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

Lepri, Gemma ; Airò, Paolo ; Distler, Oliver ; Andréasson, Kristofer LU ; Braun-Moscovici, Yolanda ; Hachulla, Eric ; Balbir-Gurman, Alexandra ; De Langhe, Ellen ; Rednic, Simona and Ingegnoli, Francesca , et al. (2023) In Journal of Scleroderma and Related Disorders 8(3). p.210-220
Abstract

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... (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.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
autoimmunity, fibrotic diseases, outcomes, overlap syndrome, primary biliary cholangitis, Systemic sclerosis
in
Journal of Scleroderma and Related Disorders
volume
8
issue
3
pages
210 - 220
publisher
SAGE Publications
external identifiers
  • pmid:37744053
  • scopus:85152269676
ISSN
2397-1983
DOI
10.1177/23971983231155948
language
English
LU publication?
yes
id
15488b5b-1bfb-42df-9d86-fd0094c8213f
date added to LUP
2023-07-21 09:39:38
date last changed
2024-04-19 23:51:23
@article{15488b5b-1bfb-42df-9d86-fd0094c8213f,
  abstract     = {{<p>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 &lt; 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.</p>}},
  author       = {{Lepri, Gemma and Airò, Paolo and Distler, Oliver and Andréasson, Kristofer and Braun-Moscovici, Yolanda and Hachulla, Eric and Balbir-Gurman, Alexandra and De Langhe, Ellen and Rednic, Simona and Ingegnoli, Francesca and Rosato, Edoardo and Groseanu, Laura and Ionescu, Ruxandra and Bellando-Randone, Silvia and Garzanova, Liudmila and Beretta, Lorenzo and Bellocchi, Chiara and Moiseev, Sergey and Novikov, Pavel and Szabo, Iulia and Krasowska, Dorota and Codullo, Veronica and Walker, Ulrich A. and Manolaraki, Chrysoula and Guiducci, Serena and Truchetet, Marie Elise and Iannone, Florenzo and Tofani, Lorenzo and Bruni, Cosimo and Smith, Vanessa and Cuomo, Giovanna and Krusche, Martin and Matucci-Cerinic, Marco and Allanore, Yannick}},
  issn         = {{2397-1983}},
  keywords     = {{autoimmunity; fibrotic diseases; outcomes; overlap syndrome; primary biliary cholangitis; Systemic sclerosis}},
  language     = {{eng}},
  number       = {{3}},
  pages        = {{210--220}},
  publisher    = {{SAGE Publications}},
  series       = {{Journal of Scleroderma and Related Disorders}},
  title        = {{Systemic sclerosis and primary biliary cholangitis : Longitudinal data to determine the outcomes}},
  url          = {{http://dx.doi.org/10.1177/23971983231155948}},
  doi          = {{10.1177/23971983231155948}},
  volume       = {{8}},
  year         = {{2023}},
}