Myasthenia gravis causes: Difference between revisions
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==Causes== | ==Causes== | ||
Myasthenia gravis may be caused by: | Myasthenia gravis may be caused by: | ||
* Thymus abnormalities: Thymus abnormalities including thymic hyperplasia and [[thymoma]] are very common in myasthenia gravis and [[thymectomy]] is one of the treatment of this disease.<ref name="pmid8190158">{{cite journal |vauthors=Drachman DB |title=Myasthenia gravis |journal=N. Engl. J. Med. |volume=330 |issue=25 |pages=1797–810 |date=June 1994 |pmid=8190158 |doi=10.1056/NEJM199406233302507 |url=}}</ref><ref name="pmid12360217">{{cite journal |vauthors=Vincent A |title=Unravelling the pathogenesis of myasthenia gravis |journal=Nat. Rev. Immunol. |volume=2 |issue=10 |pages=797–804 |date=October 2002 |pmid=12360217 |doi=10.1038/nri916 |url=}}</ref> In [[thymus]], we have myoid cells which present intact [[Acetylcholine receptor|AChR]] on their surface. On the other hand thymic epithelial cells produce [[Acetylcholine receptor|AChR]] subunits which activate [[helper T cells]]. These [[T cell|T cells]] attack [[Acetylcholine receptor|AChR]] on the myoid cells and the cascade of [[antibody]] production and [[complement]] activation will begin.<ref name="pmid18567866">{{cite journal |vauthors=Willcox N, Leite MI, Kadota Y, Jones M, Meager A, Subrahmanyam P, Dasgupta B, Morgan BP, Vincent A |title=Autoimmunizing mechanisms in thymoma and thymus |journal=Ann. N. Y. Acad. Sci. |volume=1132 |issue= |pages=163–73 |date=2008 |pmid=18567866 |doi=10.1196/annals.1405.021 |url=}}</ref><ref name="pmid17675582">{{cite journal |vauthors=Leite MI, Jones M, Ströbel P, Marx A, Gold R, Niks E, Verschuuren JJ, Berrih-Aknin S, Scaravilli F, Canelhas A, Morgan BP, Vincent A, Willcox N |title=Myasthenia gravis thymus: complement vulnerability of epithelial and myoid cells, complement attack on them, and correlations with autoantibody status |journal=Am. J. Pathol. |volume=171 |issue=3 |pages=893–905 |date=September 2007 |pmid=17675582 |pmc=1959483 |doi=10.2353/ajpath.2007.070240 |url=}}</ref><ref name="pmid18644632">{{cite journal |vauthors=Hohlfeld R, Wekerle H |title=Reflections on the "intrathymic pathogenesis" of myasthenia gravis |journal=J. Neuroimmunol. |volume=201-202 |issue= |pages=21–7 |date=September 2008 |pmid=18644632 |doi=10.1016/j.jneuroim.2008.05.020 |url=}}</ref> | * Thymus abnormalities: | ||
Thymus abnormalities including thymic hyperplasia and [[thymoma]] are very common in myasthenia gravis and [[thymectomy]] is one of the treatment of this disease.<ref name="pmid8190158">{{cite journal |vauthors=Drachman DB |title=Myasthenia gravis |journal=N. Engl. J. Med. |volume=330 |issue=25 |pages=1797–810 |date=June 1994 |pmid=8190158 |doi=10.1056/NEJM199406233302507 |url=}}</ref><ref name="pmid12360217">{{cite journal |vauthors=Vincent A |title=Unravelling the pathogenesis of myasthenia gravis |journal=Nat. Rev. Immunol. |volume=2 |issue=10 |pages=797–804 |date=October 2002 |pmid=12360217 |doi=10.1038/nri916 |url=}}</ref> In [[thymus]], we have myoid cells which present intact [[Acetylcholine receptor|AChR]] on their surface. On the other hand thymic epithelial cells produce [[Acetylcholine receptor|AChR]] subunits which activate [[helper T cells]]. These [[T cell|T cells]] attack [[Acetylcholine receptor|AChR]] on the myoid cells and the cascade of [[antibody]] production and [[complement]] activation will begin.<ref name="pmid18567866">{{cite journal |vauthors=Willcox N, Leite MI, Kadota Y, Jones M, Meager A, Subrahmanyam P, Dasgupta B, Morgan BP, Vincent A |title=Autoimmunizing mechanisms in thymoma and thymus |journal=Ann. N. Y. Acad. Sci. |volume=1132 |issue= |pages=163–73 |date=2008 |pmid=18567866 |doi=10.1196/annals.1405.021 |url=}}</ref><ref name="pmid17675582">{{cite journal |vauthors=Leite MI, Jones M, Ströbel P, Marx A, Gold R, Niks E, Verschuuren JJ, Berrih-Aknin S, Scaravilli F, Canelhas A, Morgan BP, Vincent A, Willcox N |title=Myasthenia gravis thymus: complement vulnerability of epithelial and myoid cells, complement attack on them, and correlations with autoantibody status |journal=Am. J. Pathol. |volume=171 |issue=3 |pages=893–905 |date=September 2007 |pmid=17675582 |pmc=1959483 |doi=10.2353/ajpath.2007.070240 |url=}}</ref><ref name="pmid18644632">{{cite journal |vauthors=Hohlfeld R, Wekerle H |title=Reflections on the "intrathymic pathogenesis" of myasthenia gravis |journal=J. Neuroimmunol. |volume=201-202 |issue= |pages=21–7 |date=September 2008 |pmid=18644632 |doi=10.1016/j.jneuroim.2008.05.020 |url=}}</ref> | |||
* Genetic: | * Genetic: | ||
# [[MHC|The Major Histocompatibility Complex]]: | # [[MHC|The Major Histocompatibility Complex]]: | ||
| Line 14: | Line 15: | ||
# The [[FCGR2A|FCGR2]] [[Locus]]: | # The [[FCGR2A|FCGR2]] [[Locus]]: | ||
# The [[CTLA-4|CTLA4]] [[Locus]]: | # The [[CTLA-4|CTLA4]] [[Locus]]: | ||
* environment: | |||
# Drugs: | |||
# Infections: | |||
# Trauma: | |||
==References== | ==References== | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Causes
Myasthenia gravis may be caused by:
- Thymus abnormalities:
Thymus abnormalities including thymic hyperplasia and thymoma are very common in myasthenia gravis and thymectomy is one of the treatment of this disease.[1][2] In thymus, we have myoid cells which present intact AChR on their surface. On the other hand thymic epithelial cells produce AChR subunits which activate helper T cells. These T cells attack AChR on the myoid cells and the cascade of antibody production and complement activation will begin.[3][4][5]
- Genetic:
- The Major Histocompatibility Complex:
- The CHRNA1 Locus:
- The PTPN22 Gene:
- The FCGR2 Locus:
- The CTLA4 Locus:
- environment:
- Drugs:
- Infections:
- Trauma:
References
- ↑ Drachman DB (June 1994). "Myasthenia gravis". N. Engl. J. Med. 330 (25): 1797–810. doi:10.1056/NEJM199406233302507. PMID 8190158.
- ↑ Vincent A (October 2002). "Unravelling the pathogenesis of myasthenia gravis". Nat. Rev. Immunol. 2 (10): 797–804. doi:10.1038/nri916. PMID 12360217.
- ↑ Willcox N, Leite MI, Kadota Y, Jones M, Meager A, Subrahmanyam P, Dasgupta B, Morgan BP, Vincent A (2008). "Autoimmunizing mechanisms in thymoma and thymus". Ann. N. Y. Acad. Sci. 1132: 163–73. doi:10.1196/annals.1405.021. PMID 18567866.
- ↑ Leite MI, Jones M, Ströbel P, Marx A, Gold R, Niks E, Verschuuren JJ, Berrih-Aknin S, Scaravilli F, Canelhas A, Morgan BP, Vincent A, Willcox N (September 2007). "Myasthenia gravis thymus: complement vulnerability of epithelial and myoid cells, complement attack on them, and correlations with autoantibody status". Am. J. Pathol. 171 (3): 893–905. doi:10.2353/ajpath.2007.070240. PMC 1959483. PMID 17675582.
- ↑ Hohlfeld R, Wekerle H (September 2008). "Reflections on the "intrathymic pathogenesis" of myasthenia gravis". J. Neuroimmunol. 201-202: 21–7. doi:10.1016/j.jneuroim.2008.05.020. PMID 18644632.