Systemic sclerosis and primary biliary cholangitis: Longitudinal data to determine the outcomes

Autor: Gemma Lepri, Paolo Airò, Oliver Distler, Kristofer Andréasson, Yolanda Braun-Moscovici, Eric Hachulla, Alexandra Balbir-Gurman, Ellen De Langhe, Simona Rednic, Francesca Ingegnoli, Edoardo Rosato, Laura Groseanu, Ruxandra Ionescu, Silvia Bellando-Randone, Liudmila Garzanova, Lorenzo Beretta, Chiara Bellocchi, Sergey Moiseev, Pavel Novikov, Iulia Szabo, Dorota Krasowska, Veronica Codullo, Ulrich A. Walker, Chrysoula Manolaraki, Serena Guiducci, Marie-Elise Truchetet, Florenzo Iannone, Lorenzo Tofani, Cosimo Bruni, Vanessa Smith, Giovanna Cuomo, Martin Krusche, Marco Matucci-Cerinic, Yannick Allanore
Přispěvatelé: Lepri, G., Airo, P., Distler, O., Andreasson, K., Braun-Moscovici, Y., Hachulla, E., Balbir-Gurman, A., De Langhe, E., Rednic, S., Ingegnoli, F., Rosato, E., Groseanu, L., Ionescu, R., Bellando-Randone, S., Garzanova, L., Beretta, L., Bellocchi, C., Moiseev, S., Novikov, P., Szabo, I., Krasowska, D., Codullo, V., Walker, U. A., Manolaraki, C., Guiducci, S., Truchetet, M. -E., Iannone, F., Tofani, L., Bruni, C., Smith, V., Cuomo, G., Krusche, M., Matucci-Cerinic, M., Allanore, Y.
Rok vydání: 2023
Předmět:
Zdroj: Journal of Scleroderma and Related Disorders. :239719832311559
ISSN: 2397-1991
2397-1983
DOI: 10.1177/23971983231155948
Popis: Background: Several studies described the cross-sectional characteristics of systemic sclerosis patients and coexisting primary biliary cholangitis, but longitudinal prognostic data are lacking. Aims: To describe the systemic sclerosis–primary biliary cholangitis phenotype, including baseline characteristics and outcomes. Methods: 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. Results: 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 ( p = 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 ( p = 0.0015). Moreover, a greater incidence of pulmonary arterial hypertension ( p Conclusion: 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.
Databáze: OpenAIRE
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