RMD Open (Mar 2023)

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

  • Carlo Alberto Scirè,
  • Andrea Doria,
  • Marcello Govoni,
  • Gerolamo Bianchi,
  • Marco Matucci-Cerinic,
  • Florenzo Iannone,
  • Ennio Lubrano,
  • Corrado Campochiaro,
  • Veronica Codullo,
  • Alessandra Della Rossa,
  • Gemma Lepri,
  • Elisabetta Zanatta,
  • Beretta Lorenzo,
  • Doria Andrea,
  • Lepri Gemma,
  • Lorenzo Beretta,
  • Greta Carrara,
  • Alarico Ariani,
  • Simone Parisi,
  • Marta Saracco,
  • Francesco Girelli,
  • Maria De Santis,
  • Federica Lumetti,
  • Dilia Giuggioli,
  • Enrico Fusaro,
  • Simone Barsotti,
  • Ilaria Cavazzana,
  • Federica Furini,
  • Carlo Salvarani,
  • Serena Guiducci,
  • Franco Cozzi,
  • Valeria Riccieri,
  • Francesca Ingegnoli,
  • Edoardo Rosato,
  • Antonietta Gigante,
  • Rosario Foti,
  • Elisa Visalli,
  • Fabio Cacciapaglia,
  • Lorenzo Dagna,
  • Franco Franceschini,
  • Silvia Bellando-Randone,
  • Giovanna Cuomo,
  • Gianluigi Bajocchi,
  • Alessandro Giollo,
  • Giacomo De Luca,
  • Giuseppina Abignano,
  • Carlo Sciré,
  • Anna Zanetti,
  • Giovanni Zanframundo,
  • Edoardo Cipolletta,
  • Silvia Laura Bosello,
  • Clodoveo Ferri,
  • Amelia Spinella,
  • Giuseppa Pagano Mariano,
  • Maurizio Caminiti,
  • Giuseppe Murdaca,
  • Salvatore D'Angelo,
  • Gianpiero Landolfi,
  • Nicoletta Romeo,
  • Gian Domenico Sebastiani,
  • Erika Pigatto,
  • Rossella De Angelis,
  • Davide Rozza,
  • Maria-Grazia Lazzaroni,
  • Anna Maria Iuliano,
  • Giovanni Ciano,
  • Gianluca Bagnato,
  • Ilenia De Andres,
  • Cecilia Agnes,
  • Luca Magnani,
  • Claudio Di Vico,
  • Greta Pellagrino,
  • Elena Generali,
  • Gianna Mennillo,
  • Licia Vultaggio,
  • Clara Lisa Peroni,
  • Abignano Giuseppina,
  • Agnes Cecilia,
  • Amato Giorgio,
  • Ariani Alarico,
  • Bagnato Gianluca,
  • Bajoicchi Gianluigi,
  • Barsotti Simone,
  • Bellando-Randone Silvia,
  • Benenati Alessia,
  • Bianchi Gerolamo,
  • Bosello Silvia,
  • Cacciapaglia Fabio,
  • Calabrese Francesca,
  • Caminiti Maurizio,
  • Campochiaro Corrado,
  • Carignola Renato,
  • Ciano Giovanni,
  • Cipolletta Edoardo,
  • Codullo Veronica,
  • Cozzi Franco,
  • Cuomo Giovanna,
  • D’Angelo Salvatore,
  • Dagna Lorenzo,
  • Dall’Ara Francesca,
  • De Andres Ilenia,
  • De Angelis Rossella,
  • De Cata Angelo,
  • De Luca Giacomo,
  • De Santis Maria,
  • Della Rossa Alessandra,
  • Di Vico Claudio,
  • Doveri Marica,
  • Foti Rosario,
  • Furini Federica,
  • Fusaro Enrico,
  • Generali Elena,
  • Gigante Antonietta,
  • Giollo Alessandro,
  • Girelli Francesco,
  • Giuggioli Dilia,
  • Govoni Marcello,
  • Guiducci Serena,
  • Iannone Florenzo,
  • Ingegnoli Francesca,
  • Iuliano Anna Maria,
  • Lazzaroni Maria Grazia,
  • Lubrano Ennio,
  • Lumetti Federica,
  • Magnani Luca,
  • Mennillo Gianna,
  • Murdaca Giuseppe Ospedale,
  • Pagano Mariano Giuseppa,
  • Parisi Simone,
  • Pellegrino Greta,
  • Peroni Clara Lisa,
  • Pigatto Erika,
  • Riccieri Valeria,
  • Romeo Nicoletta,
  • Rosato Edoardo,
  • Sambataro Gianluca,
  • Saracco Marta,
  • Sebastiani Giandomenico,
  • Spinella Amelia,
  • Talotta Rossella,
  • Visalli Elisa,
  • Vultaggio Licia,
  • Zanatta Elisabetta,
  • Zanframundo Giovanni

DOI
https://doi.org/10.1136/rmdopen-2022-002890
Journal volume & issue
Vol. 9, no. 1

Abstract

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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.