Lepri Gemma, Airò Paolo, Distler Oliver, Andréasson Kristofer, Braun-Moscovici Yolanda, Hachulla Eric, Balbir-Gurman Alexandra, De Langhe Ellen, Rednic Simona, Ingegnoli Francesca, Rosato Edoardo, Groseanu Laura, Ionescu Ruxandra, Bellando-Randone Silvia, Garzanova Liudmila, Beretta Lorenzo, Bellocchi Chiara, Moiseev Sergey, Novikov Pavel, Szabo Iulia, Krasowska Dorota, Codullo Veronica, Walker Ulrich A, Manolaraki Chrysoula, Guiducci Serena, Truchetet Marie-Elise, Iannone Florenzo, Tofani Lorenzo, Bruni Cosimo, Smith Vanessa, Cuomo Giovanna, Krusche Martin, Matucci-Cerinic Marco, Allanore Yannick
Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Rheumatology and Clinical Immunology, Spedali Civili of Brescia, Brescia, Italy.
J Scleroderma Relat Disord. 2023 Oct;8(3):210-220. doi: 10.1177/23971983231155948. Epub 2023 Apr 4.
Several studies described the cross-sectional characteristics of systemic sclerosis patients and coexisting primary biliary cholangitis, but longitudinal prognostic data are lacking.
To describe the systemic sclerosis-primary biliary cholangitis phenotype, including baseline characteristics and outcomes.
We performed a multicentre the European Scleroderma Trials and Research Group study of systemic sclerosis patients with primary biliary cholangitis or with primary biliary cholangitis-specific antibodies, matched with systemic sclerosis controls free from hepatobiliary involvement matched for disease duration and cutaneous subset. Data were recorded at baseline and at the last available visit.
A total of 261 patients were enrolled (115 primary biliary cholangitis-systemic sclerosis, 161 systemic sclerosis). At baseline, systemic sclerosis-primary biliary cholangitis patients had a higher prevalence of anti-centromere antibodies ( = 0.0023) and a lower prevalence of complete absence of digital ulcers. The milder vascular involvement was confirmed at follow-up when crucial differences emerged in the percentage of patients experiencing digital ulcers; a significantly higher number of patients who never experienced digital ulcers were observed among primary biliary cholangitis-systemic sclerosis patients ( = 0.0015). Moreover, a greater incidence of pulmonary arterial hypertension ( < 0.001) and of conduction blocks ( = 0.0256) was observed in systemic sclerosis patients without primary biliary cholangitis. Patients with primary biliary cholangitis had higher levels of liver enzymes at baseline than systemic sclerosis patients; a significant decrease in liver enzymes was observed at follow-up. Out of 18 patients with cholangitis, one received a liver transplant at follow-up.
Our data show that systemic sclerosis-primary biliary cholangitis exhibit a mild systemic sclerosis and primary biliary cholangitis phenotype with outcomes being in general favourable.
多项研究描述了系统性硬化症患者合并原发性胆汁性胆管炎的横断面特征，但缺乏纵向预后数据。
描述系统性硬化症-原发性胆汁性胆管炎的表型，包括基线特征和预后。
我们进行了一项多中心欧洲硬皮病试验与研究组研究，纳入患有原发性胆汁性胆管炎或具有原发性胆汁性胆管炎特异性抗体的系统性硬化症患者，并与无肝胆受累的系统性硬化症对照患者进行匹配，匹配因素为病程和皮肤亚型。在基线和最后一次可获得的随访时记录数据。
共纳入261例患者（115例原发性胆汁性胆管炎-系统性硬化症患者，161例系统性硬化症患者）。在基线时，系统性硬化症-原发性胆汁性胆管炎患者抗着丝点抗体的患病率较高（P = 0.0023），完全没有指端溃疡的患病率较低。随访时证实血管受累较轻，出现指端溃疡的患者百分比存在关键差异；在原发性胆汁性胆管炎-系统性硬化症患者中观察到从未经历过指端溃疡的患者数量显著更多（P = 0.0015）。此外，在无原发性胆汁性胆管炎的系统性硬化症患者中观察到肺动脉高压（P < 0.001）和传导阻滞（P = 0.0256）的发生率更高。原发性胆汁性胆管炎患者在基线时的肝酶水平高于系统性硬化症患者；随访时观察到肝酶显著下降。在18例胆管炎患者中，有1例在随访时接受了肝移植。
我们的数据表明，系统性硬化症-原发性胆汁性胆管炎表现出轻度的系统性硬化症和原发性胆汁性胆管炎表型，总体预后良好。
