Autoimmune cholangitis (AC) is a recently proposed entity that describes a specific group of patients presenting overlapping features of primary biliary cirrhosis (PBC) and autoimmune hepatitis. The disease is characterized by clinical cholestasis, high titer antinuclear antibody, negative antimitochondrial antibody, and histolologically, findings of PBC coexisting with varying degrees of parenchymal inflammation. In this report, we describe a patient with Sjögren's syndrome who fulfilled the diagnostic criteria of AC associated with unique arthropathy compatible with arthritis of PBC. This case illustrates the unusual coexistence of two diseases that may share similar pathogenic processes.
- Autoimmune cholangitis
- Sjögren's syndrome