J Rheumatol 2021 Nov 15. Epub 2021 Nov 15.
This study was supported by the Italian Society for Rheumatology (SIR). R. De Angelis, MD, A.M. Risa, MD, Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona; D. Giuggioli, MD, F. Lumetti, MD, A. Spinella, MD, C. Ferri, MD, Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena; G. Bajocchi, MD, L. Magnani, MD, C. Salvarani, MD, Rheumatology Unit, S. Maria Hospital-USL, IRCCS Institute, Reggio Emilia; L. Dagna, MD, C. Campochiaro, MD, G. De Luca, MD, Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan; G. Zanframundo, MD, V. Codullo, MD, Department of Rheumatology, Policlinico San Matteo, Pavia; R. Foti, MD, E. Visalli, MD, AOU Policlinico Vittorio Emanuele, Catania; F. Cacciapaglia, MD, F. Iannone, MD, Rheumatology Unit, Department of Emergency Surgery and Organ Transplantations, University of Bari, Bari; G. Cuomo, MD, F. Masini, MD, Luigi Vanvitelli University, Naples; A. Ariani, MD, Department of Medicine, Internal Medicine and Rheumatology, Azienda Ospedaliero Universitaria di Parma, Parma; E. Rosato, MD, A. Gigante, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; S. Guiducci, MD, S. Bellando-Randone, MD, Department of Rheumatology, University of Florence, Florence; F. Girelli, MD, Department of Medicine, Rheumatology Unit, Ospedale GB Morgagni-L Pierantoni, Forlì; V. Riccieri, MD, G. Pellegrino, MD, Department of Rheumatology, Sapienza University of Rome, Rome; E. Zanatta, MD, A. Doria, MD, Department of Rheumatology, University of Padua, Padova; S. Bosello, MD, Institute of Rheumatology and Affine Sciences, Division of Rheumatology, Catholic University of the Sacred Heart, Rome; I. Cavazzana, MD, F. Dall'Ara, MD, M.G. Lazzaroni, MD, Department of Rheumatology, Spedali Civili di Brescia, Brescia; F. Ingegnoli, MD, Division of Clinical Rheumatology, ASST Pini, Dept. of Clinical Sciences & Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan; M. De Santis, MD, E.Generali, MD, Humanitas Clinical and Research Center IRCCS, Milan; G. Murdaca, MD, S. Martino Hospital-University of Genoa, Genoa; G. Abignano, MD, G. Mennillo, MD, S. D'Angelo, MD, Department of Translational and Precision Medicine, Sapienza University of Rome; N. Romeo, MD, S. Croce e Carle Hospital, Cuneo; A. Della Rossa, MD, S. Barsotti, MD, Department of Rheumatology, University of Pisa, Pisa; M. Caminiti, MD, G. Pagano Mariano, MD, F. Calabrese, MD, Departmental Rheumatology Unit, Grande Ospedale Metropolitano, Reggio Calabria; A. Iuliano, MD, G.D. Sebastiani, MD, Rheumatology Unit, San Camillo-Forlanini Hospital, Rome; G. Ciano, MD, Local Health Department, Avellino; L. Beretta, MD, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico di Milano, Milan; G. Bagnato, MD, Department of Clinical and Experimental Medicine, University of Messina, Messina; E. Lubrano, MD, Department of Rheumatology, University of Molise, Campobasso; I. De Andres, MD, Rheumatology Unit, Azienda Ospedaliera di Rilievo Nazionale ed Alta Specializzazione Garibaldi, Catania; A. Giollo, MD, Rheumatology Section, Department of Medicine, University of Verona, Verona; M. Saracco, MD, Rheumatology Unit, Mauriziano-Umberto I Hospital, Turin; C. Agnes, MD, San Lorenzo Hospital; E. Pigatto, MD, F. Cozzi, MD, Department of Medicine, Villa Salus Hospital, Venice; F. Furini, MD, L. Vultaggio, MD, M. Govoni, MD, Rheumatology Unit, Department of Medical Sciences, University of Ferrara and Azienda Ospedaliera-Universitaria S. Anna di Ferrara, Ferrara; S. Parisi, MD, C.L. Peroni, MD, E. Fusaro, MD, Rheumatology Unit, Città della Salute e della Scienza, Turin; D.Rozza, MS, A. Zanetti, MS, G. Carrara, MS, G. Landolfi, MS, Epidemiology Unit, Italian Society for Rheumatology, Milan; C.A. Scirè, MD, Epidemiology Unit, Italian Society for Rheumatology, Milan, and Rheumatology Unit, University of Ferrara-S. Anna Hospital, Ferrara; G. Bianchi, MD, Rheumatology Unit, Department of Musculoskeletal Sciences, Local Health Trust 3, La Colletta Hospital, Genoa; \M. Matucci-Cerinic, MD, Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, and Department of Rheumatology, University of Florence, Florence, Italy. The authors declare no conflicts of interest relevant to this article. Address correspondence to Prof. R. De Angelis, MD, PhD, Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, "Carlo Urbani" Hospital, Via Aldo Moro, 25, 60035 Jesi, Ancona, Italy. Email: Accepted for publication October 27, 2021.
Objective: There is still a great deal to learn about the influence of sex in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying sex differences in disease expression, with a special focus on demographic, clinical, and serological characteristics.
Methods: A multicenter SSc cohort of 2281 patients, including 247 men, was recruited in the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry. Demographic data, disease manifestations, serological profile, and internal organ involvement were compared.
Results: The overall female/male ratio was 8.2:1. Female/male ratios for limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma subsets were 8.7:1, 4.9:1, and 10.7:1, respectively. A shorter time from onset of Raynaud phenomenon to SSc diagnosis, an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, whereas a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein B was detected in the female group. Males exhibited lower left ventricular ejection fraction, as well as higher prevalence of conduction blocks, arrhythmias, ground glass, and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs.
Conclusion: Our study further supports the presence of several sex-related differences in patients with SSc. These differences were pronounced in the severity of cutaneous, peripheral vascular, and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterized the female sex.