Autoimmune hepatitis in patients with primary Sjögren's syndrome: a series of two-hundred and two patients

Abstract

Based on the revised criteria of the American-European Consensus Group, we retrospectively established the diagnosis of primary or secondary Sjögren's syndrome for 202 patients referred to a Sjögren's syndrome clinic. Of these, 58 patients and 8 patients fulfilled criteria for primary and secondary Sjögren's syndrome, respectively. Of the 58 patients with primary Sjögren's syndrome, one (1.7%) had definite autoimmune hepatitis, as defined by the International Autoimmune Hepatitis Group diagnostic criteria. One additional symptomatic patient who did not fulfill criteria for primary Sjögren's syndrome had definite autoimmune hepatitis. None of the patients with secondary Sjögren's syndrome had definite autoimmune hepatitis. Two (1%) of the 194 patients with primary Sjögren's syndrome or clinical symptoms had primary biliary cirrhosis. These values are lower than those reported by prior studies with smaller patient populations and likely represent a more accurate estimate of the true prevalence of these diseases in patients with primary Sjögren's syndrome.

Keywords: Autoimmune hepatitis; Sjögren's syndrome; primary biliary cirrhosis.

Figure 1

Lymphocyte and plasma cell rich portal inflammation with a focus of mild interface activity (40× magnification)

Figure 2

Immunohistochemical staining demonstrates many plasma cells expressing surface IgG.