[Prognostic implications of extra-hepatic clinical manifestations, autoimmunity and microscopic nail capillaroscopy in patients with primary biliary cirrhosis].

Abstract:

BACKGROUND AND OBJECTIVES:Primary biliary cirrhosis (PBC) is associated to any systemic autoimmune disease (SAD), in particular systemic sclerosis (SSc). To investigate the prevalence of SAD in a cohort of patients with PBC, specifically the prevalence of SSc and its clinical subtypes, and determining the clinical and biological profile of patients with associated PBC and SSc. METHODS:Observational study of 62 patients with PBC following a protocol that included an anamnesis and physical examination to detect the presence of SAD as well as a nailfold capillaroscopy and an immunological study with specific SSc autoantibodies. A comparative analysis was conducted between patients with isolated PBC and patients with PBC and an associated SAD. RESULTS:SAD was associated to PBC in 22 patients (35,4%), and SSc was the most frequent illness, identified in 13 cases (21%). Five patients (8%) without previous diagnosis of SAD fulfilled pre-scleroderma criteria, according to LeRoy and Medsger criteria. The presence of anticentromere antibodies (54,5% vs. 5%, P<.001) was the unique immunological determination identified more frequently in patients with PBC-SAD. The SSc suggestive capillary pattern was visualized in 11 patients (20,4%), mainly the slow pattern. No factors associated with greater morbi-mortality were identified in the PBC-SAD group. CONCLUSIONS:It does exist a subgroup of patients with PBC and clinical-biological features suggestive of an SAD, which advise a protocolized study to detect early the association to an SAD.

journal_name

Med Clin (Barc)

journal_title

Medicina clinica

authors

Marí-Alfonso B,Amengual-Guedan MJ,Vergara-Gómez M,Simeón-Aznar CP,Fonollosa-Plà V,Jove-Buxeda E,Oliva-Morera J,Tolosa-Vilella C

doi

10.1016/j.medcli.2015.02.026

subject

Has Abstract

pub_date

2016-01-01 00:00:00

pages

8-15

issue

1

eissn

0025-7753

issn

1578-8989

pii

S0025-7753(15)00191-8

journal_volume

146

pub_type

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