现实生活中系统性硬化症患者指端溃疡的患病率及临床相关性：来自意大利风湿病学会SPRING注册研究的经验
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.
作者信息
Orlandi Martina, De Luca Giacomo, Ferri Clodoveo, Spinella Amelia, Lumetti Federica, Costantini Riccardo Cuoghi, De Angelis Rossella, Riccieri Valeria, Bosello Silvia Laura, Cacciapaglia Fabio, Codullo Veronica, Bajocchi Gianluigi, Campochiaro Corrado, Zanframundo Giovanni, Foti Rosario, Cuomo Giovanna, Ariani Alarico, Rosato Edoardo, Girelli Francesco, Zanatta Elisabetta, Cavazzana Ilaria, Ingegnoli Francesca, De Santis Maria, Murdaca Giuseppe, Abignano Giuseppina, Giorgio Pettiti, Della Rossa Alessandra, Caminiti Maurizio, Iuliano Anna Maria, Ciano Giovanni, Beretta Lorenzo, Bagnato Gianluca, Lubrano Ennio, De Andres Ilenia, Giollo Alessandro, Saracco Marta, Agnes Cecilia, Cipolletta Edoardo, Magnani Luca, Visalli Elisa, Iandoli Carlo, Gigante Antonietta, Pellegrino Greta, Pigatto Erika, Lazzaroni Maria Grazia, Franceschini Franco, Generali Elena, Mennillo Gianna, Barsotti Simone, Mariano Giuseppa Pagano, Furini Federica, Vultaggio Licia, Parisi Simone, Peroni Clara Lisa, Bianchi Gerolamo, Fusaro Enrico, Sebastiani Gian Domenico, Govoni Marcello, D'Angelo Salvatore, Cozzi Franco, Guiducci Serena, Doria Andrea, Salvarani Carlo, Iannone Florenzo, Dagna Lorenzo, Matucci-Cerinic Marco, Bellando-Randone Silvia, Giuggioli Dilia
机构信息
Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia School of Medicine, Via del Pozzo, 71-41125, Modena, Italy.
Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), & Inflammation, Fibrosis and Aging Initiative (INFLAGE), IRCCS San Raffaele Scientific Institute, Milan, Italy.
出版信息
Clin Rheumatol. 2025 May 15. doi: 10.1007/s10067-025-07449-1.
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.
引言
指端溃疡（DU）是系统性硬化症（SSc）最常见的表现之一。DU的出现似乎是内脏受累的一个警示信号，且与疾病的不良预后相关。本研究的目的是评估来自SPRING注册中心的一个大型SSc队列中DU的患病率及其与临床表现/亚型的关系。
方法
符合2013年美国风湿病学会/欧洲抗风湿病联盟（ACR/EULAR）分类标准且无指端溃疡数据缺失的SSc患者纳入一项横断面研究。构建逻辑回归模型以检验DU的存在与SSc相关特征之间的关联。
结果
在1873例符合条件的SSc患者中，DU的存在与胃肠道受累（比值比[OR]分别为1.88、2.04和1.74；p<0.001）、血清抗拓扑异构酶Ⅰ抗体（ATA）阳性（OR 2.15；p<0.001）以及毛细血管扩张、指硬皮病、指凹痕瘢痕和钙质沉着显著相关（OR分别为1.40，p = 0.005；OR 3.43，p<0.001；OR 9.12，p<0.001和OR 2.77，p<0.001）。在多变量回归模型中，即使在对协变量进行调整后，ATA阳性（OR 1.76，p = 0.039）、手指肿胀（OR 2.82，p<0.001）和较高的改良EUSTAR疾病活动指数（revEUSTAR-AI）（OR 6.63，p<0.001）仍为DU存在的危险因素。此外，有既往免疫抑制治疗史的SSc患者中DU的发生率较低（OR 0.53，p = 0.032）。
结论
在我们的意大利SSc队列中，DU与手指肿胀、高revEUSTR-AI及ATA血清阳性显著相关。值得注意的是，免疫抑制治疗与DU的低发生率相关，提示它们可能有助于预防这些有害表现。要点 • 指端溃疡与手指肿胀、高疾病活动度及抗Scl70血清阳性显著相关。 • 免疫抑制治疗与指端溃疡的低发生率相关，提示它们可能有助于预防这些有害表现。
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