Annals of Indian Academy of Neurology
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Year : 2011  |  Volume : 14  |  Issue : 4  |  Page : 316-318

Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis

1 Neurology Division, Department of Internal Medicine, Federal University of Paraná, Curitiba PR, Brazil
2 Gastroenterology Division, Department of Internal Medicine, Federal University of Paraná, Curitiba PR, Brazil

Correspondence Address:
R H Scola
Serviço de Neurologia, Hospital de Clínicas da UFPR, Rua General Carneiro 181/3o andar, 80060-900 Curitiba PR
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-2327.91966

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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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