Abstract
BackgroundThe 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.MethodsStudies were identified using multiple sources and meta-analysis performed to calculate pooled estimates for IR, PR and MR.Results55 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.ConclusionsWe 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.
Highlights
The aim was to collate all myasthenia gravis (MG) epidemiological studies including AChR MG and MuSK MG specific studies
Myasthenia gravis (MG) is an archetypal autoimmune disorder in which muscle weakness occurs as a result of impairment of neuromuscular transmission
Systematic review of MG epidemiology has been carried out in the past by Phillips [5]; but 28 further studies have been performed since and this review predates the discovery of MuSK-Ab MG
Summary
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. Myasthenia gravis (MG) is an archetypal autoimmune disorder in which muscle weakness occurs as a result of impairment of neuromuscular transmission. A large number of MG epidemiological studies have been performed worldwide over the last 60 years with marked variability in observed incidence and prevalence of the disease. Systematic review of MG epidemiology has been carried out in the past by Phillips [5]; but 28 further studies have been performed since and this review predates the discovery of MuSK-Ab MG. Phillips commented upon apparent increasing IR and PR with time but without similar change in MR, and proposed that these trends were due to improved diagnosis and a changing natural history of disease related to better treatment
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