Introduction <p>Digital ulcers (DU) are one of the most frequent manifestations in systemic sclerosis (SSc). The presence of DU seems to be a sentinel sign of internal organ involvement and is related to a poor prognosis of the disease. The aim of this study was to evaluate the prevalence and the relationship of DU with clinical manifestations/variants in a large SSc cohort from the SPRING registry.</p> Methods <p>SSc patients fulfilling the ACR/EULAR 2013 classification criteria without missing data on digital ulcers were enrolled in a cross-sectional study. Logistic regression models were built to test the association between the presence of DU and SSc-related features.</p> Results <p>Among 1873 eligible SSc patients, the presence of DU was significantly associated with gastrointestinal involvement (OR 1.88, 2.04 and 1.74; <i>p</i> &lt; 0.001) and serum ATA positivity (OR 2.15; <i>p</i> &lt; 0.001), as well as with telangiectasias, sclerodactyly, digital pitting scar, and calcinosis (OR 1.40, <i>p</i> = 0.005; OR 3.43, <i>p</i> &lt; 0.001, OR 9.12, <i>p</i> &lt; 0.001 and OR 2.77, <i>p</i> &lt; 0.001; respectively). In the multivariable regression models, even after adjustment for covariates, ATA positivity (OR 1.76, <i>p</i> = 0.039), puffy fingers (OR 2.82, <i>p</i> &lt; 0.001), and a higher revEUSTAR-AI (OR 6.63, <i>p</i> &lt; 0.001) emerged as risk factors for the presence of DU. Moreover, a low presence of DU was recorded in SSc patients with a history of previous immunosuppressive treatments (OR 0.53, <i>p</i> = 0.032).</p> Conclusion <p>In our Italian SSc cohort, DUs were significantly associated with the presence of puffy fingers, high revEUSTR-AI, and ATA seropositivity. Noteworthy, immunosuppressive treatments were associated with a low rate of DU, suggesting that they might contribute to the prevention of these harmful manifestations.</p> <p><Table Float="No" ID="Taba"> <tgroup cols="1"> <colspec align="left" colname="c1" colnum="1" /> <tbody> <row> <entry align="left" colname="c1"> <p><b>Key Points</b></p> </entry> </row> <row> <entry align="left" colname="c1"> <p>• <i>Digital ulcers were significantly associated with the presence of puffy fingers, high disease activity, and anti-Scl70 seropositivity.</i></p> <p>• <i>Immunosuppressive treatments were associated with a low rate of digital ulcers, suggesting that they might contribute to the prevention of these harmful manifestations.</i></p> </entry> </row> </tbody> </tgroup> </Table></p>

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Prevalence and clinical relevance of digital ulcers in systemic sclerosis patients from the real-life: the experience of the SPRING Registry of the Italian Society for Rheumatology

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

摘要

Introduction

Digital ulcers (DU) are one of the most frequent manifestations in systemic sclerosis (SSc). The presence of DU seems to be a sentinel sign of internal organ involvement and is related to a poor prognosis of the disease. The aim of this study was to evaluate the prevalence and the relationship of DU with clinical manifestations/variants in a large SSc cohort from the SPRING registry.

Methods

SSc patients fulfilling the ACR/EULAR 2013 classification criteria without missing data on digital ulcers were enrolled in a cross-sectional study. Logistic regression models were built to test the association between the presence of DU and SSc-related features.

Results

Among 1873 eligible SSc patients, the presence of DU was significantly associated with gastrointestinal involvement (OR 1.88, 2.04 and 1.74; p < 0.001) and serum ATA positivity (OR 2.15; p < 0.001), as well as with telangiectasias, sclerodactyly, digital pitting scar, and calcinosis (OR 1.40, p = 0.005; OR 3.43, p < 0.001, OR 9.12, p < 0.001 and OR 2.77, p < 0.001; respectively). In the multivariable regression models, even after adjustment for covariates, ATA positivity (OR 1.76, p = 0.039), puffy fingers (OR 2.82, p < 0.001), and a higher revEUSTAR-AI (OR 6.63, p < 0.001) emerged as risk factors for the presence of DU. Moreover, a low presence of DU was recorded in SSc patients with a history of previous immunosuppressive treatments (OR 0.53, p = 0.032).

Conclusion

In our Italian SSc cohort, DUs were significantly associated with the presence of puffy fingers, high revEUSTR-AI, and ATA seropositivity. Noteworthy, immunosuppressive treatments were associated with a low rate of DU, suggesting that they might contribute to the prevention of these harmful manifestations.

Key Points

Digital ulcers were significantly associated with the presence of puffy fingers, high disease activity, and anti-Scl70 seropositivity.

Immunosuppressive treatments were associated with a low rate of digital ulcers, suggesting that they might contribute to the prevention of these harmful manifestations.