Epidemiology of seropositive myasthenia gravis in Greece

Poulas, Konstantinos

doi:10.1136/jnnp.71.3.352

Cited by 95 publications

(83 citation statements)

References 18 publications

(11 reference statements)

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Section: History Of Myasthenia Gravismentioning

confidence: 99%

“…The yearly incidence has also risen in all studies [5] due to significant increase among older males as well as females [6].…”

Section: History Of Myasthenia Gravismentioning

confidence: 99%

“…The incidence and prevalence has increased particularly in older individuals [3,4]. The yearly incidence has also risen in all studies [5] due to significant increase among older males as well as females [6].The role of the antibodies that cause myasthenia gravis was clearly established in the 1970s but characterization of antigenic targets evolved till now. Confirmation that antibodies to AChR alone could cause myasthenia gravis, came from immunisation against purified AChRs [7], and the fact that monoclonal antibodies to AChR can produce similar effects in laboratory animals [8].…”

mentioning

confidence: 99%

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Myasthenia Gravis: Clinical and Immunological Aspects

Tandon¹

2017

Immunopathogenesis and Immune-Based Therapy for Selected Autoimmune Disorders

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Autoimmune diseases such as myasthenia gravis (MG) result from an altered balance between the processes of activation and regulation of immune response. MG is the most common autoimmune disorder characterized by failure of transmission at the neuromuscular junction (NMJ). Autoantibodies in MG target the acetylcholine receptors (AChRs) as well as non-AChR components like muscle-specific tyrosine kinase (MuSK).Autoantibodies against AChRs are produced by B cells in the germinal centres (GCs), formed in the medulla of MG thymus and circulated to the post-synaptic side of the neuromuscular junction (NMJ) leading to complement-mediated destruction of the postsynaptic folds of NMJ and internalization of AChRs. The incidence and prevalence of MG have increased particularly in elderly, but clinical presentations vary substantially and recognition depends on classic disease phenotype. This chapter focuses on clinical and immunological aspects of MG and its subgroups based on its characterization of the antigenic targets.Keywords: autoimmune disorders, autoantibodies, myasthenia gravis, acetylcholine receptors, B-cell receptors, cell-based assays, seronegative myasthenia gravis History of myasthenia gravisMyasthenia gravis (MG) is an autoimmune syndrome caused by the failure of neuromuscular transmission, which results from the binding of autoantibodies to proteins involved in signalling at the neuromuscular junction. Neurobiology of myasthenia gravis states that it is an antibody-mediated autoimmune disorder where antibodies to the acetylcholine receptors (AChR) cause complement-mediated destruction of the post-synaptic folds of the neuromuscular junction (NMJ) and internalisation of AChRs. This results in reduced muscle-nerve synaptic transmission and fatigable muscle weakness, one of the characteristic clinical features, in MG patients. It mainly affects the voluntary muscles including muscles of the neck, eyelids, © 2017 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The incidence and prevalence has increased particularly in older individuals [3,4]. The yearly incidence has also risen in all studies [5] due to significant increase among older males as well as females [6].The role of the antibodies that cause myasthenia gravis was clearly established in the 1970s but characterization of antigenic targets evolved till now. Confirmation that antibodies to AChR alone could cause myasthenia gravis, came from immunisation against purified AChRs [7], and the fact that monoclonal antibodies to AChR can produce similar effects in laboratory animals [8]. However they also tested that plasma exchange, removes circulating antibodies, which leads to a substantial but transient improvement in muscle function lasting up to 2 months [9]. Diagnostic and clinical classification of MGAutoan...

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Section: History Of Myasthenia Gravismentioning

confidence: 99%

“…The yearly incidence has also risen in all studies [5] due to significant increase among older males as well as females [6].…”

Section: History Of Myasthenia Gravismentioning

confidence: 99%

mentioning

confidence: 99%

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Myasthenia Gravis: Clinical and Immunological Aspects

Tandon¹

2017

Immunopathogenesis and Immune-Based Therapy for Selected Autoimmune Disorders

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“…Prosečna godišnja prevalencija za navedeno razdoblje iznosi 153,6/1.000.000 stanovnika. U svetu se vrednosti standardizovane prevalencije kreću od 27,7/1.000.000 do 354,7/1.000.000 stanovnika (13)(14)(15)(16)(17). Ovakav raspon vrednosti prevalencije mogao bi da se objasni razlikama u dijagnostičkim i terapijskim mogućnostima među zemljama.…”

Section: Diskusijaunclassified

“…MedPodml 2017, 68(1): [14][15][16][17][18][19][20] Uvod Stečena autoimuna miastenija gravis (MG) predstavlja autoimuno oboljenje u kome se javljaju antitela na različite postsinaptičke strukture neuromišićne spojnice, uglavnom na nikotinski acetilholinski receptor (nAChR), ali i na mišićno specifičnu tirozin kinazu (MuSK), protein 4 povezan sa lipoproteinom (LRP4) ili arginin (1). Ova antitela uzrokuju oštećenje neuromišićne transmisije i klinički dovode do mišićne slabosti i patološke zamorljivosti.…”

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Frequency of familial occurrence and associated autoimmune diseases in a cohort of patients with acquired autoimmune myasthenia gravis from Belgrade

Josipovic¹,

Jovanović²,

Jadžić³

et al. 2017

Med podmladak

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Introduction: Myasthenia gravis (MG) can be associated with other autoimmune diseases (AID), most frequently with Hashimoto's thyroiditis, systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). Familial occurrence (FOMG) is rare and it exists in 3.5-4.2% patients with MG. Aim: To determine the occurrence of FOMG, the occurrence of AID associated with MG and to identify potential risk factors for developing MG in a cohort of patients from Belgrade. Material and Methods: This study gathered data from 453 patient histories from the period from1992-2014, from the Clinic of Neurology, Clinical Center of Serbia. Three analyses were performed. The first one measured the frequency of associated AID in patients with MG. In the second one, patients who had MG associated with AID were compared to patients without associated AID. The third one included patients with FOMG and compared them with patients without FOMG. The demographic and clinical characteristics of these patients were analysed and comparisons were made between observed and control groups. Results: The associated AID were present in 54 (11.9%) patients, the most frequent was Hashimoto thyreoiditis (4%), then SLE (1.3%), and RA, polymyositis and pernicious anaemia (0.9%). Patients with co-occurrence of MG and other AID were, in comparison with control group, more often female, and the difference was on the border of statistical significance (p=0.056). They had late onset MG (LOMG) more frequently, a mild form of MG and positive anti-AChR antibodies, but these differences weren't statistically significant. FOMG was seen in 2.2% of patients. They had LOMG more frequently, were predominantly men, seropositive, and with mild form of the disease, but with no statistical significance compared to the control group. Conclusion: In the Belgrade cohort, MG was often associated with other AID, while FOMG was relatively rare. There was no significant difference, statistically, in the observed clinical and demographic characteristics of analysed groups, compared to control groups.

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Mortality of myasthenia gravis: a national population‐based study in China

Zhang

Wang

Long

et al. 2023

Ann Clin Transl Neurol

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Objective As a potentially life‐threatening condition, myasthenia gravis (MG) has limited epidemiological studies on mortality. We aim to provide demographic distribution, geographical variation, and temporal trend of MG‐related mortality in China. Methods The national population‐based analysis was conducted based on records derived from the National Mortality Surveillance System of China. All deaths related to MG were identified from 2013 to 2020, and MG‐related mortality was evaluated by sex, age, location, and year. Results A total of 4224 deaths were related to MG during 2013–2020, and the median age at death of MG was 59.45 years, significantly lower than that in the general population (75.47 years, P < 0.05). In 2020, the age‐standardized mortality rate of MG was 1.86 per million people and markedly higher in males than in females (2.37 vs. 1.31 per million). The mortality rate per million was lower than 1 in young children, peaking at 2.83 only in males (vs. 0.36 in females) aged 10–19 years, and substantially increased with age, reaching the highest rate of 13.31 for males and 10.58 for females aged 80 years and older. Geographical disparity across China was observed with the highest age‐standardized mortality rate in Southwest (2.53 per million). From 2013 to 2020, MG‐related mortality rate showed an increasing trend with the average annual percentage change of 3.5% (95% CI, 1.4–5.6). The notable increases occurred in age 10–19 years and over 70 years. Interpretation In China, MG‐related mortality was notably high among adolescent males and the elderly. The increasing death burden due to MG highlight challenges to disease management.

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Epidemiology of seropositive myasthenia gravis in Greece

Cited by 95 publications

References 18 publications

Myasthenia Gravis: Clinical and Immunological Aspects

Myasthenia Gravis: Clinical and Immunological Aspects

Frequency of familial occurrence and associated autoimmune diseases in a cohort of patients with acquired autoimmune myasthenia gravis from Belgrade

Mortality of myasthenia gravis: a national population‐based study in China

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