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Inicio Medicina Clínica (English Edition) Prognostic implications of extra-hepatic clinical manifestations, autoimmunity a...
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Vol. 146. Issue 1.
Pages 8-15 (January 2016)
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Vol. 146. Issue 1.
Pages 8-15 (January 2016)
Original article
Prognostic implications of extra-hepatic clinical manifestations, autoimmunity and microscopic nail capillaroscopy in patients with primary biliary cirrhosis
Implicación pronóstica de las manifestaciones clínicas extrahepáticas, autoinmunidad y capilaroscopia ungueal microscópica en pacientes con cirrosis biliar primaria
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Begoña Marí-Alfonsoa,
Corresponding author
bmari@tauli.cat

Corresponding author.
, María José Amengual-Guedanb, Mercè Vergara-Gómezc, Carmen Pilar Simeón-Aznard, Vicente Fonollosa-Plàd, Esther Jove-Buxedaa, Juan Oliva-Morerae, Carles Tolosa-Vilellaa
a Servicio de Medicina Interna, Corporación Sanitaria y Universitaria Parc Tauli, Instituto Universitario Parc Taulí, Sabadell, Barcelona, Spain
b Servicio de Laboratorio de Inmunología, UDIAT, Corporación Sanitaria y Universitaria Parc Taulí, Instituto Universitario Parc Taulí, Universidad Autónoma de Barcelona (UAB), Sabadell, Barcelona, Spain
c Servicio de Hepatología, Corporación Sanitaria y Universitaria Parc Taulí, Instituto Universitario Parc Taulí, Universidad Autónoma de Barcelona (UAB), Sabadell, Barcelona, Spain
d Servicio de Medicina Interna, Hospital Universitario Vall d’Hebrón, Barcelona, Spain
e Unidad de Estadística, Fundación Parc Taulí, Corporación Sanitaria y Universitaria Parc Taulí, Instituto Universitario Parc Taulí, Universidad Autónoma de Barcelona (UAB), Sabadell, Barcelona, Spain
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Figures (1)
Tables (4)
Table 1. Epidemiological data and clinical characteristics of the cohort of patients with PBC.
Table 2. Extrahepatic symptoms and signs at some point of the disease. Comparative study between groups.
Table 3. Immunological study results and comparative analysis between groups: 4a PBC-no SAD group vs. PBC-SAD; 4b. PBC-SSc group vs. PBC-no SSc.
Table 4. Comparison of SAD and SSC prevalence in different PBC series.
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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<0.001) 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.

Keywords:
Primary biliary cirrhosis
Systemic autoimmune disease
Autoimmunity
Capillaroscopy
Systemic sclerosis
Resumen
Introducción y objetivos

La cirrosis biliar primaria (CBP) se asocia a algunas enfermedades autoinmunes sistémicas (EAS), en particular a la esclerosis sistémica (ES).

Determinar la prevalencia de EAS en una cohorte de pacientes con CBP, específicamente la ES y sus diferentes subtipos clínicos, y establecer el perfil clínico-biológico propio de estos pacientes.

Métodos

Estudio observacional de 62 pacientes con CBP, con un protocolo que incluía una anamnesis y exploración física dirigidas a detectar una EAS, la realización de una capilaroscopia ungueal microscópica y un amplio estudio de autoinmunidad, incluido el perfil de anticuerpos específicos de ES. Se realizó un análisis comparativo entre el grupo de pacientes con CBP aislada y los pacientes con CBP y una EAS asociada.

Resultados

Se asoció una EAS en 22 pacientes (35,4%), y la ES fue la entidad más frecuente (21%), del subtipo cutáneo limitado (11%). Cinco pacientes (8%) sin EAS previa cumplían criterios de preesclerodermia, según los criterios de LeRoy y Medsger. Los anticuerpos anticentrómero (54,5 vs. 5%, p<0,001) fueron el único parámetro inmunológico identificado con mayor frecuencia en pacientes con EAS. El patrón capilar sugestivo de ES se visualizó en 11 pacientes (20,4%). No se identificaron factores asociados a mayor morbimortalidad en ningún grupo.

Conclusiones

Existe un subgrupo de pacientes con CBP con características clínico-biológicas que sugieren la asociación con una EAS, con elevada probabilidad, y que recomiendan el estudio protocolizado de estos pacientes con CBP para detectar de forma precoz EAS.

Palabras clave:
Cirrosis biliar primaria
Enfermedad autoinmune sistémica
Autoinmunidad
Capilaroscopia
Esclerodermia

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