Myasthenia Gravis and Innate Immunity—Dedicated to the Memory of Dr. Pia Bernasconi

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cellular Immunology".

Deadline for manuscript submissions: closed (30 September 2023) | Viewed by 8002

Special Issue Editors


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Guest Editor
Neurology IV–Neuroimmunology and Neuromuscular Diseases Unit, Fondazione I.R.C.C.S. Istituto Neurologico Carlo Besta, 20133 Milan, Italy
Interests: autoimmunity; inflammation; innate immunity; microRNAs; myasthenia gravis; neuroimmunology; pharmacogenetics
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Co-Guest Editor
Neurology IV - Neuroimmunology and Neuromuscular Diseases Unit, Fondazione I.R.C.C.S. Istituto Neurologico Carlo Besta, 20133 Milan, Italy
Interests: neuroimmunology; neuromuscular diseases; myasthenia gravis; multiple sclerosis; inflammatory myopathies

Special Issue Information

Dear Colleagues,

This Special Issue is dedicated to the memory of our colleague and friend Pia Bernasconi, an enthusiastic scientist with a great passion for research, whose studies significantly contributed to the understanding of the pathogenetic link between innate immunity and autoimmunity in myasthenia gravis.

A wealth of data indicates a key contribution of the innate immune system to autoimmune diseases, but the exact cellular processes and molecular actors triggering “innate autoimmunity” are not completely known.

Myasthenia gravis (MG) is a prototypic B cell-mediated autoimmune disease, mainly caused by autoantibodies to the acetylcholine receptor (AChR) located at the neuromuscular junction. Its pathogenesis is closely linked to morphological and functional alterations (follicular hyperplasia, thymoma) of the thymus, which is widely considered the main site of autoimmunity onset in MG patients. Over the past decade, an increasing body of evidence has been accumulated showing that pathogen infections (e.g. Epstein–Barr virus), dysregulated Toll-like receptor-mediated signaling pathways, interferon overexpression, and chronic inflammation are critically involved in the intrathymic MG development. Altered immuno-miR expression and defective immunoregulatory mechanisms, related to innate immune activation, may also contribute to anti-AChR autoimmunity initiation and perpetuation from the thymus to the periphery.

This Special Issue aims to summarize new insights on the pathogenic cross-talk between innate immunity and autoimmunity in MG. Further advances in this research field could have relevant therapeutic implications for MG and other autoimmune conditions, since targeting innate immune components could represent a promising approach to counteract inflammatory autoimmune processes.

Dr. Paola Cavalcante
Dr. Renato Mantegazza
Guest Editors

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Keywords

  • myasthenia gravis
  • thymus
  • innate immunity
  • inflammation
  • autoimmunity
  • Toll-like receptors
  • interferons
  • chemokines
  • germinal centers
  • Th17 cells
  • immuno-miRs

Published Papers (2 papers)

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14 pages, 1103 KiB  
Article
Towards Personalized Medicine in Myasthenia Gravis: Role of Circulating microRNAs miR-30e-5p, miR-150-5p and miR-21-5p
by Francesca Beretta, Yu-Fang Huang and Anna Rostedt Punga
Cells 2022, 11(4), 740; https://doi.org/10.3390/cells11040740 - 20 Feb 2022
Cited by 6 | Viewed by 2936
Abstract
Myasthenia gravis (MG) is an autoimmune neuromuscular disease characterized by fatigable skeletal muscle weakness with a fluctuating unpredictable course. One main concern in MG is the lack of objective biomarkers to guide individualized treatment decisions. Specific circulating serum microRNAs (miRNAs) miR-30e-5p, miR-150-5p and [...] Read more.
Myasthenia gravis (MG) is an autoimmune neuromuscular disease characterized by fatigable skeletal muscle weakness with a fluctuating unpredictable course. One main concern in MG is the lack of objective biomarkers to guide individualized treatment decisions. Specific circulating serum microRNAs (miRNAs) miR-30e-5p, miR-150-5p and miR-21-5p levels have been shown to correlate with clinical course in specific MG patient subgroups. The aim of our study was to better characterize these miRNAs, regardless of the MG subgroup, at an early stage from diagnosis and determine their sensitivity and specificity for MG diagnosis, as well as their predictive power for disease relapse. Serum levels of these miRNAs in 27 newly diagnosed MG patients were compared with 245 healthy individuals and 20 patients with non-MG neuroimmune diseases. Levels of miR-30e-5p and miR-150-5p significantly differed between MG patients and healthy controls; however, no difference was seen compared with patients affected by other neuroimmune diseases. High levels of miR-30e-5p predicted MG relapse (p = 0.049) with a hazard ratio of 2.81. In summary, miR-150-5p is highly sensitive but has low specificity for MG, while miR-30e-5p has the greatest potential as a predictive biomarker for the disease course in MG, regardless of subgroup. Full article
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18 pages, 1151 KiB  
Review
Myasthenia Gravis: An Acquired Interferonopathy?
by Cloé A. Payet, Axel You, Odessa-Maud Fayet, Nadine Dragin, Sonia Berrih-Aknin and Rozen Le Panse
Cells 2022, 11(7), 1218; https://doi.org/10.3390/cells11071218 - 4 Apr 2022
Cited by 11 | Viewed by 3901
Abstract
Myasthenia gravis (MG) is a rare autoimmune disease mediated by antibodies against components of the neuromuscular junction, particularly the acetylcholine receptor (AChR). The thymus plays a primary role in AChR-MG patients. In early-onset AChR-MG and thymoma-associated MG, an interferon type I (IFN-I) signature [...] Read more.
Myasthenia gravis (MG) is a rare autoimmune disease mediated by antibodies against components of the neuromuscular junction, particularly the acetylcholine receptor (AChR). The thymus plays a primary role in AChR-MG patients. In early-onset AChR-MG and thymoma-associated MG, an interferon type I (IFN-I) signature is clearly detected in the thymus. The origin of this chronic IFN-I expression in the thymus is not yet defined. IFN-I subtypes are normally produced in response to viral infection. However, genetic diseases called interferonopathies are associated with an aberrant chronic production of IFN-I defined as sterile inflammation. Some systemic autoimmune diseases also share common features with interferonopathies. This review aims to analyze the pathogenic role of IFN-I in these diseases as compared to AChR-MG in order to determine if AChR-MG could be an acquired interferonopathy. Full article
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