Sjögren’s Syndrome Complicated with Type 2 Autoimmune Hepatitis

Abstract

This case details a 33-year-old female who presented with a suggestive autoimmune history, arthralgias, and splenomegaly, and tested positive for Sjögren’s syndrome and anti-liver–kidney microsomal antibody. This was further validated by findings from a liver biopsy, confirming a very rare association with Type 2 autoimmune hepatitis. Primary Sjögren's syndrome is a sporadic disease with a global prevalence of 61 per 100,000 people and a total prevalence of 0.4% for secondary Sjögren's syndrome. The prevalence of autoimmune hepatitis in association with primary Sjögren's syndrome is 4–47%. It is divided into two types, associated with characteristic antibodies. Type 2 autoimmune hepatitis is rarely reported with Sjögren’s syndrome; much of the association reported in the literature has been with Type 1 autoimmune hepatitis.