Liver involvement in patients with primary Sjogren's syndrome is rare, usually without clinical significance and histologically characterized by a feature like stage 1 primary biliary cirrhosis. We describe herein a case of acute and severe autoimmune hepatitis in a patient suffering from primary Sjogren's syndrome. The diagnosis of Sjogren's syndrome was performed in 1989. In June 1995 the patient presented severe weakness, jaundice and elevation of transaminases; 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. Antimitochondrial antibodies, anti-smooth muscle antibodies and antibodies against liver kidney microsomes were negative. An abdomen ultrasound examination revealed hepatomegaly, with irregular echogenic structure and lymphoadenomegaly near the celiac tripod. Liver biopsy demonstrated a picture of autoimmune hepatitis. The patient was treated with prednisone 50 mg/day and azathioprine 50 mg/day, with improvement in clinical and liver 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.

Sjogren's syndrome associated with autoimmune hepatitis. A case report

BIASI, Domenico;BAMBARA, Lisa Maria
1997

Abstract

Liver involvement in patients with primary Sjogren's syndrome is rare, usually without clinical significance and histologically characterized by a feature like stage 1 primary biliary cirrhosis. We describe herein a case of acute and severe autoimmune hepatitis in a patient suffering from primary Sjogren's syndrome. The diagnosis of Sjogren's syndrome was performed in 1989. In June 1995 the patient presented severe weakness, jaundice and elevation of transaminases; 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. Antimitochondrial antibodies, anti-smooth muscle antibodies and antibodies against liver kidney microsomes were negative. An abdomen ultrasound examination revealed hepatomegaly, with irregular echogenic structure and lymphoadenomegaly near the celiac tripod. Liver biopsy demonstrated a picture of autoimmune hepatitis. The patient was treated with prednisone 50 mg/day and azathioprine 50 mg/day, with improvement in clinical and liver 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.
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11562/1464
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus ND
  • ???jsp.display-item.citation.isi??? ND
social impact