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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; [...]

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

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  • Media type: E-Article
  • Title: 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
  • Contributor: 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; [...]
  • imprint: BMJ, 2023
  • Published in: RMD Open
  • Language: English
  • DOI: 10.1136/rmdopen-2022-002890
  • ISSN: 2056-5933
  • Keywords: Immunology ; Immunology and Allergy ; Rheumatology
  • Origination:
  • Footnote:
  • Description: <jats:sec><jats:title>Objective</jats:title><jats:p>To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>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&lt;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&lt;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&lt;0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p&lt;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&lt;0.001).</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>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.</jats:p></jats:sec>
  • Access State: Open Access