title: Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis.
abstract: 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.
authors.affiliation: Neurology Division, Department of Internal Medicine, Federal University of Paraná, Curitiba PR, Brazil.
authors.forename: P J
authors.initials: PJ
authors.lastname: Lorenzoni
authors.forename: R H
authors.initials: RH
authors.lastname: Scola
authors.forename: C S K
authors.initials: CS
authors.lastname: Kay
authors.forename: D A
authors.initials: DA
authors.lastname: Muzzillo
authors.forename: L C
authors.initials: LC
authors.lastname: Werneck