Title :

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

Author(s) :

Lepri, G. [Auteur]
Airò, P. [Auteur]
Distler, O. [Auteur]
Andréasson, K. [Auteur]
Braun-Moscovici, Y. [Auteur]
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Balbir-Gurman, A. [Auteur]
De Langhe, E. [Auteur]
Rednic, S. [Auteur]
Ingegnoli, F. [Auteur]
Rosato, E. [Auteur]
Groseanu, L. [Auteur]
Ionescu, R. [Auteur]
Bellando-Randone, S. [Auteur]
Garzanova, L. [Auteur]
Beretta, L. [Auteur]
Bellocchi, C. [Auteur]
Moiseev, S. [Auteur]
Novikov, P. [Auteur]
Szabo, I. [Auteur]
Krasowska, D. [Auteur]
Codullo, V. [Auteur]
Walker, U. A. [Auteur]
Manolaraki, C. [Auteur]
Guiducci, S. [Auteur]
Truchetet, M. E. [Auteur]
Iannone, F. [Auteur]
Tofani, L. [Auteur]
Bruni, C. [Auteur]
Smith, V. [Auteur]
Cuomo, G. [Auteur]
Krusche, M. [Auteur]
Matucci-Cerinic, M. [Auteur]
Allanore, Y. [Auteur]

Journal title :

Journal of Scleroderma and Related Disorders

Abbreviated title :

J Scleroderma Relat Disord

Volume number :

8

Pages :

210-220

Publication date :

2023-09-26

ISSN :

2397-1991

English keyword(s) :

Systemic sclerosis
primary biliary cholangitis
outcomes
fibrotic diseases
overlap syndrome
autoimmunity

English abstract : [en]

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 ...
Show more >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 < 0.001) and of conduction blocks (p = 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. 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.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-02-06T22:27:03Z
2024-03-15T10:11:46Z