Systemic sclerosis and primary biliary cholangitis: Longitudinal data to determine the outcomes-Reference-Cited by-同舟云学术

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

Published:2023-04-04 Issue:3 Volume:8 Page:210-220
ISSN:2397-1983
Container-title:Journal of Scleroderma and Related Disorders
language:en
Short-container-title:Journal of Scleroderma and Related Disorders

Author:

Lepri Gemma¹^ORCID,Airò Paolo²^ORCID,Distler Oliver³,Andréasson Kristofer⁴^ORCID,Braun-Moscovici Yolanda⁵^ORCID,Hachulla Eric⁶,Balbir-Gurman Alexandra⁵,De Langhe Ellen⁷,Rednic Simona⁸,Ingegnoli Francesca⁹,Rosato Edoardo¹⁰,Groseanu Laura¹¹,Ionescu Ruxandra¹¹,Bellando-Randone Silvia¹,Garzanova Liudmila¹²^ORCID,Beretta Lorenzo¹³,Bellocchi Chiara¹³^ORCID,Moiseev Sergey¹⁴,Novikov Pavel¹⁴,Szabo Iulia⁸,Krasowska Dorota¹⁵,Codullo Veronica¹⁶,Walker Ulrich A.¹⁷,Manolaraki Chrysoula¹⁷,Guiducci Serena¹,Truchetet Marie-Elise¹⁸,Iannone Florenzo¹⁹^ORCID,Tofani Lorenzo¹,Bruni Cosimo¹³^ORCID,Smith Vanessa²⁰,Cuomo Giovanna²¹^ORCID,Krusche Martin²²,Matucci-Cerinic Marco¹¹³,Allanore Yannick²³

Affiliation:

1. Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

2. Rheumatology and Clinical Immunology, Spedali Civili of Brescia, Brescia, Italy

3. Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland

4. Section of Rheumatology, Department of Clinical Sciences, Lund University, Lund, Sweden

5. Rheumatology Department, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel

6. Department of Internal Medicine, Hôpital Claude Huriez, Lille, France

7. ERN ReCONNET, Division of Rheumatology, University Hospitals Leuven, Leuven, Belgium

8. Department of Rheumatology, Emergency County Teaching Hospital, University of Medicine and Pharmacy Iuliu Hatieganu, Cluj-Napoca, Romania

9. Clinical Rheumatology Unit, ASST Pini-CTO, Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milano, Italy

10. Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy

11. Carol Davila University of Medicine and Pharmacy, Bucharest, Romania

12. Laboratory of Microcirculation and Inflammation, VA Nasonova Institute of Rheumatology, Moscow, Russian Federation

13. Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, La Fondazione IRCCS Ca’ Granda Ospedale Maggiore di Milano Policlinico, Milano, Italy

14. Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russian Federation

15. Department of Dermatology, Venereology and Pediatric Dermatology, Medical University of Lublin, Lublin, Poland

16. Rheumatology, Policlinico San Matteo, Pavia, Italy

17. Department of Rheumatology, Universitätsspital Basel, Basel, Switzerland

18. Rheumatology Department, Bordeaux University Hospital, Bordeaux, France

19. Rheumatology Unit – DETO, School of Medicine, University of Bari, Bari, Italy

20. Department of Rheumatology, Ghent University Hospital and Department of Internal Medicine, Ghent University, Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium

21. Department of Precision of Medicine, University of Campania – L. Vanvitelli, Naples, Italy

22. Division of Rheumatology and Systemic Inflammatory Diseases, University Hospital Hamburg-Eppendorf (UKE), Hamburg, Germany

23. Rheumatology, Cochin Hospital, APHP, Paris Cité University, Paris, France

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 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.

Publisher

SAGE Publications

Subject

Immunology,Rheumatology,Immunology and Allergy

Link

http://journals.sagepub.com/doi/pdf/10.1177/23971983231155948

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