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@article{De:2023:10.1136/rmdopen-2022-002890,
author = {De, Angelis R and Ferri, C and Giuggioli, D and Bajocchi, G and Dagna, L and Bellando-Randone, S and Zanframundo, G and Foti, R and Cacciapaglia, F and Cuomo, G and Ariani, A and Rosato, E and Lepri, G and Girelli, F and Riccieri, V and Zanatta, E and Bosello, SL and Cavazzana, I and Ingegnoli, F and De, Santis M and Murdaca, G and Abignano, G and Romeo, N and Della, Rossa A and Caminiti, M and Iuliano, AM and Ciano, G and Beretta, L and Bagnato, G and Lubrano, E and De, Andres I and Giollo, A and Saracco, M and Agnes, C and Cipolletta, E and Lumetti, F and Spinella, A and Magnani, L and Campochiaro, C and De, Luca G and Codullo, V and Visalli, E and Di, Vico C and Gigante, A and Pellagrino, G and Pigatto, E and Lazzaroni, M-G and Franceschini, F and Generali, E and Mennillo, G and Barsotti, S and Mariano, GP and Furini, F and Vultaggio, L and Parisi, S and Peroni, CL and Rozza, D and Zanetti, A and Carrara, G and Landolfi, G and Scirè, CA and Bianchi, G and Fusaro, E and Sebastiani, G},
doi = {10.1136/rmdopen-2022-002890},
journal = {RMD Open},
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.},
url = {http://dx.doi.org/10.1136/rmdopen-2022-002890},
volume = {9},
year = {2023}
}

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TY  - JOUR
AB  - 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.
AU  - De,Angelis R
AU  - Ferri,C
AU  - Giuggioli,D
AU  - Bajocchi,G
AU  - Dagna,L
AU  - Bellando-Randone,S
AU  - Zanframundo,G
AU  - Foti,R
AU  - Cacciapaglia,F
AU  - Cuomo,G
AU  - Ariani,A
AU  - Rosato,E
AU  - Lepri,G
AU  - Girelli,F
AU  - Riccieri,V
AU  - Zanatta,E
AU  - Bosello,SL
AU  - Cavazzana,I
AU  - Ingegnoli,F
AU  - De,Santis M
AU  - Murdaca,G
AU  - Abignano,G
AU  - Romeo,N
AU  - Della,Rossa A
AU  - Caminiti,M
AU  - Iuliano,AM
AU  - Ciano,G
AU  - Beretta,L
AU  - Bagnato,G
AU  - Lubrano,E
AU  - De,Andres I
AU  - Giollo,A
AU  - Saracco,M
AU  - Agnes,C
AU  - Cipolletta,E
AU  - Lumetti,F
AU  - Spinella,A
AU  - Magnani,L
AU  - Campochiaro,C
AU  - De,Luca G
AU  - Codullo,V
AU  - Visalli,E
AU  - Di,Vico C
AU  - Gigante,A
AU  - Pellagrino,G
AU  - Pigatto,E
AU  - Lazzaroni,M-G
AU  - Franceschini,F
AU  - Generali,E
AU  - Mennillo,G
AU  - Barsotti,S
AU  - Mariano,GP
AU  - Furini,F
AU  - Vultaggio,L
AU  - Parisi,S
AU  - Peroni,CL
AU  - Rozza,D
AU  - Zanetti,A
AU  - Carrara,G
AU  - Landolfi,G
AU  - Scirè,CA
AU  - Bianchi,G
AU  - Fusaro,E
AU  - Sebastiani,GD
AU  - Govoni,M
AU  - D'Angelo,S
AU  - Cozzi,F
AU  - Guiducci,S
AU  - Doria,A
AU  - Salvarani,C
AU  - Iannone,F
AU  - Matucci-Cerinic,M
AU  - SPRING-SIR,Systemic Sclerosis PRogression INvestiGation group of the Italian Society of Rheumatology
DO  - 10.1136/rmdopen-2022-002890
PY  - 2023///
TI  - 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.
T2  - RMD Open
UR  - http://dx.doi.org/10.1136/rmdopen-2022-002890
UR  - https://www.ncbi.nlm.nih.gov/pubmed/36868782
VL  - 9
ER  -

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