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: P J Lorenzoni, R H Scola, C S K Kay, D A Muzzillo, L C Werneck
• Journal: Annals of Indian Academy of Neurology (Ann Indian Acad Neurol) Vol. 14 Issue 4 Pg. 316-8 (Oct 2011) ISSN: 1998-3549 [Electronic] India
• PMID: 22346028 (Publication Type: Case Reports)