Premium
Spontaneous occurrence of chronic non‐suppurative destructive cholangitis and antimitochondrial autoantibodies in MRL/ lpr mice: Possible animal model for primary biliary cirrhosis
Author(s) -
Tsuneyama Koichi,
Nose Masato,
Nisihara Miyuki,
Katayanagi Kazuyoshi,
Harada Kenichi,
Nakanuma Yasuni
Publication year - 2001
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1046/j.1440-1827.2001.01223.x
Subject(s) - primary biliary cirrhosis , medicine , primary sclerosing cholangitis , pathology , biliary cirrhosis , autoantibody , bile duct , intrahepatic bile ducts , autoimmune disease , autoimmunity , cirrhosis , antibody , immunology , gastroenterology , disease
MRL/MP mice bearing the lymphoproliferative gene lpr (known as MRL/MP‐ lpr / lpr or MRL/ Ipr mice) are known to spontaneously develop severe autoimmune diseases such as glomerulonephritis, arteritis and arthritis at an early stage of their life. They have also been reported to develop severe sialadenitis, suggesting that this mouse could be a model for Sjögren’s syndrome. Primary biliary cirrhosis, an autoimmune disease characterized by chronic non‐suppurative destructive cholangitis and the occurrence of antimitochondrial antibodies, is frequently associated with Sjögren’s syndrome. In this study, we examined whether cholangitis and/or antimitochondrial antibodies occur in this mouse model, using more than 100 young and old MRL/ Ipr mice. We frequently found portal inflammation associated with cholangitis of small intrahepatic bile ducts, especially in older mice. There was also infiltration of inflammatory cells (monocytes) as well as CD4‐positive T cells. Epithelioid granuloma and bile‐duct loss were also occasionally found. These histological features resemble primary biliary cirrhosis. In addition, antimitochondrial antibodies were shown by immunocytochemistry to be present in the sera of MRL/ Ipr mice. There is currently no established animal model for primary biliary cirrhosis. Therefore, further studies on MRL/ Ipr mice, with respect to pathogenesis of primary biliary cirrhosis, are warranted.