title: [Prognostic implications of extra-hepatic clinical manifestations, autoimmunity and microscopic nail capillaroscopy in patients with primary biliary cirrhosis].
abstract: OBJECTIVE
  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.
authors.affiliation: Servicio de Medicina Interna, Corporación Sanitaria y Universitaria Parc Tauli, Instituto Universitario Parc Taulí, Sabadell (Barcelona), España. Electronic address: bmari@tauli.cat.
authors.forename: Begoña
authors.initials: B
authors.lastname: Marí-Alfonso
authors.affiliation: Servicio de Laboratorio de Inmunología, UDIAT, Corporación Sanitaria y Universitaria Parc Taulí, Instituto Universitario Parc Taulí, Universidad Autónoma de Barcelona (UAB), Sabadell (Barcelona), España.
authors.forename: María José
authors.initials: MJ
authors.lastname: Amengual-Guedan
authors.affiliation: Servicio de Hepatología, Corporación Sanitaria y Universitaria Parc Taulí, Instituto Universitario Parc Taulí, Universidad Autónoma de Barcelona (UAB), Sabadell (Barcelona), España.
authors.forename: Mercè
authors.initials: M
authors.lastname: Vergara-Gómez
authors.affiliation: Servicio de Medicina Interna, Hospital Universitario Vall d'Hebrón, Barcelona, España.
authors.forename: Carmen Pilar
authors.initials: CP
authors.lastname: Simeón-Aznar
authors.affiliation: Servicio de Medicina Interna, Hospital Universitario Vall d'Hebrón, Barcelona, España.
authors.forename: Vicente
authors.initials: V
authors.lastname: Fonollosa-Plà
authors.affiliation: Servicio de Medicina Interna, Corporación Sanitaria y Universitaria Parc Tauli, Instituto Universitario Parc Taulí, Sabadell (Barcelona), España.
authors.forename: Esther
authors.initials: E
authors.lastname: Jove-Buxeda
authors.affiliation: Unidad de Estadística, Fundación Parc Taulí, Corporación Sanitaria y Universitaria Parc Taulí, Instituto Universitario Parc Taulí, Universidad Autónoma de Barcelona (UAB), Sabadell (Barcelona), España.
authors.forename: Juan
authors.initials: J
authors.lastname: Oliva-Morera
authors.affiliation: Servicio de Medicina Interna, Corporación Sanitaria y Universitaria Parc Tauli, Instituto Universitario Parc Taulí, Sabadell (Barcelona), España.
authors.forename: Carles
authors.initials: C
authors.lastname: Tolosa-Vilella
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D020678:Microscopic Angioscopy; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology
keywords: Autoimmunity; Autoinmunidad; Capilaroscopia; Capillaroscopy; Cirrosis biliar primaria; Enfermedad autoinmune sistémica; Esclerodermia; Primary biliary cirrhosis; Systemic autoimmune disease; Systemic sclerosis