TY - JOUR
T1 - Prognostic implications of extra-hepatic clinical manifestations, autoimmunity and microscopic nail capillaroscopy in patients with primary biliary cirrhosis
AU - Vergara-Gómez, Mercè
AU - Tolosa-Vilella, Carles
AU - Simeón-Aznar, Carmen Pilar
AU - Amengual-Guedan, María José
AU - Fonollosa-Plà, Vicente
AU - Marí-Alfonso, Begoña
AU - Oliva-Morera, Juan
AU - Jove-Buxeda, Esther
PY - 2016/1/1
Y1 - 2016/1/1
N2 - © 2015 Elsevier España, S.L.U. 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.
AB - © 2015 Elsevier España, S.L.U. 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.
KW - Autoimmunity
KW - Primary biliary cirrhosis
KW - Capillaroscopy
KW - Systemic sclerosis
KW - Systemic autoimmune disease
UR - https://dialnet.unirioja.es/servlet/articulo?codigo=5308594
U2 - 10.1016/j.medcli.2015.02.026
DO - 10.1016/j.medcli.2015.02.026
M3 - Article
SN - 0025-7753
VL - 146
SP - 8
EP - 15
JO - Medicina Clinica
JF - Medicina Clinica
IS - 1
ER -