SJOGRENS-SYNDROME ASSOCIATED WITH AUTOIMMUNE HEPATITIS - A CASE-REPORT

Liver involvement in patients with primary Sjogren's syndrome is rare, usually without clinical significance and histologically characterize d by a feature like stage 1 primary biliary cirrhosis. We describe her ein a case of acute and severe autoimmune hepatitis in a patient suffe ring from primary Sjogren's syndrome. The diagnosis of Sjogren's syndr ome was performed in 1989, In June 1995 the patient presented severe w eakness, jaundice and elevation of trans aminases; moreover IgG raised to 5560 mg/dl and ANA titre increased to 1:20480. The patient denied alcohol and drug use and a viral hepatitis was excluded. Antimitochond rial antibodies, anti-smooth muscle antibodies and antibodies against liver kidney microsomes were negative. An abdomen ultrasound examinati on revealed hepatomegaly, with irregular echogenic structure and lymph oadenomegaly near the celiac tripod, Liver biopsy demonstrated a pictu re of autoimmune hepatitis. The patient was treated with prednisone 50 mg/day and azathioprine 50 mg/day, with improvement in clinical and l iver function indices, At present, the patient is given only 10 mg/day of prednisone. The association of Sjogren's syndrome with autoimmune hepatitis is very rare: in the literature only one other similar case has been reported.