Clonal accumulation of V beta 5.1-positive cells in the liver of a patient with autoimmune cholangiopathy.
We describe a 43-year-old woman with clinical features compatible with autoimmune cholangiopathy recently reported by Ben Ari et al. She was negative for anti-mitochondrial antibody, positive for high titer anti-nuclear antibody with homogeneous pattern, high levels of serum immunoglobulin G and nearly normal levels of serum immunoglobulin M for more than five years. In the early stages of the disease, the elevations of serum transaminase, alkaline phosphatase and gamma-glutamyl transpeptidase were well controlled by the administration of ursodeoxycholic acid. After five years of follow-up, she showed the second exacerbation of liver function tests, which then rapidly improved by prednisone administration. To analyze the antigen diversity recognized by T-cells in the liver, T-cell receptor repertoire was examined by immuno-histochemistry. The liver biopsy obtained in the early stage showed clonal accumulation of V beta 5.1-positive cells in portal areas, which was found in patients neither with primary biliary cirrhosis nor autoimmune hepatitis. In conclusion, these data suggest that T-cell response in autoimmune cholangiopathy is different from those two autoimmune liver diseases, which may imply a distinct entity of the disease.
Shimizu
Y
Y
Third Department of Internal Medicine, Toyama Medical and Pharmaceutical University, Japan.
Higuchi
K
K
Kashii
Y
Y
Miyamoto
M
M
Tsukishiro
T
T
Watanabe
A
A
D000328:Adult; D000974:Antibodies, Antinuclear / Q000378:metabolism; D001327:Autoimmune Diseases / Q000276:immunology*; D001706:Biopsy; D002761:Cholangitis / Q000276:immunology*; D005260:Female; D006505:Hepatitis / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D007075:Immunoglobulin M / Q000378:metabolism; D007150:Immunohistochemistry; D008099:Liver / Q000276:immunology*; D008105:Liver Cirrhosis, Biliary / Q000276:immunology; D011241:Prednisone / Q000627:therapeutic use; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000378:metabolism*; D013601:T-Lymphocytes / Q000378:metabolism*; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D000974:Antibodies, Antinuclear; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D016693:Receptors, Antigen, T-Cell, alpha-beta; D014580:Ursodeoxycholic Acid; D011241:Prednisone