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Semin Arthritis Rheum. 2018 Jun;47(6):849-857. doi: 10.1016/j.semarthrit.2017.10.004. Epub 2017 Oct 6.

Hepatobiliary involvement in systemic sclerosis and the cutaneous subsets: Characteristics and survival of patients from the Spanish RESCLE Registry.

Author information

1
Department of Internal Medicine, Hospital Universitario Parc Taulí, Institut d'Investigació i Innovació Parc Taulí I3PT, Sabadell, Barcelona, Spain; Department of Medicine, Universidad Autónoma de Barcelona, Barcelona, Spain.
2
Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
3
Department of Medicine, Universidad Autónoma de Barcelona, Barcelona, Spain; Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
4
Department of Internal Medicine, Hospital Universitario de Bellvitge, L'Hospitalet de Llobregat, Barcelona, Spain.
5
Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital de Cabueñes, Gijón, Asturias, Spain.
6
Department of Internal Medicine, Hospital Universitario y Politécnico La Fe, Valencia, Spain.
7
Department of Internal Medicine, Hospital Universitario Mútua de Terrassa, Terrassa, Barcelona, Spain.
8
Unit of Autoimmune Diseases, Department of Internal Medicine, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain.
9
Department of Systemic Autoimmune Diseases, Clinical Institute of Medicine and Dermatology, Hospital Universitario Clinic, Barcelona, Spain.
10
Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain.
11
Department of Internal Medicine, Hospital Universitario Virgen del Rocío, Sevilla, Spain.
12
Department of Internal Medicine, Hospital Universitario Miguel Servet, Zaragoza, Spain.
13
Department of Internal Medicine, Hospital Universitario Cruces, Barakaldo, Bizkaia, Spain.
14
Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Campus de la Salud, Complejo Universitario de Granada, Granada, Spain.
15
Unit of Systemic Autoimmune Diseases and Thrombosis, Department of Internal Medicine, Complejo Hospitalario Universitario de Vigo, Vigo, Pontevedra, Spain.
16
Department of Internal Medicine, Hospital Universitario Virgen de las Nieves, Granada, Spain.
17
Department of Internal Medicine, Complejo Asistencial Universitario de Salamanca, Salamanca, Spain.
18
Department of Internal Medicine, Hospital General San Jorge, Huesca, Spain.
19
Department of Internal Medicine, Hospital Universitario Rey Juan Carlos, Móstoles, Madrid, Spain.
20
Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Consorci Hospitalari de Vic, Vic, Barcelona, Spain.
21
Department of Internal Medicine, Hospital Universitario Reina Sofía, Córdoba, Spain.
22
Department of Internal Medicine, Hospital Universitario Parc Taulí, Institut d'Investigació i Innovació Parc Taulí I3PT, Sabadell, Barcelona, Spain; Department of Medicine, Universidad Autónoma de Barcelona, Barcelona, Spain. Electronic address: ctolosa@tauli.cat.

Abstract

OBJECTIVE:

To assess the prevalence and causes of hepatobiliary involvement (HBI) in systemic sclerosis (SSc), to investigate the clinical characteristics and prognosis of SSc patients with HBI (SSc-HBI) and without HBI (SSc-non-HBI), and to compare both groups according to the cutaneous SSc subsets.

METHODS:

In all, 1572 SSc patients were collected in the RESCLE registry up to January 2015, and all hepatobiliary disturbances were recorded. We investigated the HBI-related characteristics and survival from the entire SSc cohort and according to the following cutaneous subsets: diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc sine scleroderma (ssSSc).

RESULTS:

Out of 1572, 118 (7.5%) patients had HBI. Primary biliary cholangitis (PBC) was largely the main cause (n = 67, 4.3%), followed by autoimmune hepatitis (n = 19, 1.2%), and anti-mitochondrial negative PBC (n = 6, 0.4%). Other causes of HBI were as follows: secondary liver diseases (n = 11, 0.7%), SSc-related HBI (n = 7, 0.4%), nodular regenerative hyperplasia (n = 3, 0.2%), liver cirrhosis (n = 3, 0.2%), and HBI of unknown origin (n = 2, 0.1%). In multivariate analysis, HBI was independently associated to lesser risk of dcSSc (5.1% vs. 24.4%), and higher frequency of calcinosis (26% vs. 18%), left ventricular diastolic dysfunction (46% vs. 27%), sicca syndrome (51% vs. 29%), and anti-centromere antibodies (ACA, 73% vs. 44%). According to the cutaneous subsets, HBI was associated (1) in lcSSc, to longer time from SSc onset to diagnosis (10.8 ± 12.5 vs. 7.2 ± 9.3 years), sicca syndrome (54% vs. 33%), and ACA (80% vs. 56%); (2) in ssSSc, to sicca syndrome (44% vs. 19%), and (3) in dcSSc, no associations were found. HBI was the cause of death in 2.3% patients but the cumulative survival according to the presence or absence of HBI showed no differences.

CONCLUSIONS:

HBI prevalence in SSc is 7.5% and dcSSc is the least involved subset. PBC is the main cause of HBI. Patients with SSc-HBI exhibited specific clinical and immunologic profile. Survival is similar for SSc patients with HBI.

KEYWORDS:

Autoimmune hepatitis; Hepatobiliary involvement; Primary biliary cholangitis; SSc sine scleroderma; Survival; Systemic sclerosis

[Indexed for MEDLINE]

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