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 clinicaauthors
Marí-Alfonso B,Amengual-Guedan MJ,Vergara-Gómez M,Simeón-Aznar CP,Fonollosa-Plà V,Jove-Buxeda E,Oliva-Morera J,Tolosa-Vilella Cdoi
10.1016/j.medcli.2015.02.026subject
Has Abstractpub_date
2016-01-01 00:00:00pages
8-15issue
1eissn
0025-7753issn
1578-8989pii
S0025-7753(15)00191-8journal_volume
146pub_type
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