Granulomatous hepatitis and Sjogren's syndrome: an association

Sjogren's syndrome (SS) is a common autoimmune disorder in which liver involvement is frequent, but generally mild and subclinical. Multiple hepatic histologies have been reported, but to our knowledge an association with granulomatous hepatitis (GH) has never been described. We recently evaluated an individual in whom biopsy-proven GH was associated with concomitant SS.

To clarify the possible association between GH and SS.

We retrospectively reviewed all cases of biopsy-proven GH seen in our institution from 1991 to 2004. Overall, there were 16 individuals with GH identified of which 4 were considered idiopathic in origin. These individuals underwent an extensive evaluation for the presence of SS as well as other disorders known to be associated with GH.

Of the 4 identified cases with a previously suspected idiopathic GH, 3 met criteria for primary SS. All 3 individuals underwent minor salivary gland biopsy, which was diagnostic for this condition.

These results suggest a likely association between these 2 conditions. Further epidemiological studies will be necessary to confirm this finding.