Characterization of the thymus in Lrp4 myasthenia gravis: four cases

Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction. Most patients have pathogenic autoantibodies against the acetylcholine receptor (AChR). In the last years a novel subpopulation of MG patients has been described that harbors antibodies against low-density lipoprotein rec...

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Main Authors: Koneczny, Inga (Author) , Marx, Alexander (Author)
Format: Article (Journal)
Language:English
Published: 2019
In: Autoimmunity reviews
Year: 2018, Volume: 18, Issue: 1, Pages: 50-55
ISSN:1873-0183
DOI:10.1016/j.autrev.2018.07.011
Online Access:Resolving-System, Volltext: https://doi.org/10.1016/j.autrev.2018.07.011
Verlag: http://www.sciencedirect.com/science/article/pii/S156899721830260X
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Author Notes:Inga Koneczny, Dorit Rennspiess, Florit Marcuse, Nathalie Dankerlui, Myurgia Abdul Hamid, Marina Mané-Damas, Jos Maessen, Paul Van Schil, Abhishek Saxena, Paraskevi Zisimopoulou, Konstantinos Lazaridis, Mark Woodhall, Katerina Karagiorgou, John Tzartos, Socrates Tzartos, Marc H. De Baets, Peter C. Molenaar, Alexander Marx, Axel zur Hausen, Mario Losen, Pilar Martinez-Martinez

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520 |a Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction. Most patients have pathogenic autoantibodies against the acetylcholine receptor (AChR). In the last years a novel subpopulation of MG patients has been described that harbors antibodies against low-density lipoprotein receptor-related protein 4 (Lrp4), another postsynaptic neuromuscular antigen. In early-onset AChR MG (EOMG), the thymus plays an important role in immunopathogenesis, and early thymectomy is beneficial. It is still unknown if the thymus plays any role in Lrp4-MG. In this pilot study, we compared thymus samples from four patients with Lrp4-MG (one pre-treated with immunosuppressive drugs), four non-MG controls and five EOMG patients (not pretreated with immunosuppressive drugs). 
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