Ferri Clodoveo, De Angelis Rossella, Giuggioli Dilia, Bajocchi Gianluigi, Dagna Lorenzo, Zanframundo Giovanni, Foti Rosario, Cacciapaglia Fabio, Cuomo Giovanna, Ariani Alarico, Rosato Edoardo, Guiducci Serena, Girelli Francesco, Riccieri Valeria, Zanatta Elisabetta, Bosello Silvia, Cavazzana Ilaria, Ingegnoli Francesca, De Santis Maria, Murdaca Giuseppe, Abignano Giuseppina, Romeo Nicoletta, Della Rossa Alessandra, Caminiti Maurizio, Iuliano Annamaria, Ciano Giovanni, Beretta Lorenzo, Bagnato Gianluca, Lubrano Ennio, De Andres Ilenia, Giollo Alessandro, Saracco Marta, Agnes Cecilia, Lumetti Federica, Spinella Amelia, Magnani Luca, Campochiaro Corrado, De Luca Giacomo, Codullo Veronica, Visalli Elisa, Masini Francesco, Gigante Antonietta, Bellando-Randone Silvia, Pellegrino Greta, Pigatto Erika, Lazzaroni Maria Grazia, Franceschini Franco, Generali Elena, Mennillo Gianna, Barsotti Simone, Mariano Giuseppa Pagano, Calabrese Francesca, Furini Federica, Vultaggio Licia, Parisi Simone, Peroni Clara Lisa, Rozza Davide, Zanetti Anna, Carrara Greta, Landolfi Giampiero, Scirè Carlo Alberto, Bianchi Gerolamo, Fusaro Enrico, Sebastiani Gian Domenico, Govoni Marcello, D'Angelo Salvatore, Cozzi Franco, Doria Andrea, Iannone Florenzo, Salvarani Carlo, Matucci-Cerinic Marco
Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy.
Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy.
Autoimmun Rev. 2022 Oct;21(10):103159. doi: 10.1016/j.autrev.2022.103159. Epub 2022 Jul 28.
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.
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.
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.
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.
系统性硬化症（SSc）的特点是病因复杂，涉及宿主基因以及导致纤维生成改变和弥漫性微血管病变的环境因素（感染性/毒性因素）。在来自不同地理区域的患者群体中可观察到广泛的临床表型。我们调查了意大利不同地理大区三级转诊中心确诊的SSc患者中特定临床和血清学表型的患病率。将观察结果与世界文献报道的结果进行了比较。
获取了由意大利风湿病学会（SIR）推动的SPRING（系统性硬化症进展研究组）登记处38个三级转诊中心招募的1538例确诊SSc患者（161例男性，占10.5%；平均年龄59.8±26.9岁；平均病程8.9±7.7年）的临床特征，并根据意大利地理大区进行聚类。
与意大利北部和中部的患者相比，意大利南部的患者具有更严重的临床和/或血清学SSc表型；即，他们的弥漫性皮肤型SSc、指端溃疡、干燥综合征、肌肉受累、关节炎、心肺症状、HRCT显示的间质性肺受累的百分比增加，血清抗Scl70自身抗体的患病率也增加。在同一SSc人群中，免疫抑制药物经常被使用。文献综述强调了SSc表型的地理异质性，尽管由于方法学方法的差异，观察结果几乎无法比较。
意大利各大区SSc患者亚组之间的表型差异可能与遗传/环境共同因素相关，也可能与信息和医疗设施的全国网络分布不均有关。
