Triple-seronegative myasthenia gravis: clinical and epidemiological characteristics

Author:

Rodrigues Paula Raquel do Vale Pascoal12ORCID,Kay Cláudia Suemi Kamoi1ORCID,Ducci Renata Dal-Pra1ORCID,Utiumi Marco Antonio Takashi2ORCID,Fustes Otto Jesus Hernandez1ORCID,Werneck Lineu Cesar12ORCID,Lorenzoni Paulo José1ORCID,Scola Rosana Herminia12ORCID

Affiliation:

1. Universidade Federal do Paraná, Hospital de Clínicas, Departamento de Clínica Médica, Serviço de Neurologia, Serviço de Doenças Neuromusculares, Curitiba PR, Brazil.

2. Universidade Federal do Paraná, Programa de Pós-Graduação em Medicina Interna, Curitiba PR, Brazil.

Abstract

Abstract Background Myasthenia gravis (MG) is an autoimmune disease usually caused by antibodies against the acetylcholine receptor (AChR-Abs), muscle-specific tyrosine kinase (MuSK-Abs), or low-density lipoprotein receptor-related protein 4 (LRP4-Abs). However, there are MG patients who do not have these antibodies and are thus said to have triple-seronegative (triple-SN) MG. Objective This study aims to describe the frequency and clinical and epidemiological characteristics of patients with triple-SN MG. Methods This was a retrospective cross-sectional study carried out through the analysis of medical records. Descriptive and analytical statistical analysis was performed comparing subgroups of myasthenic patients, classified according to serological profile. Results The sample population consisted of 93 MG patients: 85 were positive for antibodies, 80 (86%) with AChR-Abs, 5 (5.4%) with MuSK-Abs, and no MG patients with LRP4-Abs. Eight patients (8.6%) had triple-SN MG; they had a median age at disease onset of 30 years (21-45). Their most common initial symptoms were ptosis, diplopia, and generalized weakness. Most patients presented with mild symptoms at their last visit, reflecting a median MG composite scale score of 4 (0-6), and 75% of patients had an adequate response to treatment. Conclusion Our study showed a low frequency of triple-SN MG in Brazilian MG patients. Triple-SN MG was predominant in females, who presented with ptosis, diplopia, and generalized weakness, and most patients had an adequate response to immunosuppressive treatment. There was no significant difference between triple-SN MG and the other subgroups.

Publisher

Georg Thieme Verlag KG

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