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Review
. 2022 Oct;21(10):103159.
doi: 10.1016/j.autrev.2022.103159. Epub 2022 Jul 28.

Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature

Clodoveo Ferri  1 , Rossella De Angelis  2 , Dilia Giuggioli  3 , Gianluigi Bajocchi  4 , Lorenzo Dagna  5 , Giovanni Zanframundo  6 , Rosario Foti  7 , Fabio Cacciapaglia  8 , Giovanna Cuomo  9 , Alarico Ariani  10 , Edoardo Rosato  11 , Serena Guiducci  12 , Francesco Girelli  13 , Valeria Riccieri  14 , Elisabetta Zanatta  15 , Silvia Bosello  16 , Ilaria Cavazzana  17 , Francesca Ingegnoli  18 , Maria De Santis  19 , Giuseppe Murdaca  20 , Giuseppina Abignano  21 , Nicoletta Romeo  22 , Alessandra Della Rossa  23 , Maurizio Caminiti  24 , Annamaria Iuliano  25 , Giovanni Ciano  26 , Lorenzo Beretta  27 , Gianluca Bagnato  28 , Ennio Lubrano  29 , Ilenia De Andres  30 , Alessandro Giollo  31 , Marta Saracco  32 , Cecilia Agnes  33 , Federica Lumetti  3 , Amelia Spinella  3 , Luca Magnani  4 , Corrado Campochiaro  5 , Giacomo De Luca  5 , Veronica Codullo  6 , Elisa Visalli  7 , Francesco Masini  9 , Antonietta Gigante  11 , Silvia Bellando-Randone  12 , Greta Pellegrino  14 , Erika Pigatto  34 , Maria Grazia Lazzaroni  17 , Franco Franceschini  17 , Elena Generali  19 , Gianna Mennillo  21 , Simone Barsotti  23 , Giuseppa Pagano Mariano  24 , Francesca Calabrese  24 , Federica Furini  35 , Licia Vultaggio  35 , Simone Parisi  36 , Clara Lisa Peroni  36 , Davide Rozza  37 , Anna Zanetti  37 , Greta Carrara  37 , Giampiero Landolfi  37 , Carlo Alberto Scirè  38 , Gerolamo Bianchi  39 , Enrico Fusaro  36 , Gian Domenico Sebastiani  25 , Marcello Govoni  35 , Salvatore D'Angelo  21 , Franco Cozzi  34 , Andrea Doria  15 , Florenzo Iannone  8 , Carlo Salvarani  4 , Marco Matucci-Cerinic  40 ; SPRING-SIR (Systemic Sclerosis PRogression INvestiGation group of the Italian Society of Rheumatology)
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Free article
Review

Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature

Clodoveo Ferri et al. Autoimmun Rev. 2022 Oct.
Free article

Abstract

Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature.

Materials and methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 ± 26.9 yrs.; mean disease duration 8.9 ± 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas.

Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches.

Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities.

Keywords: Environmental; Geographical areas; Macro-areas; Referral; Scleroderma; Systemic sclerosis.

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Conflict of interest statement

Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

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