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.

Epidemiology of seropositive myasthenia gravis in Sardinia: A population-based study in the district of Sassari / 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; Saddi, Valeria; Melis, Maurizio; Cocco, Eleonora; Spagni, Gregorio; Iorio, Raffaele; Damato, Valentina; Maestri, Michelangelo; Sotgiu, Stefano; Sotgiu, Giovanni; Solla, Paolo. - In: MUSCLE & NERVE. - ISSN 1097-4598. - 69:5(2024). [10.1002/mus.28077]

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

Sechi, Elia;Deiana, Giovanni A;Puci, Mariangela;Zara, Pietro;Porcu, Caterina;Nieddu, Arianna;Manca, Davide;Ercoli, Tommaso;Melis, Maurizio;Sotgiu, Stefano;Solla, Paolo
2024-01-01

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.
2024
Epidemiology of seropositive myasthenia gravis in Sardinia: A population-based study in the district of Sassari / 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; Saddi, Valeria; Melis, Maurizio; Cocco, Eleonora; Spagni, Gregorio; Iorio, Raffaele; Damato, Valentina; Maestri, Michelangelo; Sotgiu, Stefano; Sotgiu, Giovanni; Solla, Paolo. - In: MUSCLE & NERVE. - ISSN 1097-4598. - 69:5(2024). [10.1002/mus.28077]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11388/337753
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