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

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 (

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.

Informations de copyright

© The Author(s) 2023.

Déclaration de conflit d'intérêts

The author(s) declared the following potential conflicts of interest with respect to the research, authorship and/or publication of this article: G.L., P.A., O.D., K.A., Y.B.-M., E.H., A.B.-G., E.D.L., S.R., F.I., E.R., L.G., R.I., S.B.-R., L.G., L.B., C.B., S.M., P.N., I.S., D.K., V.C., U.A.W., C.M., S.G., M.-E.T., L.T., G.C., M.K., M.M.-C. and Y.A. have no conflict of interests (COIs) to declare. F.I. received honoraria or speaking fees from AbbVie, BMS, Galapagos, MSD, Lilly and Pfizer outside this work. C.B. received consulting fees and/or honoraria from Actelion and Boehringer Ingelheim; research grants from the Gruppo Italiano Lotta alla Sclerodermia (GILS), the European Scleroderma Trials and Research Group (EUSTAR) and the Scleroderma Clinical Trials Consortium (SCTC); educational grants from AbbVie; all outside the submitted work. V.S. is the Senior Clinical Investigator of the Research Foundation – Flanders (Belgium) (FWO) [1.8.029.20N]. The FWO was not involved in study design, collection, analysis and interpretation of data and writing of the report, nor in the decision to submit the manuscript for publication.