title: Autoimmune hepatitis in primary Sjogren's syndrome: pathological study of the livers and labial salivary glands in 17 patients with primary Sjogren's syndrome.
abstract: Although primary Sjogren's syndrome (pSS) is an autoimmune exocrinopathy, the involvement of liver has been reported. Because no study focusing on autoimmune hepatitis (AIH) in pSS has been published, the purpose of the present study was to perform a clinical and histological examination of the liver, focusing on AIH, in 17 pSS patients. The patients had liver enzyme abnormalities without hepatitis virus infection. In all cases, biopsied livers were examined, and in 10 cases biopsied labial salivary glands were also examined histologically. Based on the authors' diagnostic criteria for AIH in pSS, the liver diseases consisted of AIH (eight cases, 47%), primary biliary cirrhosis (PBC; six cases, 35%), non-specified chronic hepatitis (two cases, 12%) and acute hepatitis (one case, 6%). Lymphoplasmacytic infiltrate, with predominancy of CD3(+) T cells, was noted in both the liver and salivary glands in the patients with AIH. The patients with AIH with severe interface hepatitis had a good response to immunosuppressive therapy. The comparison of liver histology between the PBC with pSS group and the PBC without pSS group showed that the incidence of lymphoid non-suppurative cholangitis was higher in PBC with pSS. In conclusion, the present study offers new information on the relatively common occurrence, diagnostic criteria and treatment effects of AIH in pSS.
authors.affiliation: First Department of Pathology, Juntendo University, School of Medicine, Hongo 2-1-1, Bunkyo-ku, Tokyo 113-8421, Japan. machian@med.juntendo.ac.jp
authors.forename: Toshiharu
authors.initials: T
authors.lastname: Matsumoto
authors.forename: Toshio
authors.initials: T
authors.lastname: Morizane
authors.forename: Yuuji
authors.initials: Y
authors.lastname: Aoki
authors.forename: Shigetaka
authors.initials: S
authors.lastname: Yamasaki
authors.forename: Mikako
authors.initials: M
authors.lastname: Nakajima
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Enomoto
authors.forename: Shigeto
authors.initials: S
authors.lastname: Kobayashi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hashimoto
mesh_terms: D000328:Adult; D000368:Aged; D000410:Alanine Transaminase / Q000097:blood; D015415:Biomarkers; D001706:Biopsy; D017252:CD3 Complex / Q000378:metabolism; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D006521:Hepatitis, Chronic / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007124:Immunoenzyme Techniques; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000378:metabolism / Q000473:pathology*; D008105:Liver Cirrhosis, Biliary / Q000378:metabolism / Q000473:pathology; D008875:Middle Aged; D012470:Salivary Glands, Minor / Q000378:metabolism / Q000473:pathology*; D012859:Sjogren's Syndrome / Q000150:complications / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D013601:T-Lymphocytes / Q000378:metabolism / Q000473:pathology
chemical_list: D015415:Biomarkers; D017252:CD3 Complex; D007166:Immunosuppressive Agents; D000410:Alanine Transaminase