Abstract
Two cases of familial Sjögren's syndrome associated with chronic active 'lupoid' hepatitis were reported.
A 48-year-old female, case 1, admitted for the complaints of easy fatigability, anorexia, and dry mouth. Seroimmunologic and histological examination revealed typical chronic active 'lupoid' hepatitis associated with Sjögren's syndrome.
By the survey of familial abnormality, case 2, a 45-year-old younger sister of case 1 provided the clinical feature of Sjögren's syndrome without liver disease.
Familial Sjögren's syndrome is rare and eighteen families were reported until now. In those reports, systemic lupus erythematosus, progressive systemic sclerosis, rheumatoid arthritis, sclerosing cholangitis, and Hashimoto's thyroiditis are associated with Sjögren's syndrome, the association of chronic active 'lupoid' hepatitis reported here is not described.
