Open AccessCoexistence of primary sclerosing cholangitis in a patient with myasthenia gravis
Author(s) -
PJ Lorenzoni,
R.H. Scola,
Cláudia Suemi Kamoi Kay,
DA Muzzillo,
Werneck Lc
Publication year - 2011
Publication title -
annals of indian academy of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.427
H-Index - 31
eISSN - 1998-3549
pISSN - 0972-2327
DOI - 10.4103/0972-2327.91966
Subject(s) - primary sclerosing cholangitis , medicine , myasthenia gravis , autoantibody , cholestasis , thymectomy , autoimmune hepatitis , antibody , acetylcholine receptor , pathology , gastroenterology , serology , liver biopsy , primary biliary cirrhosis , edrophonium , pathogenesis , immunology , biopsy , disease , receptor
Myasthenia gravis (MG) is an immune-mediated disease that compromises the postsynaptic membrane of the neuromuscular junction. Primary sclerosing cholangitis (PSC) is considered an immune-mediated cholestatic liver disease. Both MG and PSC include an autoimmune pathogenesis, so there is some evidence that patients with MG or PSC have a higher risk of developing autoantibodies and other immune disorders than normal controls, but the coexistence of these two disorders has never been documented. We report a 40-year-old woman who presented with MG when she was 20 years old and developed PSC 20 years after a thymectomy. Liver biochemistry revealed cholestasis. Magnetic resonance imaging showed multifocal strictures and beads involving the intrahepatic bile ducts. A liver biopsy confirmed sclerosing cholangitis. Serological analysis demonstrated positive autoantibodies (Anti-nuclear antibodies, anti-smooth muscle antibodies). Repetitive stimulation had a decremental response, and antibodies to acetylcholine receptors were detectable. To our knowledge, this is the first case of PSC in a patient with MG. The main characteristics of both MG and PSC combination are discussed.