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A systematic review of population based epidemiological studies in Myasthenia Gravis

TLDR
There is evidence of increasing frequency of MG with year of study and improved study quality, which probably reflects improved case ascertainment and other factors must also influence disease onset resulting in the observed variation in IR across geographically and genetically similar populations.
Abstract
The aim was to collate all myasthenia gravis (MG) epidemiological studies including AChR MG and MuSK MG specific studies. To synthesize data on incidence rate (IR), prevalence rate (PR) and mortality rate (MR) of the condition and investigate the influence of environmental and technical factors on any trends or variation observed. Studies were identified using multiple sources and meta-analysis performed to calculate pooled estimates for IR, PR and MR. 55 studies performed between 1950 and 2007 were included, representing 1.7 billion population-years. For All MG estimated pooled IR (eIR): 5.3 per million person-years (C.I.:4.4, 6.1), range: 1.7 to 21.3; estimated pooled PR: 77.7 per million persons (C.I.:64.0, 94.3), range 15 to 179; MR range 0.1 to 0.9 per millions person-years. AChR MG eIR: 7.3 (C.I.:5.5, 7.8), range: 4.3 to 18.0; MuSK MG IR range: 0.1 to 0.32. However marked variation persisted between populations studied with similar methodology and in similar areas. We report marked variation in observed frequencies of MG. We show evidence of increasing frequency of MG with year of study and improved study quality. This probably reflects improved case ascertainment. But other factors must also influence disease onset resulting in the observed variation in IR across geographically and genetically similar populations.

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Journal ArticleDOI

Myasthenia gravis: subgroup classification and therapeutic strategies.

TL;DR: Patients with myasthenia gravis should be classified into subgroups to help with therapeutic decisions and prognosis, and additional immunomodulatory drugs are emerging, but therapeutic decisions are hampered by the scarcity of controlled studies.
Journal ArticleDOI

Myasthenia gravis: a comprehensive review of immune dysregulation and etiological mechanisms.

TL;DR: The influence of the pro-inflammatory environment is discussed, particularly the role of TNF-α and Th17-related cytokines, which could explain the escape of thymic T cells from regulation and the chronic inflammation in the MG thymus.
Journal ArticleDOI

The different roles of the thymus in the pathogenesis of the various myasthenia gravis subtypes.

TL;DR: The role of the thymus is still obscure in MG defined by antibodies against the agrin receptor LRP4 and in MG without all of the above autoantibdies (triple sero-negative MG) since these MG subtypes have been described only recently and thymectomy has been their standard treatment.
Journal ArticleDOI

Myasthenia gravis — autoantibody characteristics and their implications for therapy

TL;DR: The different MG subtypes, the sensitivity and specificity of the various antibodies involved in MG for distinguishing between these sub types, and the value of antibody assays in guiding optimal therapy are discussed.
References
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Journal ArticleDOI

Measuring inconsistency in meta-analyses

TL;DR: A new quantity is developed, I 2, which the authors believe gives a better measure of the consistency between trials in a meta-analysis, which is susceptible to the number of trials included in the meta- analysis.
Book

Myasthenia Gravis

Age standardization of rates: a new who standard

TL;DR: The World Health Organization (WHO) adopted a standard based on the average age-structure of those populations to be compared (the world) over the likely period of time that a new standard will be used (some 25-30 years), using the latest UN assessment for 1998 (UN Population Division, 1998) from these estimates, an average world population agestructure was constructed for the period 2000-2025 as discussed by the authors.
Journal ArticleDOI

Antibody to acetylcholine receptor in myasthenia gravis Prevalence, clinical correlates, and diagnostic value

TL;DR: Assay of antireceptor antibody should prove a useful test in the diagnosis of myasthenia gravis, and presence or titer of antibody did not appear to correlate with age, sex, steroid therapy, or duration of symptoms.
Journal ArticleDOI

Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies

TL;DR: The results indicate the involvement of MuSK antibodies in the pathogenesis of AChR-Ab–seronegative MG, thus defining two immunologically distinct forms of the disease.
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