Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis
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 evidenc...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2011-01-01
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| Series: | Annals of Indian Academy of Neurology |
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| Online Access: | http://www.annalsofian.org/article.asp?issn=0972-2327;year=2011;volume=14;issue=4;spage=316;epage=318;aulast=Lorenzoni |
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| author | P J Lorenzoni R H Scola C. S. K. Kay D A Muzzillo L C Werneck |
| author_facet | P J Lorenzoni R H Scola C. S. K. Kay D A Muzzillo L C Werneck |
| author_sort | P J Lorenzoni |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-14T13:28:55Z |
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| id | doaj.art-7c03a7ab127b4ccf9cc221d2122691e2 |
| institution | Directory Open Access Journal |
| issn | 0972-2327 1998-3549 |
| language | English |
| last_indexed | 2024-12-14T13:28:55Z |
| publishDate | 2011-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Annals of Indian Academy of Neurology |
| spelling | doaj.art-7c03a7ab127b4ccf9cc221d2122691e22022-12-21T22:59:46ZengWolters Kluwer Medknow PublicationsAnnals of Indian Academy of Neurology0972-23271998-35492011-01-0114431631810.4103/0972-2327.91966Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravisP J LorenzoniR H ScolaC. S. K. KayD A MuzzilloL C WerneckMyasthenia 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.http://www.annalsofian.org/article.asp?issn=0972-2327;year=2011;volume=14;issue=4;spage=316;epage=318;aulast=LorenzoniAutoimmune diseasesmyasthenia gravissclerosing cholangitisthymectomy |
| spellingShingle | P J Lorenzoni R H Scola C. S. K. Kay D A Muzzillo L C Werneck Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis Annals of Indian Academy of Neurology Autoimmune diseases myasthenia gravis sclerosing cholangitis thymectomy |
| title | Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis |
| title_full | Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis |
| title_fullStr | Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis |
| title_full_unstemmed | Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis |
| title_short | Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis |
| title_sort | coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis |
| topic | Autoimmune diseases myasthenia gravis sclerosing cholangitis thymectomy |
| url | http://www.annalsofian.org/article.asp?issn=0972-2327;year=2011;volume=14;issue=4;spage=316;epage=318;aulast=Lorenzoni |
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