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DOI 10.1038/s41598-024-63162-0

Медицинская библиотека · Scientific reports · 2024

Comparison of juvenile and adult myasthenia gravis in a French cohort with focus on thymic histology.

Truffault Frédérique, Auger Ludivine, Dragin Nadine, Vilquin Jean-Thomas, Fadel Elie, Thomas de Montpreville Vincent, Mansuet-Lupo Audrey, Regnard Jean-François, Alifano Marco, Sharshar Tarek, Behin Anthony, Eymard Bruno, Bolgert Francis, Demeret Sophie, Berrih-Aknin Sonia, Le Panse Rozen

СтатьяСравнительное исследованиеПеревод в очереди

Аннотация

Myasthenia gravis (MG) is an autoimmune disease characterized by muscle fatigability due to acetylcholine receptor (AChR) autoantibodies. To better characterize juvenile MG (JMG), we analyzed 85 pre- and 132 post-pubescent JMG (with a cutoff age of 13) compared to 721 adult MG patients under 40 years old using a French database. Clinical data, anti-AChR antibody titers, thymectomy, and thymic histology were analyzed. The proportion of females was higher in each subgroup. No significant difference in the anti-AChR titers was observed. Interestingly, the proportion of AChR+ MG patients was notably lower among adult MG patients aged between 30 and 40 years, at 69.7%, compared to over 82.4% in the other subgroups. Thymic histological data were examined in patients who underwent thymectomy during the year of MG onset. Notably, in pre-JMG, the percentage of thymectomized patients was significantly lower (32.9% compared to more than 42.5% in other subgroups), and the delay to thymectomy was twice as long. We found a positive correlation between anti-AChR antibodies and germinal center grade across patient categories. Additionally, only females, particularly post-JMG patients, exhibited the highest rates of lymphofollicular hyperplasia (95% of cases) and germinal center grade. These findings reveal distinct patterns in JMG patients, particularly regarding thymic follicular hyperplasia, which appears to be exacerbated in females after puberty.

Для цитирования: Truffault Frédérique, Auger Ludivine, Dragin Nadine, Vilquin Jean-Thomas, Fadel Elie, Thomas de Montpreville Vincent, Mansuet-Lupo Audrey, Regnard Jean-François, Alifano Marco, Sharshar Tarek, Behin Anthony, Eymard Bruno, Bolgert Francis, Demeret Sophie, Berrih-Aknin Sonia, Le Panse Rozen. Comparison of juvenile and adult myasthenia gravis in a French cohort with focus on thymic histology.. Scientific reports. 2024. DOI: 10.1038/s41598-024-63162-0.

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