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. Issue 1 (3rd March 2023)

• Record Type: Journal Article
• Authors: De Angelis, Rossella; Ferri, Clodoveo; Giuggioli, Dilia; Bajocchi, Gianluigi; Dagna, Lorenzo; Bellando-Randone, Silvia; Zanframundo, Giovanni; Foti, Rosario; Cacciapaglia, Fabio; Cuomo, Giovanna; Ariani, Alarico; Rosato, Edoardo; Lepri, Gemma; Girelli, Francesco; Riccieri, Valeria; Zanatta, Elisabetta; Bosello, Silvia Laura; Cavazzana, Ilaria; Ingegnoli, Francesca; De Santis, Maria; Murdaca, Giuseppe; Abignano, Giuseppina; Romeo, Nicoletta; Della Rossa, Alessandra; Caminiti, Maurizio; Iuliano, Anna Maria; Ciano, Giovanni; Beretta, Lorenzo; Bagnato, Gianluca; Lubrano, Ennio; De Andres, Ilenia; Giollo, Alessandro; Saracco, Marta; Agnes, Cecilia; Cipolletta, Edoardo; Lumetti, Federica; Spinella, Amelia; Magnani, Luca; Campochiaro, Corrado; De Luca, Giacomo; Codullo, Veronica; Visalli, Elisa; Di Vico, Claudio; Gigante, Antonietta; Pellagrino, Greta; Pigatto, Erika; Lazzaroni, Maria-Grazia; Franceschini, Franco; Generali, Elena; Mennillo, Gianna; Barsotti, Simone; Mariano, Giuseppa Pagano; Furini, Federica; Vultaggio, Licia; Parisi, Simone; Peroni, Clara Lisa; Rozza, Davide; Zanetti, Anna; Carrara, Greta; Landolfi, Gianpiero; Scirè, Carlo Alberto; Bianchi, Gerolamo; Fusaro, Enrico; Sebastiani, Gian Domenico; Govoni, Marcello; D'Angelo, Salvatore; Cozzi, Franco; Guiducci, Serena; Doria, Andrea; Salvarani, Carlo; Iannone, Florenzo; Matucci-Cerinic, Marco;
• Other Names: author non-byline.; Ferri Clodoveo author non-byline.; Matucci-Cerinic Marco author non-byline.; Giuseppina Abignano author non-byline.; Cecilia Agnes author non-byline.; Giorgio Amato author non-byline.; Alarico Ariani author non-byline.; Gianluca Bagnato author non-byline.; Gianluigi Bajoicchi author non-byline.; Simone Barsotti author non-byline.; Silvia Bellando-Randone author non-byline.; Alessia Benenati author non-byline.; Lorenzo Beretta author non-byline.; Gerolamo Bianchi author non-byline.; Silvia Bosello author non-byline.; Fabio Cacciapaglia author non-byline.; Francesca Calabrese author non-byline.; Maurizio Caminiti author non-byline.; Corrado Campochiaro author non-byline.; Renato Carignola author non-byline.; Giovanni Ciano author non-byline.; Edoardo Cipolletta author non-byline.; Veronica Codullo author non-byline.; Franco Cozzi author non-byline.; Giovanna Cuomo author non-byline.; Salvatore D'Angelo author non-byline.; Lorenzo Dagna author non-byline.; Francesca Dall'Ara author non-byline.; Ilenia De Andres author non-byline.; Rossella De Angelis author non-byline.; Angelo De Cata author non-byline.; Giacomo De Luca author non-byline.; Maria De Santis author non-byline.; Alessandra Della Rossa author non-byline.; Claudio Di Vico author non-byline.; Andrea Doria author non-byline.; Marica Doveri author non-byline.; Rosario Foti author non-byline.; Federica Furini author non-byline.; Enrico Fusaro author non-byline.; Elena Generali author non-byline.; Antonietta Gigante author non-byline.; Alessandro Giollo author non-byline.; Francesco Girelli author non-byline.; Dilia Giuggioli author non-byline.; Marcello Govoni author non-byline.; Serena Guiducci author non-byline.; Florenzo Iannone author non-byline.; Francesca Ingegnoli author non-byline.; Maria Iuliano Anna author non-byline.; Grazia Lazzaroni Maria author non-byline.; Gemma Lepri author non-byline.; Ennio Lubrano author non-byline.; Federica Lumetti author non-byline.; Luca Magnani author non-byline.; Gianna Mennillo author non-byline.; Ospedale Murdaca Giuseppe author non-byline.; Giuseppa Pagano Mariano author non-byline.; Simone Parisi author non-byline.; Greta Pellegrino author non-byline.; Lisa Peroni Clara author non-byline.; Erika Pigatto author non-byline.; Valeria Riccieri author non-byline.; Nicoletta Romeo author non-byline.; Edoardo Rosato author non-byline.; Gianluca Sambataro author non-byline.; Marta Saracco author non-byline.; Giandomenico Sebastiani author non-byline.; Amelia Spinella author non-byline.; Rossella Talotta author non-byline.; Elisa Visalli author non-byline.; Licia Vultaggio author non-byline.; Elisabetta Zanatta author non-byline.; Giovanni Zanframundo author non-byline.; Scirè Carlo author non-byline.; Carrara Greta author non-byline.; Landolfi Gianpiero author non-byline.; Rozza Davide author non-byline.; Zanetti Anna author non-byline.;
• Is Part Of: RMD open. Volume 9:Issue 1(2023)
• Publication Date: 2023-03-03
• Subjects: Scleroderma, Systemic -- Autoimmunity -- Autoimmune Diseases -- Epidemiology; Musculoskeletal system -- Diseases -- Periodicals; Rheumatism -- Periodicals; 616.7005
• DOI: 10.1136/rmdopen-2022-002890
• Languages: English
• ISSNs: 2056-5933

Abstract

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.