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Epidemiology of seropositive myasthenia gravis in Sardinia: A population‐based study in the district of Sassari.

Authors :
Sechi, Elia
Deiana, Giovanni A.
Puci, Mariangela
Zara, Pietro
Ortu, Enzo
Porcu, Caterina
Carboni, Nicola
Chessa, Paola
Ruiu, Elisa
Nieddu, Arianna
Tacconi, Paolo
Russo, Antonello
Manca, Davide
Sechi, M. Margherita
Guida, Melania
Ricciardi, Roberta
Ercoli, Tommaso
Mascia, Marcello M.
Muroni, Antonella
Profice, Paolo
Source :
Muscle & Nerve. May2024, Vol. 69 Issue 5, p637-642. 6p.
Publication Year :
2024

Abstract

Introduction/Aims: The global incidence and prevalence of myasthenia gravis (MG) range between 6–31/million and 10–37/100,000, respectively. Sardinia is a high‐risk region for different immune‐mediated disorders, but the epidemiology of MG remains unclear. We determined the epidemiology of MG with acetylcholine receptor (AChR)‐immunoglobulin G (IgG) and muscle‐specific tyrosine kinase (MuSK)‐IgG in the district of Sassari (North‐Western Sardinia; population, 325,288). Methods: From the laboratory of the University Hospital of Sassari (reference for AChR/MuSK‐IgG testing in Sardinia since 1998) and the main neurology units in Sardinia, we retrospectively identified MG patients with (1) AChR‐IgG and/or MuSK‐IgG positivity by radioimmunoprecipitation assay; and (2) residency in the district of Sassari. Incidence (January 2010–December 2019) and prevalence (December 31, 2019) were calculated. Results: A total of 202 patients were included (incident, 107; prevalent, 180). Antibody specificities were AChR (n = 187 [93%]) and MuSK (n = 15 [7%]). The crude MG incidence (95% confidence interval) was 32.6 (26.8–39.2)/million, while prevalence was 55.3 (47.7–63.9)/100,000. After age‐standardization to the world population, incidence decreased to 18.4 (14.3–22.5)/million, while prevalence decreased to 31.6 (26.1–37.0)/100,000. Among incident cases, age strata (years) at MG onset were: <18 (2%), 18–49 (14%), 50–64 (21%), and ≥65 (63%). Discussion: Sardinia is a high‐risk region for MG, with a prevalence that exceeds the European threshold for rare disease. Identification of the environmental and genetic determinants of this risk may improve our understanding of disease pathophysiology. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
0148639X
Volume :
69
Issue :
5
Database :
Academic Search Index
Journal :
Muscle & Nerve
Publication Type :
Academic Journal
Accession number :
176474038
Full Text :
https://doi.org/10.1002/mus.28077