglandular Syndrome from a single reference centre in context of different classifications

Author

Gatta, E., Maltese, V., Cimino, E., Cavadini, M., Anelli, V., Di Lodovico, E., Piovani, E., Zammarchi, I., Gozzoli, G., Agosti, B., Pirola, I., Delbarba, A., Girelli, A., Buoso, C., Bambini, F., Alfieri, D., Bremi, W., Facondo, P., Lupo, R., Bezzi, F., Fredi, M., Mazzola, A. M., Gandossi, E., Saullo, M., Marini, F., Licini, M., Pezzaioli, L. C., Pini, L., Franceschini, F., Ricci, C., and Cappelli, C.

Published
2024
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5. POS1415 MATERNAL AND FETAL OUTCOMES OF PROSPECTIVELY FOLLOWED PREGNANCIES IN SYSTEMIC SCLEROSIS: THE EXPERIENCE OF A MULTIDISCIPLINARY PREGNANCY CLINIC IN 2008-2023

Author

Moschetti, L., primary, Andreoli, L., additional, Semeraro, P., additional, Pedretti, E., additional, Crisafulli, F., additional, Ramazzotto, F., additional, Nalli, C., additional, Lini, D., additional, Filippini, M., additional, Zanardini, C., additional, Fredi, M., additional, Taglietti, M., additional, Franceschini, F., additional, Tincani, A., additional, Zatti, S., additional, Airo’, P., additional, and Lazzaroni, M. G., additional

Published
2024
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8. AB1129 PATIENTS AND PHYSICIANS TREATMENT PREFERENCES FOR SYSTEMIC LUPUS ERYTHEMATOSUS: A COMPARATIVE ANALYSIS OF COMPLEMENTARY DISCRETE CHOICE EXPERIMENTS

Author

Piga, M., primary, Quartuccio, L., additional, Atzeni, F., additional, Callori, M., additional, Doria, A., additional, Emmi, G., additional, Franceschini, F., additional, Gerosa, M., additional, Mosca, M., additional, Pasqualetti, P., additional, Pelissero, R., additional, Sebastiani, G. D., additional, Conti, F., additional, and Govoni, M., additional

Published
2024
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9. POS0846 THE EVALUATION OF LOWER EXTREMITIES ARTERIAL DISEASE IN SYSTEMIC SCLEROSIS PATIENTS THROUGH RHEUMATOLOGIST-VASCULAR SURGEON MULTIDISCIPLINARY MANAGEMENT: PRELIMINARY DATA FROM AN ITALIAN SINGLE CENTRE STUDY

Author

Lazzaroni, M. G., primary, Baggi, P., additional, Moschetti, L., additional, Pedretti, E., additional, Barison, C., additional, Manzoni, E., additional, Franceschini, F., additional, Bonardelli, S., additional, and Airo’, P., additional

Published
2024
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18. Management of pregnancy in autoimmune rheumatic diseases: maternal disease course, gestational and neonatal outcomes and use of medications in the prospective Italian P-RHEUM.it study

Author

Andreoli, L, Gerardi, M, Gerosa, M, Rozza, D, Crisafulli, F, Erra, R, Lini, D, Trespidi, L, Padovan, M, Ruffilli, F, Serale, F, Cuomo, G, Raffeiner, B, Semeraro, P, Tani, C, Chimenti, M, Conigliaro, P, Hoxha, A, Nalli, C, Fredi, M, Lazzaroni, M, Filippini, M, Taglietti, M, Franceschini, F, Zatti, S, Loardi, C, Orabona, R, Ramazzotto, F, Zanardini, C, Fontana, G, Gozzoli, G, Barison, C, Bizioli, P, Caporali, R, Carrea, G, Ossola, M, Maranini, B, Silvagni, E, Govoni, M, Morano, D, Verteramo, R, Doria, A, Del Ross, T, Favaro, M, Calligaro, A, Tonello, M, Larosa, M, Zen, M, Zambon, A, Mosca, M, Zucchi, D, Elefante, E, Gori, S, Iannone, F, Anelli, M, Lavista, M, Abbruzzese, A, Fasano, C, D'Angelo, S, Cutro, M, Picerno, V, Carbone, T, Padula, A, Rovere-Querini, P, Canti, V, De Lorenzo, R, Cavallo, L, Ramoni, V, Montecucco, C, Codullo, V, Milanesi, A, Pazzola, G, Comitini, G, Marvisi, C, Salvarani, C, Epis, O, Benedetti, S, Di Raimondo, G, Gagliardi, C, Lomater, C, Crepaldi, G, Bellis, E, Bellisai, F, Garcia Gonzalez, E, Pata, A, Zerbinati, M, Urban, M, Mattioli, I, Iuliano, A, Sebastiani, G, Brucato, A, Bizzi, E, Cutolo, M, Santo, L, Tonetta, S, Landolfi, G, Carrara, G, Bortoluzzi, A, Scirè, C, Tincani, A, Andreoli, Laura, Gerardi, Maria Chiara, Gerosa, Maria, Rozza, Davide, Crisafulli, Francesca, Erra, Roberta, Lini, Daniele, Trespidi, Laura, Padovan, Melissa, Ruffilli, Francesca, Serale, Francesca, Cuomo, Giovanna, Raffeiner, Bernd, Semeraro, Paolo, Tani, Chiara, Chimenti, Maria Sole, Conigliaro, Paola, Hoxha, Ariela, Nalli, Cecilia, Fredi, Micaela, Lazzaroni, Maria Grazia, Filippini, Matteo, Taglietti, Marco, Franceschini, Franco, Zatti, Sonia, Loardi, Chiara, Orabona, Rossana, Ramazzotto, Francesca, Zanardini, Cristina, Fontana, Giulia, Gozzoli, Giorgia, Barison, Claudia, Bizioli, Paola, Caporali, Roberto Felice, Carrea, Giulia, Ossola, Manuela Wally, Maranini, Beatrice, Silvagni, Ettore, Govoni, Marcello, Morano, Danila, Verteramo, Rosita, Doria, Andrea, Del Ross, Teresa, Favaro, Maria, Calligaro, Antonia, Tonello, Marta, Larosa, Maddalena, Zen, Margherita, Zambon, Alessandra, Mosca, Marta, Zucchi, Dina, Elefante, Elena, Gori, Sabrina, Iannone, Florenzo, Anelli, Maria Grazia, Lavista, Marlea, Abbruzzese, Anna, Fasano, Carlo Giuseppe, D'Angelo, Salvatore, Cutro, Maria Stefania, Picerno, Valentina, Carbone, Teresa, Padula, Angela Anna, Rovere-Querini, Patrizia, Canti, Valentina, De Lorenzo, Rebecca, Cavallo, Ludovica, Ramoni, Véronique, Montecucco, Carlomaurizio, Codullo, Veronica, Milanesi, Alessandra, Pazzola, Giulia, Comitini, Giuseppina, Marvisi, Chiara, Salvarani, Carlo, Epis, Oscar Massimiliano, Benedetti, Sara, Di Raimondo, Giuseppina, Gagliardi, Clizia, Lomater, Claudia, Crepaldi, Gloria, Bellis, Elisa, Bellisai, Francesca, Garcia Gonzalez, Estrella, Pata, Anna Paola, Zerbinati, Martina, Urban, Maria Letizia, Mattioli, Irene, Iuliano, Annamaria, Sebastiani, Giandomenico, Brucato, Antonio Luca, Bizzi, Emanuele, Cutolo, Maurizio, Santo, Leonardo, Tonetta, Sara, Landolfi, Gianpiero, Carrara, Greta, Bortoluzzi, Alessandra, Scirè, Carlo Alberto, Tincani, Angela, Andreoli, L, Gerardi, M, Gerosa, M, Rozza, D, Crisafulli, F, Erra, R, Lini, D, Trespidi, L, Padovan, M, Ruffilli, F, Serale, F, Cuomo, G, Raffeiner, B, Semeraro, P, Tani, C, Chimenti, M, Conigliaro, P, Hoxha, A, Nalli, C, Fredi, M, Lazzaroni, M, Filippini, M, Taglietti, M, Franceschini, F, Zatti, S, Loardi, C, Orabona, R, Ramazzotto, F, Zanardini, C, Fontana, G, Gozzoli, G, Barison, C, Bizioli, P, Caporali, R, Carrea, G, Ossola, M, Maranini, B, Silvagni, E, Govoni, M, Morano, D, Verteramo, R, Doria, A, Del Ross, T, Favaro, M, Calligaro, A, Tonello, M, Larosa, M, Zen, M, Zambon, A, Mosca, M, Zucchi, D, Elefante, E, Gori, S, Iannone, F, Anelli, M, Lavista, M, Abbruzzese, A, Fasano, C, D'Angelo, S, Cutro, M, Picerno, V, Carbone, T, Padula, A, Rovere-Querini, P, Canti, V, De Lorenzo, R, Cavallo, L, Ramoni, V, Montecucco, C, Codullo, V, Milanesi, A, Pazzola, G, Comitini, G, Marvisi, C, Salvarani, C, Epis, O, Benedetti, S, Di Raimondo, G, Gagliardi, C, Lomater, C, Crepaldi, G, Bellis, E, Bellisai, F, Garcia Gonzalez, E, Pata, A, Zerbinati, M, Urban, M, Mattioli, I, Iuliano, A, Sebastiani, G, Brucato, A, Bizzi, E, Cutolo, M, Santo, L, Tonetta, S, Landolfi, G, Carrara, G, Bortoluzzi, A, Scirè, C, Tincani, A, Andreoli, Laura, Gerardi, Maria Chiara, Gerosa, Maria, Rozza, Davide, Crisafulli, Francesca, Erra, Roberta, Lini, Daniele, Trespidi, Laura, Padovan, Melissa, Ruffilli, Francesca, Serale, Francesca, Cuomo, Giovanna, Raffeiner, Bernd, Semeraro, Paolo, Tani, Chiara, Chimenti, Maria Sole, Conigliaro, Paola, Hoxha, Ariela, Nalli, Cecilia, Fredi, Micaela, Lazzaroni, Maria Grazia, Filippini, Matteo, Taglietti, Marco, Franceschini, Franco, Zatti, Sonia, Loardi, Chiara, Orabona, Rossana, Ramazzotto, Francesca, Zanardini, Cristina, Fontana, Giulia, Gozzoli, Giorgia, Barison, Claudia, Bizioli, Paola, Caporali, Roberto Felice, Carrea, Giulia, Ossola, Manuela Wally, Maranini, Beatrice, Silvagni, Ettore, Govoni, Marcello, Morano, Danila, Verteramo, Rosita, Doria, Andrea, Del Ross, Teresa, Favaro, Maria, Calligaro, Antonia, Tonello, Marta, Larosa, Maddalena, Zen, Margherita, Zambon, Alessandra, Mosca, Marta, Zucchi, Dina, Elefante, Elena, Gori, Sabrina, Iannone, Florenzo, Anelli, Maria Grazia, Lavista, Marlea, Abbruzzese, Anna, Fasano, Carlo Giuseppe, D'Angelo, Salvatore, Cutro, Maria Stefania, Picerno, Valentina, Carbone, Teresa, Padula, Angela Anna, Rovere-Querini, Patrizia, Canti, Valentina, De Lorenzo, Rebecca, Cavallo, Ludovica, Ramoni, Véronique, Montecucco, Carlomaurizio, Codullo, Veronica, Milanesi, Alessandra, Pazzola, Giulia, Comitini, Giuseppina, Marvisi, Chiara, Salvarani, Carlo, Epis, Oscar Massimiliano, Benedetti, Sara, Di Raimondo, Giuseppina, Gagliardi, Clizia, Lomater, Claudia, Crepaldi, Gloria, Bellis, Elisa, Bellisai, Francesca, Garcia Gonzalez, Estrella, Pata, Anna Paola, Zerbinati, Martina, Urban, Maria Letizia, Mattioli, Irene, Iuliano, Annamaria, Sebastiani, Giandomenico, Brucato, Antonio Luca, Bizzi, Emanuele, Cutolo, Maurizio, Santo, Leonardo, Tonetta, Sara, Landolfi, Gianpiero, Carrara, Greta, Bortoluzzi, Alessandra, Scirè, Carlo Alberto, and Tincani, Angela

Abstract

Objectives To investigate pregnancy outcomes in women with autoimmune rheumatic diseases (ARD) in the Italian prospective cohort study P-RHEUM.it. Methods Pregnant women with different ARD were enrolled for up to 20 gestational weeks in 29 Rheumatology Centres for 5 years (2018-2023). Maternal and infant information were collected in a web-based database. Results We analysed 866 pregnancies in 851 patients (systemic lupus erythematosus was the most represented disease, 19.6%). Maternal disease flares were observed in 135 (15.6%) pregnancies. 53 (6.1%) pregnancies were induced by assisted reproduction techniques, 61 (7%) ended in miscarriage and 11 (1.3%) underwent elective termination. Obstetrical complications occurred in 261 (30.1%) pregnancies, including 2.3% pre-eclampsia. Two cases of congenital heart block were observed out of 157 pregnancies (1.3%) with anti-Ro/SSA. Regarding treatments, 244 (28.2%) pregnancies were treated with glucocorticoids, 388 (44.8%) with hydroxychloroquine, 85 (9.8%) with conventional synthetic disease-modifying anti-rheumatic drugs and 122 (14.1%) with biological disease-modifying anti-rheumatic drugs. Live births were 794 (91.7%), mostly at term (84.9%); four perinatal deaths (0.5%) occurred. Among 790 newborns, 31 (3.9%) were small-for-gestational-age and 169 (21.4%) had perinatal complications. Exclusive maternal breast feeding was received by 404 (46.7%) neonates. The Edinburgh Postnatal Depression Scale was compiled by 414 women (52.4%); 89 (21.5%) scored positive for emotional distress. Conclusions Multiple factors including preconception counselling and treat-to-target with pregnancy-compatible medications may have contributed to mitigate disease-related risk factors, yielding limited disease flares, good pregnancy outcomes and frequency of complications which were similar to the Italian general obstetric population. Disease-specific issues need to be further addressed to plan preventative measures.

Published
2024

19. Similarities and differences between younger and older disease onset patients with newly diagnosed systemic lupus erythematosus

Author

Prevete, I, Iuliano, A, Cauli, A, Piga, M, Iannone, F, Coladonato, L, Bortoluzzi, A, Silvagni, E, Tani, C, Elefante, E, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Frediani, B, Garcia, E, Scire, C, Zanetti, A, Rozza, D, Carrara, G, Sebastiani, G, Prevete I., Iuliano A., Cauli A., Piga M., Iannone F., Coladonato L., Bortoluzzi A., Silvagni E., Tani C., Elefante E., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Frediani B., Garcia E. G., Scire C. A., Zanetti A., Rozza D., Carrara G., Sebastiani G. D., Prevete, I, Iuliano, A, Cauli, A, Piga, M, Iannone, F, Coladonato, L, Bortoluzzi, A, Silvagni, E, Tani, C, Elefante, E, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Frediani, B, Garcia, E, Scire, C, Zanetti, A, Rozza, D, Carrara, G, Sebastiani, G, Prevete I., Iuliano A., Cauli A., Piga M., Iannone F., Coladonato L., Bortoluzzi A., Silvagni E., Tani C., Elefante E., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Frediani B., Garcia E. G., Scire C. A., Zanetti A., Rozza D., Carrara G., and Sebastiani G. D.

Abstract

Objective Several studies show that age at onset has an impact on the clinical-serological presentation, comorbidities and disease course of patients with systemic lupus erythematosus (SLE). We evaluated whether, in patients with recent onset SLE, the age at onset correlates with clinical-serological manifestations and with comorbidities. Methods We analysed 171 patients with a SLE diagnosis obtained within 12 months of diagnosis enrolled in the Early Lupus project. Based on the age of onset of the first disease symptom, they were stratified into 2 groups: early onset (18–45 years) and late onset (>45 years). The analysis was replicated by stratifying patients based on age at diagnosis (fulfillment of ACR classification criteria). Each comparison was made at baseline and at 36 months of follow-up. Results Baseline: patients with late onset displayed comorbidities (hypertension, dyslipidaemia and osteoporosis) more frequently than early onset group. 11.4% of late onset patients had a malignancy in medical history, not recorded in the early onset cohort. The two groups differed neither in organ involvement (domain BILAG) nor in disease activity (ECLAM). Patients with early onset showed a disease with signs of higher serologic activity (higher frequency of anti-dsDNA positivity and lower mean C3 and C4 levels) and had malar rash more frequently than the late onset group (36.2% vs. 18.2%, p=0.042). Similar results were obtained by stratifying patients by age of diagnosis (18-45 years and >45 years), except for the higher frequency of discoid rash in the group with age at diagnosis >45 years (18% vs. 6.6%, p=0.045). 36 months: the 2 groups of patients independently of the stratification applied did not differ in the accumulation of damage, but showed a different pattern of 8 organ involvement. Musculoskeletal involvement was more frequent both in the late onset group (18.6% vs. 7.3%, p=0.043) and in the group with age at diagnosis >45 years (20.4% vs. 5.9%, p

Published
2023

20. 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, 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., 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.

Abstract

Objective To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort. Methods Data 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. Results Among 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) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001). Conclusion The 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

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

Author

Perricone, C, Scarsi, M, Brucato, A, Pisano, P, Pigatto, E, Becattini, C, Cingolani, A, Tiso, F, Prota, R, Tomasoni, L, Cutolo, M, Tardella, M, Rozza, D, Zerbino, C, Andreoni, M, Poletti, V, Bartoloni, E, Gerli, R, Cafaro, G, Mendeni, M, Colombo, E, Medico, M, Cabras, P, Schiesaro, M, Franco, L, Fantoni, M, Friso, L, Gallo, V, Franceschini, F, Paolino, S, Salaffi, F, Scire, C, Zanetti, A, Diana, C, Passaro, A, Foti, R, Serino, F, Cassol, M, Bucaneve, G, Rocchi, R, Perricone C., Scarsi M., Brucato A., Pisano P., Pigatto E., Becattini C., Cingolani A., Tiso F., Prota R., Tomasoni L. R., Cutolo M., Tardella M., Rozza D., Zerbino C., Andreoni M., Poletti V., Bartoloni E., Gerli R., Cafaro G., Mendeni M., Colombo E., Medico M. D., Cabras P., Schiesaro M. G., Franco L., Fantoni M., Friso L., Gallo V., Franceschini F., Paolino S., Salaffi F., Scire C. A., Zanetti A., Diana C., Passaro A., Foti R., Serino F. S., Cassol M., Bucaneve G., Rocchi R. E., Perricone, C, Scarsi, M, Brucato, A, Pisano, P, Pigatto, E, Becattini, C, Cingolani, A, Tiso, F, Prota, R, Tomasoni, L, Cutolo, M, Tardella, M, Rozza, D, Zerbino, C, Andreoni, M, Poletti, V, Bartoloni, E, Gerli, R, Cafaro, G, Mendeni, M, Colombo, E, Medico, M, Cabras, P, Schiesaro, M, Franco, L, Fantoni, M, Friso, L, Gallo, V, Franceschini, F, Paolino, S, Salaffi, F, Scire, C, Zanetti, A, Diana, C, Passaro, A, Foti, R, Serino, F, Cassol, M, Bucaneve, G, Rocchi, R, Perricone C., Scarsi M., Brucato A., Pisano P., Pigatto E., Becattini C., Cingolani A., Tiso F., Prota R., Tomasoni L. R., Cutolo M., Tardella M., Rozza D., Zerbino C., Andreoni M., Poletti V., Bartoloni E., Gerli R., Cafaro G., Mendeni M., Colombo E., Medico M. D., Cabras P., Schiesaro M. G., Franco L., Fantoni M., Friso L., Gallo V., Franceschini F., Paolino S., Salaffi F., Scire C. A., Zanetti A., Diana C., Passaro A., Foti R., Serino F. S., Cassol M., Bucaneve G., and Rocchi R. E.

Abstract

Objective: To evaluate whether the addition of colchicine to standard of care (SOC) results in better outcomes in hospitalized patients with COVID-19. Design: This interventional, multicenter, randomized, phase 2 study, evaluated colchicine 1.5 mg/day added to SOC in hospitalized COVID-19 patients (COLVID-19 trial) and 227 patients were recruited. The primary outcome was the rate of critical disease in 30 days defined as need of mechanical ventilation, intensive care unit (ICU), or death. Results: 152 non-anti-SARS-CoV-2-vaccinated patients (colchicine vs controls: 77vs75, mean age 69.1±13.1 vs 67.9±15 years, 39% vs 33.3% females, respectively) were analyzed. There was no difference in co-primary end-points between patients treated with colchicine compared to controls (mechanical ventilation 5.2% vs 4%, ICU 1.3% vs 5.3%, death 9.1% vs 6.7%, overall 11 (14.3%) vs 10 (13.3%) patients, P=ns, respectively). Mean time to discharge was similar (colchicine vs controls 14.1±10.4 vs 14.7±8.1 days). Older age (>60 years, P=0.025), P/F<275 mmHg (P=0.005), AST>40 U/L (P<0.001), pre-existent heart (P=0.02), lung (P=0.003), upper-gastrointestinal (P=0.014), lower-gastrointestinal diseases (P=0.009) and cancer (P=0.008) were predictive of achieving the primary outcome. Diarrhoea (9.1% vs 0%, p=0.0031) and increased levels of AST at 6 days (76.9±91.8 vs 33.5±20.7 U/l, P=0.016) were more frequent in the colchicine group. Conclusion: Colchicine did not reduce the rate and the time to the critical stage. Colchicine was relatively safe although adverse hepatic effects require caution. We confirm that older (>60 years) patients with comorbidities are characterized by worse outcome.

Published
2023

24. Evaluation of a large set of patients with Autoimmune Polyglandular Syndrome from a single reference centre in context of different classifications

Author

Gatta, E., primary, Maltese, V., additional, Cimino, E., additional, Cavadini, M., additional, Anelli, V., additional, Di Lodovico, E., additional, Piovani, E., additional, Zammarchi, I., additional, Gozzoli, G., additional, Agosti, B., additional, Pirola, I., additional, Delbarba, A., additional, Girelli, A., additional, Buoso, C., additional, Bambini, F., additional, Alfieri, D., additional, Bremi, W., additional, Facondo, P., additional, Lupo, R., additional, Bezzi, F., additional, Fredi, M., additional, Mazzola, A. M., additional, Gandossi, E., additional, Saullo, M., additional, Marini, F., additional, Licini, M., additional, Pezzaioli, L. C., additional, Pini, L., additional, Franceschini, F., additional, Ricci, C., additional, and Cappelli, C., additional

Published
2023
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27. 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

28. The AutoInflammatory Diseases Alliance Registry of monogenic autoinflammatory diseases

Author

Gaggiano, C, Vitale, A, Tufan, A, Ragab, G, Aragona, E, Wiesik-Szewczyk, E, Ait-Idir, D, Conti, G, Iezzi, L, Maggio, Mc, Cattalini, M, La Torre, F, Lopalco, G, Verrecchia, E, de Paulis, A, Sahin, A, Insalaco, A, Sfikakis, Pp, Marino, A, Frassi, M, Ogunjimi, B, Opris-Belinski, D, Parronchi, P, Emmi, G, Shahram, F, Ciccia, F, Piga, M, Hernández-Rodríguez, J, Pereira, Rmr, Alessio, M, Naddei, R, Olivieri, An, Giudice, Ed, Sfriso, P, Ruscitti, P, Gobbi, Fl, Kucuk, H, Sota, J, Hussein, Ma, Malizia, G, Jahnz-Różyk, K, Sari-Hamidou, R, Romeo, M, Ricci, F, Cardinale, F, Iannone, F, Casa, Fd, Natale, Mf, Laskari, K, Giani, T, Franceschini, F, Sabato, V, Yildirim, D, Caggiano, V, Hegazy, Mt, Marzo, Rd, Kucharczyk, A, Khellaf, G, Tarsia, M, Almaghlouth, Ia, Laymouna, Ah, Mastrorilli, V, Dotta, L, Benacquista, L, Grosso, S, Crisafulli, F, Parretti, V, Giordano, Hf, Mahmoud, Aaa, Nuzzolese, R, Musso, M, Chighizola, Cb, Gentileschi, S, Morrone, M, Cola, Id, Spedicato, V, Giardini, Ham, Vasi, I, Renieri, A, Fabbiani, A, Mencarelli, Ma, Frediani, B, Balistreri, A, Tosi, Gm, Fabiani, C, Lidar, M, Rigante, D, Cantarini, L, Gaggiano C, Vitale A, Tufan A, Ragab G, Aragona E, Wiesik-Szewczyk E, Ait-Idir D, Iezzi L, Maggio MC, Cattalini M, La Torre F, Lopalco G, Verrecchia E, de Paulis A, Sahin A, Insalaco A, Sfikakis PP, Marino A, Frassi M, Ogunjimi B, Opris-Belinski D, Parronchi P, Emmi G, Shahram F, Ciccia F, Piga M, Hernández-Rodríguez J, Pereira RMR, Alessio M, Naddei R, Olivieri AN, Giudice ED, Sfriso P, Ruscitti P, Gobbi FL, Kucuk H, Sota J, Hussein MA, Malizia G, Jahnz-Różyk K, Sari-Hamidou R, Romeo M, Ricci F, Cardinale F, Iannone F, Casa FD, Natale MF, Laskari K, Giani T, Franceschini F, Sabato V, Yildirim D, Caggiano V, Hegazy MT, Marzo RD, Kucharczyk A, Khellaf G, Tarsia M, Almaghlouth IA, Laymouna AH, Mastrorilli V, Dotta L, Benacquista L, Grosso S, Crisafulli F, Parretti V, Giordano HF, Mahmoud AAA, Nuzzolese R, Musso M, Chighizola CB, Gentileschi S, Morrone M, Cola ID, Spedicato V, Giardini HAM, Vasi I, Renieri A, Fabbiani A, Mencarelli MA, Frediani B, Balistreri A, Tosi GM, Fabiani C, Lidar M, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, Conti G, Gaggiano, C, Vitale, A, Tufan, A, Ragab, G, Aragona, E, Wiesik-Szewczyk, E, Ait-Idir, D, Conti, G, Iezzi, L, Maggio, Mc, Cattalini, M, La Torre, F, Lopalco, G, Verrecchia, E, de Paulis, A, Sahin, A, Insalaco, A, Sfikakis, Pp, Marino, A, Frassi, M, Ogunjimi, B, Opris-Belinski, D, Parronchi, P, Emmi, G, Shahram, F, Ciccia, F, Piga, M, Hernández-Rodríguez, J, Pereira, Rmr, Alessio, M, Naddei, R, Olivieri, An, Giudice, Ed, Sfriso, P, Ruscitti, P, Gobbi, Fl, Kucuk, H, Sota, J, Hussein, Ma, Malizia, G, Jahnz-Różyk, K, Sari-Hamidou, R, Romeo, M, Ricci, F, Cardinale, F, Iannone, F, Casa, Fd, Natale, Mf, Laskari, K, Giani, T, Franceschini, F, Sabato, V, Yildirim, D, Caggiano, V, Hegazy, Mt, Marzo, Rd, Kucharczyk, A, Khellaf, G, Tarsia, M, Almaghlouth, Ia, Laymouna, Ah, Mastrorilli, V, Dotta, L, Benacquista, L, Grosso, S, Crisafulli, F, Parretti, V, Giordano, Hf, Mahmoud, Aaa, Nuzzolese, R, Musso, M, Chighizola, Cb, Gentileschi, S, Morrone, M, Cola, Id, Spedicato, V, Giardini, Ham, Vasi, I, Renieri, A, Fabbiani, A, Mencarelli, Ma, Frediani, B, Balistreri, A, Tosi, Gm, Fabiani, C, Lidar, M, Rigante, D, Cantarini, L, Gaggiano C, Vitale A, Tufan A, Ragab G, Aragona E, Wiesik-Szewczyk E, Ait-Idir D, Iezzi L, Maggio MC, Cattalini M, La Torre F, Lopalco G, Verrecchia E, de Paulis A, Sahin A, Insalaco A, Sfikakis PP, Marino A, Frassi M, Ogunjimi B, Opris-Belinski D, Parronchi P, Emmi G, Shahram F, Ciccia F, Piga M, Hernández-Rodríguez J, Pereira RMR, Alessio M, Naddei R, Olivieri AN, Giudice ED, Sfriso P, Ruscitti P, Gobbi FL, Kucuk H, Sota J, Hussein MA, Malizia G, Jahnz-Różyk K, Sari-Hamidou R, Romeo M, Ricci F, Cardinale F, Iannone F, Casa FD, Natale MF, Laskari K, Giani T, Franceschini F, Sabato V, Yildirim D, Caggiano V, Hegazy MT, Marzo RD, Kucharczyk A, Khellaf G, Tarsia M, Almaghlouth IA, Laymouna AH, Mastrorilli V, Dotta L, Benacquista L, Grosso S, Crisafulli F, Parretti V, Giordano HF, Mahmoud AAA, Nuzzolese R, Musso M, Chighizola CB, Gentileschi S, Morrone M, Cola ID, Spedicato V, Giardini HAM, Vasi I, Renieri A, Fabbiani A, Mencarelli MA, Frediani B, Balistreri A, Tosi GM, Fabiani C, Lidar M, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, and Conti G

Abstract

Objective: The present manuscript aims to describe an international, electronic-based, user-friendly and interoperable patient registry for monogenic autoinflammatory diseases (mAIDs), developed in the contest of the Autoinflammatory Diseases Alliance (AIDA) Network. Methods: This is an electronic platform, based on the Research Electronic Data Capture (REDCap) tool, used for real-world data collection of demographics, clinical, laboratory, instrumental and socioeconomic data of mAIDs patients. The instrument has flexibility, may change over time based on new scientific acquisitions, and communicate potentially with other similar registries; security, data quality and data governance are corner stones of the platform. Results: AIDA project will share knowledge and expertise on mAIDs. Since its start, 118 centers from 24 countries and 4 continents have joined the AIDA project. Fifty-nine centers have already obtained the approval from their local Ethics Committees. Currently, the platform counts 337 users (122 Principal Investigators, 210 Site Investigators, 2 Lead Investigators, and 3 data managers). The Registry collects baseline and follow-up data using 3,748 fields organized into 21 instruments, which include demographics, patient history, symptoms, trigger/risk factors, therapies, and healthcare information for mAIDs patients. Conclusions: The AIDA mAIDs Registry, acts both as a research tool for future collaborative real-life studies on mAIDs and as a service to connect all the figures called to participate. On this basis, the registry is expected to play a pivotal role in generating new scientific evidence on this group of rare diseases, substantially improving the management of patients, and optimizing the impact on the healthcare system. NCT 05200715 available at https://clinicaltrials.gov

Published
2022

29. Glucocorticoid tapering and associated outcome in patients with newly diagnosed systemic lupus erythematosus: the real-world GULP prospective observational study

Author

Floris, A, Chessa, E, Sebastiani, G, Prevete, I, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Bellisai, F, D'Alessandro, R, Zanetti, A, Carrara, G, Scire, C, Cauli, A, Piga, M, Floris A., Chessa E., Sebastiani G. D., Prevete I., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Bellisai F., D'Alessandro R., Zanetti A., Carrara G., Scire C. A., Cauli A., Piga M., Floris, A, Chessa, E, Sebastiani, G, Prevete, I, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Bellisai, F, D'Alessandro, R, Zanetti, A, Carrara, G, Scire, C, Cauli, A, Piga, M, Floris A., Chessa E., Sebastiani G. D., Prevete I., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Bellisai F., D'Alessandro R., Zanetti A., Carrara G., Scire C. A., Cauli A., and Piga M.

Abstract

Objective A subanalysis of the multicentre Early Lupus inception cohort was performed to investigate the real-world Glucocorticoids (GCs) Use in newly diagnosed systemic lupus erythematosus (SLE) Patients (GULP). Methods Patients starting prednisone (PDN) ≥5 mg/day and concomitant hydroxychloroquine or immunosuppressant within 12 months of SLE classification were enrolled. Core set variables were recorded at baseline and every 6 months, including changes in PDN dose, European Consensus Lupus Activity Measurement (ECLAM) and Systemic Lupus International Collaborating Clinics damage index. Regression models analysed predictors of tapering PDN<5 mg/day at any time and outcomes associated with different patterns of GCs tapering. Results The GULP study included 127 patients with SLE; 73 (57.5%) tapered and maintained PDN <5 mg/ day, and 17 (13.4%) discontinued PDN within a 2-year follow-up. Renal involvement (HR: 0.41; p=0.009) and lower C3 serum levels (HR: 1.04; p=0.025) predicted a lack of PDN tapering below 5 mg/day. High ECLAM scores were associated with a greater probability of increasing PDN dose (OR: 1.6; p=0.004), independently of daily intake. Disease relapse rate did not statistically differ (p=0.706) between patients tapering PDN <5 mg/day (42/99, 42.4%) and those tapering PDN without dropping below 5 mg/day (13/28, 46.4%). Every month on PDN <5 mg/day associated with lower damage accrual (IRR: 0.96; p=0.007), whereas never tapering PDN <5 mg/day associated with a higher risk of developing GC-related damage (OR 5.9; p=0.014). Conclusion Tapering PDN <5 mg/day was achieved and maintained in half of newly diagnosed patients with SLE and may represent a good balance between the need to prevent damage accrual and the risk of disease relapse.

Published
2022

30. 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

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, 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., 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. G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Calabrese F., 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., 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, 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, 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., 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. G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Calabrese F., 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., Doria A., Iannone F., Salvarani C., and Matucci-Cerinic M.

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

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

Author

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, 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. A. P., Scire C. A., 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. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., Gonzalez-Gay M. A., 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, 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. A. P., Scire C. A., 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. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., and Gonzalez-Gay M. A.

Abstract

Objective 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. Conclusion 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

32. AB0771 FEMALE GENDER AND STEROID TREATMENT AS MAIN INFLUENCING FACTORS IN THE PERCEPTION OF QUALITY OF LIFE IN ANCA-ASSOCIATED VASCULITIS: FINAL RESULTS FROM THE ITALIAN VERSION OF ANCA-ASSOCIATED VASCULITIS PATIENT-REPORTED OUTCOME (AAV-PRO_ITA) QUESTIONNAIRE

Author

Treppo, E., primary, Isola, M., additional, De Martino, M., additional, Padoan, R., additional, Urban, M. L., additional, Monti, S., additional, Sartorelli, S., additional, Giollo, A., additional, Argolini, L. M., additional, Marvisi, C., additional, Gattamelata, A., additional, Regola, F., additional, Ferro, F., additional, Cassone, G., additional, Motta, F., additional, Berti, A., additional, Conticini, E., additional, Guiducci, S., additional, Matucci-Cerinic, M., additional, Lo Gullo, A., additional, Manfredi, A., additional, Frediani, B., additional, Bortolotti, R., additional, Selmi, C., additional, Baldini, C., additional, Franceschini, F., additional, Conti, F., additional, Emmi, G., additional, Caporali, R., additional, Rossini, M., additional, Dagna, L., additional, Montecucco, C., additional, Schiavon, F., additional, Salvarani, C., additional, De Vita, S., additional, and Quartuccio, L., additional

Published
2023
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35. POS1176 HOSPITALIZATION RATES, FEATURES, AND DISCHARGE DIAGNOSES OF A LARGE NATIONWIDE COHORT OF ANCA-ASSOCIATED VASCULITIS

Author

Berti, A., primary, Ottone, M., additional, Sartorelli, S., additional, Treppo, E., additional, Bettiol, A., additional, Padoan, R., additional, Regola, F., additional, Monti, S., additional, Marvisi, C., additional, Giollo, A., additional, Argolini, L. M., additional, Righini, M., additional, Gattamelata, A., additional, Cassone, G., additional, Sottini, L., additional, Maule, M., additional, Toniati, P., additional, Palermo, B. L., additional, Bello, F., additional, Guella, S., additional, Izzo, R., additional, Muratore, F., additional, Catanoso, M. G., additional, Fassio, A., additional, Cataleta, P., additional, Buscaroli, A., additional, Giorgi Rossi, P., additional, Franceschini, F., additional, Caporali, R., additional, Montecucco, C., additional, Conti, F., additional, Emmi, G., additional, Quartuccio, L., additional, Paolazzi, G., additional, Dagna, L., additional, Schiavon, F., additional, Salvarani, C., additional, and Bortolotti, R., additional

Published
2023
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39. POS1075 RETENTION RATE OF BIOLOGIC AND TARGETED SYNTHETIC ANTI-RHEUMATIC DRUGS IN ELDERLY PATIENTS AFFECTED BY RHEUMATOID ARTHRITIS: DATA FROM ITALIAN GISEA REGISTRY

Author

Manfredi, A., primary, Iannone, F., additional, Bugatti, S., additional, Favalli, E. G., additional, Franceschini, F., additional, Atzeni, F., additional, Bazzani, C., additional, Cantatore, F. P., additional, Caporali, R., additional, Cauli, A., additional, Conti, F., additional, Ferraccioli, G., additional, Foti, R., additional, Fusaro, E., additional, Iagnocco, A., additional, Govoni, M., additional, Gremese, E., additional, Lapadula, G., additional, Rai, A., additional, Rossini, M., additional, Semeraro, A., additional, and Sebastiani, M., additional

Published
2023
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40. AB0540 COAGULATION AND HAEMOSTASIS PARAMETERS IN PREGNANT WOMEN WITH SYSTEMIC LUPUS ERYTHEMATOSUS AND HEALTHY CONTROLS WITH AND WITHOUT PREECLAMPSIA

Author

Lini, D., primary, Gunnarsson, I., additional, Mahmoud Hourani Soutari, N., additional, Lalic-Cosic, S., additional, MC Nicholas, A., additional, Charieti, R., additional, Bremme, K., additional, Franceschini, F., additional, Svenungsson, E., additional, Andreoli, L., additional, and Antovic, A., additional

Published
2023
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41. AB0848 DISEASE BURDEN IS ASSOCIATED WITH POOR PATIENT-REPORTED OUTCOMES IN WOMEN’S HEALTH-RELATED DOMAINS OF QUALITY OF LIFE IN SYSTEMIC SCLEROSIS: A STUDY ON 100 PATIENTS BY A MULTIDIMENSIONAL QUESTIONNAIRE

Author

Lazzaroni, M. G., primary, Moschetti, L., additional, Pedretti, E., additional, Lojacono, A., additional, Zatti, S., additional, Ramazzotto, F., additional, Tincani, A., additional, Franceschini, F., additional, Airò, P., additional, and Andreoli, L., additional

Published
2023
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42. AB1635 RATES OF HIGH LEVELS OF POST-PARTUM DEPRESSIVE SYMPTOMATOLOGY IN WOMEN WITH RHEUMATIC DISEASES FOLLOWED DURING PREGNANCY, AND ASSOCIATIONS WITH TYPE OF DISEASE: A STUDY BY A MULTIDISCIPLINARY TEAM

Author

Crisafulli, F., primary, Pedretti, E., additional, Andreolassi, I., additional, Zatti, S., additional, Ramazzotto, F., additional, Beretta, E., additional, Bani, C., additional, Matthey, S., additional, Della Vedova, A. M., additional, Lini, D., additional, Nalli, C., additional, Gerardi, M. C., additional, Lazzaroni, M. G., additional, Semeraro, P., additional, Fredi, M., additional, Filippini, M., additional, Taglietti, M., additional, Franceschini, F., additional, Andreoli, L., additional, and Tincani, A., additional

Published
2023
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43. POS1490 ANTI-RO/SSA-ANTIBODIES AND ELECTROCARDIOGRAPHIC ABNORMALITIES IN PATIENTS WITH SYSTEMIC LUPUS ERYHEMATOSUS (SLE): DATA OF A MULTIDISCIPLINARY STUDY IN A MONOCENTRIC COHORT

Author

Fredi, M., primary, Bertocchi, S., additional, Pedretti, E., additional, Rovelli, R., additional, Drera, A., additional, Ravasio, F., additional, Riccardi, M., additional, Serafini, L., additional, Cavazzana, I., additional, Vizzardi, E., additional, and Franceschini, F., additional

Published
2023
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47. POS1299 LONG-TERM NEURODEVELOPMENTAL OUTCOME OF CHILDREN BORN TO SYSTEMIC SCLEROSIS WOMEN: PEDIATRIC NEUROPSYCHIATRIC ASSESSMENT THROUGH A SET OF VALIDATED TOOLS

Author

Pedretti, E., primary, Galli, J., additional, Moschetti, L., additional, Nalli, C., additional, Bendoni, M., additional, Visconti, L., additional, Molinaro, A., additional, Franceschini, F., additional, Tincani, A., additional, Airò, P., additional, Andreoli, L., additional, Fazzi, E., additional, and Lazzaroni, M. G., additional

Published
2023
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48. Topic: AS01-Diagnosis/AS01c-Molecular aberrations (cytogenetic, genetic, gene expression): A CARTOGRAPHY OF UBA1 GENE TESTING, EPIDEMIOLOGY AND CLINICAL-GENOMIC CHARACTERISTICS: THE VEXAS/MDS ITALIAN EXPERIENCE

Author

Gurnari, C., primary, Pascale, M.R., additional, Vitale, A., additional, Diral, E., additional, Galossi, E., additional, Falconi, G., additional, Bruno, A., additional, Crisafulli, F., additional, Frassi, M., additional, Cattaneo, C., additional, Bertoli, D., additional, Bernardi, M., additional, Condorelli, A., additional, Morsia, E., additional, Crisà, E., additional, Triggianese, P., additional, Brussino, L., additional, Battipaglia, G., additional, Bindoli, S., additional, Sfriso, P., additional, Caroni, F., additional, Olivieri, A., additional, Kordasti, S., additional, Albano, F., additional, Pane, F., additional, Musto, P., additional, Bocchia, M., additional, Lugli, E., additional, Rambaldi, A., additional, Greco, R., additional, Franceschini, F., additional, Ciceri, F., additional, Cantarini, L., additional, and Voso, M.T., additional

Published
2023
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49. 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, Roberta, Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, Carlo Luigi, Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, Eleonora, 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, Maria Assunta, Carrara, Giancarlo, Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, Enrica Maria, Sebastiani, Gian Domenico, 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., Foti R., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Cipolletta E., Zanetti A., Carrara G., Fusaro E., Sebastiani G. D., De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, Roberta, Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, Carlo Luigi, Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, Eleonora, 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, Maria Assunta, Carrara, Giancarlo, Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, Enrica Maria, Sebastiani, Gian Domenico, 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., Foti R., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Cipolletta E., Zanetti A., Carrara G., Fusaro E., and Sebastiani G. D.

Abstract

Objective To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort. Methods Data 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. Results Among 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) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001). Conclusion The 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

50. Impact of COVID-19 and vaccination campaign on 1,755 systemic sclerosis patients during first three years of pandemic. Possible risks for individuals with impaired immunoreactivity to vaccine, ongoing immunomodulating treatments, and disease-related lung involvement during the next pandemic phase

Author

Ferri, C., Raimondo, V., Giuggioli, D., Gragnani, L., Lorini, S., Dagna, L., Bosello, Silvia Laura, Foti, R., Riccieri, V., Guiducci, S., Cuomo, G., Tavoni, A., De Angelis, R., Cacciapaglia, F., Zanatta, E., Cozzi, F., Murdaca, G., Cavazzana, I., Romeo, N., Codullo, V., Pellegrini, R., Varcasia, G., De Santis, M., Selmi, C., Abignano, G., Caminiti, M., L'Andolina, M., Olivo, D., Lubrano, E., Spinella, A., Lumetti, F., De Luca, G., Ruscitti, P., Urraro, T., Visentini, M., Bellando-Randone, S., Visalli, E., Testa, D., Sciascia, G., Masini, F., Pellegrino, G., Saccon, F., Balestri, E., Elia, G., Ferrari, S. M., Tonutti, A., Dall'Ara, F., Pagano Mariano, G., Pettiti, G., Zanframundo, G., Brittelli, R., Aiello, V., Dal Bosco, Y., Di Cola, I., Scorpiniti, D., Fusaro, E., Ferrari, T., Gigliotti, P., Campochiaro, C., Francioso, F., Iandoli, C., Caira, V., Zignego, A. L., D'Angelo, S., Franceschini, F., Matucci-Cerinic, M., Giacomelli, R., Doria, A., Santini, Stefano Angelo, Fallahi, P., Iannone, F., Antonelli, A., Bosello S. L. (ORCID:0000-0002-4837-447X), Santini S. A. (ORCID:0000-0003-1956-5899), Ferri, C., Raimondo, V., Giuggioli, D., Gragnani, L., Lorini, S., Dagna, L., Bosello, Silvia Laura, Foti, R., Riccieri, V., Guiducci, S., Cuomo, G., Tavoni, A., De Angelis, R., Cacciapaglia, F., Zanatta, E., Cozzi, F., Murdaca, G., Cavazzana, I., Romeo, N., Codullo, V., Pellegrini, R., Varcasia, G., De Santis, M., Selmi, C., Abignano, G., Caminiti, M., L'Andolina, M., Olivo, D., Lubrano, E., Spinella, A., Lumetti, F., De Luca, G., Ruscitti, P., Urraro, T., Visentini, M., Bellando-Randone, S., Visalli, E., Testa, D., Sciascia, G., Masini, F., Pellegrino, G., Saccon, F., Balestri, E., Elia, G., Ferrari, S. M., Tonutti, A., Dall'Ara, F., Pagano Mariano, G., Pettiti, G., Zanframundo, G., Brittelli, R., Aiello, V., Dal Bosco, Y., Di Cola, I., Scorpiniti, D., Fusaro, E., Ferrari, T., Gigliotti, P., Campochiaro, C., Francioso, F., Iandoli, C., Caira, V., Zignego, A. L., D'Angelo, S., Franceschini, F., Matucci-Cerinic, M., Giacomelli, R., Doria, A., Santini, Stefano Angelo, Fallahi, P., Iannone, F., Antonelli, A., Bosello S. L. (ORCID:0000-0002-4837-447X), and Santini S. A. (ORCID:0000-0003-1956-5899)

Abstract

Introduction: The impact of COVID-19 pandemic represents a serious challenge for ‘frail’ patients' populations with inflammatory autoimmune systemic diseases such as systemic sclerosis (SSc). We investigated the prevalence and severity of COVID-19, as well the effects of COVID-19 vaccination campaign in a large series of SSc patients followed for the entire period (first 38 months) of pandemic. Patients and method: This prospective survey study included 1755 unselected SSc patients (186 M, 1,569F; mean age 58.7 ± 13.4SD years, mean disease duration 8.8 ± 7.3SD years) recruited in part by telephone survey at 37 referral centers from February 2020 to April 2023. The following parameters were carefully evaluated: i. demographic, clinical, serological, and therapeutical features; ii. prevalence and severity of COVID-19; and iii. safety, immunogenicity, and efficacy of COVID-19 vaccines. Results: The prevalence of COVID-19 recorded during the whole pandemic was significantly higher compared to Italian general population (47.3 % vs 43.3 %, p < 0.000), as well the COVID-19-related mortality (1.91 % vs 0.72 %, p < 0.001). As regards the putative prognostic factors of worse outcome, COVID-19 positive patients with SSc-related interstitial lung involvement showed significantly higher percentage of COVID-19-related hospitalization compared to those without (5.85 % vs 1.73 %; p < 0.0001), as well as of mortality rate (2.01 % vs 0.4 %; p = 0.002). Over half of patients (56.3 %) received the first two plus one booster dose of vaccine; while a fourth dose was administered to 35.6 %, and only few of them (1.99 %) had five or more doses of vaccine. Of note, an impaired seroconversion was recorded in 25.6 % of individuals after the first 2 doses of vaccine, and in 8.4 % of patients also after the booster dose. Furthermore, the absence of T-cell immunoreactivity was observed in 3/7 patients tested by QuantiFERON® SARSCoV-2 Starter Set (Qiagen). The efficacy of vaccines, evalu

Published
2023

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