Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis.
Ann Indian Acad Neurol
; 14(4): 316-8, 2011 Oct.
Article
em En
| MEDLINE
| ID: mdl-22346028
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Idioma:
En
Revista:
Ann Indian Acad Neurol
Ano de publicação:
2011
Tipo de documento:
Article
País de afiliação:
Brasil