Regular Paper
Autoimmune liver disease in patients with primary Sjögren's syndrome

https://doi.org/10.1016/S0168-8278(94)80007-3Get rights and content

Forty-five patients with primary Sjögren's syndrome were studied for evidence of autoimmune liver disease. Twenty-nine patients had normal liver function tests, normal IgM and a normal test for antimitochondrial antibodies and smooth muscle antibodies. Among the remaining 16 patients, abnormal liver function tests were found in 12 (27% of all patients); eight of these patients had biochemical cholestasis. Elevated plasma IgM (>2 g/l) was observed in nine patients, three with normal liver function tests, while antimitochondrial antibodies were positive in six patients, one with normal liver function tests, and smooth muscle antibodies were found in three. Based on these findings and percutaneous liver biopsy, a diagnosis of primary biliary cirrhosis was established in four patients and autoimmune chronic active hepatitis in two. The present study showed that abnormal liver function tests in patients with primary Sjögren's syndrome are frequent and may indicate associated autoimmune liver disease. These data further emphasize the systemic nature of autoimmune disorders.

References (24)

  • ErikssonS et al.

    The prevalence and clinical spectrum of primary biliary cirrhosis in a defined population

    Scand J Gastroenterol

    (1984)
  • Alarcon-SegoviaD et al.

    Features of Sjögren's syndrome in primary biliary cirrhosis

    Ann Intern Med

    (1973)
  • Cited by (72)

    • Liver in Systemic Disease

      2023, MacSween's Pathology of the Liver, Eighth Edition
    • Multiple autoimmunity in Sjögren's syndrome

      2020, Revista Colombiana de Reumatologia
    • Rheumatologic Diseases and the Liver

      2019, Clinics in Liver Disease
    • The Liver in Systemic Disease

      2018, MacSween's Pathology of the Liver
    • Digestive Involvement in Primary Sjögren's Syndrome

      2017, Handbook of Systemic Autoimmune Diseases
      Citation Excerpt :

      The differing results obtained by these two studies suggest that further research is needed to evaluate whether the prevalence of HBV infection in Asian patients with primary SS is higher or lower with respect to the prevalence of HBV infection found in the general population of the same geographical area. After discarding HCV infection, primary biliary cholangitis (formerly PBC) should be considered as the main cause of liver disease in patients with primary SS [72,73,77]. Although historically these patients have been considered as having a “secondary” SS, it seems more rational to use the term “SS associated with PBC,” due to the clinical-based evidence that SS is associated with (and not secondary to) other autoimmune diseases.

    • Polyautoimmunity in Sjögren Syndrome

      2016, Rheumatic Disease Clinics of North America
      Citation Excerpt :

      AIH is diagnosed in 1.7% to 4% of patients with SS.55 AIH is the second most frequently observed autoimmune liver disease in SS (all reported cases are type I), and nearly 10% of these patients may have AIH-PBC.56 Only 10% of SS-related type I AIH patients may have positive AMA (AIH-PBC); therefore, AMA could discriminate between PBC and AIH.52

    View all citing articles on Scopus
    View full text