PBC and related extrahepatic diseases

Best Pract Res Clin Gastroenterol. 2018 Jun-Aug:34-35:49-54. doi: 10.1016/j.bpg.2018.05.013. Epub 2018 May 23.


Patients with PBC have at least 60% of probability to have an autoimmune extrahepatic condition. The pathogenesis of these conditions includes a common mechanism involving both innate and adaptive immune responses targeting cholangiocytes and different extrahepatic tissues. The recent EASL guidelines recommend the management of these conditions, although detailed practical treatments have not been indicated. Autoimmune extrahepatic conditions may include: rheumatologic, endocrine, pulmonary, gastrointestinal, dermatologic diseases. This review aims to focus the most important extrahepatic autoimmune conditions associated to PBC with practical recommendation regarding diagnostic approach and management.

Keywords: CREST disease; Dermatological diseases; Extrahepatic autoimmune conditions; Primary biliary cholangitis; Primary biliary cirrhosis; Pulmonary diseases; Rheumatoid arthritis; Scleroderma; Sjogren's syndrome; Thyroid diseases.

Publication types

  • Review

MeSH terms

  • Arthritis, Rheumatoid / diagnosis
  • Arthritis, Rheumatoid / etiology
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / etiology*
  • CREST Syndrome / diagnosis
  • CREST Syndrome / etiology
  • Humans
  • Liver Cirrhosis, Biliary / complications*
  • Liver Cirrhosis, Biliary / diagnosis
  • Lung Diseases / diagnosis
  • Lung Diseases / etiology
  • Scleroderma, Systemic / diagnosis
  • Scleroderma, Systemic / etiology
  • Sjogren's Syndrome / diagnosis
  • Sjogren's Syndrome / etiology
  • Skin Diseases / diagnosis
  • Skin Diseases / etiology
  • Thyroid Diseases / diagnosis
  • Thyroid Diseases / etiology