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

Fecha de publicación:

Autores de IIS La Fe

Participantes ajenos a IIS La Fe

  • Mari-Alfonso, B
  • Pilar Simeon-Aznar, Carmen
  • Guillen-Del Castillo, A
  • Rubio-Rivas, M
  • Trapiella-Martinez, L
  • Rodriguez Carballeira, Monica
  • Marin-Ballve, A
  • Iniesta-Arandia, N
  • Colunga-Arguelles, D
  • Jesus Castillo-Palma, Maria
  • Saez-Comet, L
  • Victoria Egurbide-Arberas, Maria
  • Ortego-Centeno, N
  • Freire, M
  • Vargas Hitos, Jose Antonio
  • Chamorro, AJ
  • Belen Madronero-Vuelta, Ana
  • Perales-Fraile, I
  • Pla-Salas, X
  • Fernandez-De-La-Puebla, RA
  • Fonollosa-Pla, V
  • Tolosa-Vilella, C
  • RESCLE Investigators
  • Systemic Autoimmune Dis Study Grp

Grupos

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 23% 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 (C). 2017 Elsevier Inc. All rights reserved.

Datos de la publicación

ISSN/ISSNe:
0049-0172, 1532-866X

SEMINARS IN ARTHRITIS AND RHEUMATISM  W B SAUNDERS CO-ELSEVIER INC

Tipo:
Review
Páginas:
849-857
PubMed:
29246416
Factor de Impacto:
2,102 SCImago ℠
Cuartil:
Q1 SCImago ℠

Citas Recibidas en Web of Science: 13

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Keywords

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

Campos de Estudio

Proyectos y Estudios Clínicos

PREVENCIÓN DE EPISODIOS DE TROMBOEMBOLIA - REGISTRO EUROPEO SOBRE TROMBOEMBOLIA VENOSA.

Investigador Principal: JOSÉ ANTONIO TODOLÍ PARRA

DAI-VTE-2013-02 . 2014

ENSAYO CLINICO FASE III, DE GRUPOS PARALELOS, RANDOMIZADO, CONTROLADO CON PLACEBO, DOBLE CIEGO Y MULTICENTRICO, PARA EVALUAR LA EFICACIA Y SEGURIDAD DE LA ADMINISTRACION DE TOCILIZUMAB EN PACIENTES DIAGNOSTICADOS DE ORBITOPATÍA TIROIDEA (OT) MODERADA A SE VERA O QUE AMENAZA LA VISTA, QUE NO HAN RESPONDIDO ADECUADAMENTE AL TRATAMIENTO CON PULSOS DE CORTICOIDES.

Investigador Principal: ENRIQUE ESPAÑA GREGORI

GRC-TCL-2010-01 . 2013

ESTUDIO OBSERVACIONAL TRANSVERSAL SOBRE LA LIMITACIÓN FUNCIONAL Y DE LAS ACTIVIDADES LABORALES Y DE LA VIDA DIARIA EN PACIENTES CON ESCLEROSIS SISTÉMICA QUE PRESENTAN O NO ÚLCERAS DIGITALES - ESTUDIO LAUDES.

Investigador Principal: JOSÉ ANTONIO TODOLÍ PARRA

ACT-NUL-2012-01

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