Journal
JOURNAL OF GASTROENTEROLOGY
Volume 46, Issue -, Pages 48-55Publisher
SPRINGER JAPAN KK
DOI: 10.1007/s00535-010-0323-4
Keywords
Autoimmune hepatitis; Autoimmune pancreatitis; IgG4; Histology; Sclerosing cholangitis
Categories
Funding
- Japanese Ministry of Health, Labour, and Welfare
- Shinshu University
Ask authors/readers for more resources
Immunoglobulin (Ig) G4-associated autoimmune hepatitis (AIH) is a recently identified and possibly new disease entity. However, the epidemiology and clinical features of IgG4-associated AIH remain uncertain. The aim of this study was to determine the prevalence and the clinical, serological, and histological characteristics of IgG4-associated AIH. We examined the clinical features, serum IgG4 concentration, liver biopsy histology, and IgG4-bearing plasma cell infiltration of 60 patients with type 1 AIH and 22 patients with autoimmune pancreatitis. High serum IgG4 concentration (a parts per thousand yen135 mg/dL) and IgG4-bearing plasma cell infiltration in the liver (a parts per thousand yen10/high-power fields [HPFs]) were found in 2 of the 60 (3.3%) patients with type 1 AIH. These patients had high serum levels of IgE, giant cell change, and rosette formation in the liver. Although corticosteroid therapy reduced the serum IgG4 concentration and normalized liver enzymes and histology, one patient developed IgG4-related sclerosing cholangitis after 5 years of follow-up. Because IgG4-associated AIH was found in over 3% of Japanese patients with type 1 AIH in our cohort, further studies are needed on this possible new disease entity and its impact on the diagnostic guidelines of AIH.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available