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Extrahepatic autoimmunity in autoimmune liver disease

Published:October 22, 2018DOI:https://doi.org/10.1016/j.ejim.2018.10.014

      Highlights

      • Patients with an autoimmune liver disease have a high probability to have an association with at least another autoimmune condition
      • The most important extrahepatic autoimmune conditions for AIH are: thyroid and celiac diseases; for PBC: rheumatologic diseases, and Hashimoto's thyroiditis; for PSC: IBD
      • A screening for TSH and an accurate physical examination should be performed in all patients with PBC
      • A screening for AMA should be performed in all patients presenting to rheumatologists with Sjogren's syndrome, scleroderma, CREST, rheumatoid arthritis

      Abstract

      The most important autoimmune liver disease include: autoimmune hepatitis, primary biliary cholangitis and primary sclerosing cholangitis. In general, about one in three patients with an autoimmune liver disease have a concomitant extrahepatic autoimmune disease, which may include rheumatological, endocrinological, gastrointestinal, pulmonary or dermatological conditions. The pathogenesis of these conditions includes the production of both innate and adaptive immune responses targeting cholangiocytes as well as different extrahepatic tissues. In this sense, extrahepatic autoimmunity represent a continuous spectrum of autoimmunity involving liver and extrahepatic tissues. This review aims to focus the clinical and pathophysiological aspects of extrahepatic autoimmunity associated to autoimmune liver diseases.

      Keywords

      Abbreviation:

      AIH (autoimmune hepatitis), AITD (autoimmune thyroid diseases), ASC (autoimmune sclerosing cholangitis), CD (Crohn's disease), IBD (inflammatory bowel disease), PBC (primary biliary cholangitis), PSC (primary sclerosing cholangitis), RA (rheumatoid arthritis), SLE (systemic lupus erythematosus), SS (Sjogren's syndrome), SSc (Systemic sclerosis), UC (ulcerative colitis)
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