Science and Technology Production
Congress
Authorship
Date
2015
Publishing House and Editing Place
Lippincott Williams & Wilkins
Summary
Information provided by the agent in
SIGEVA
Objective: To study the serological status amongst generalized Myasthenia Gravis (MG) patients and their relationship with clinical features and the outcome of the disease in a hospital-based study from Buenos Aires, Argentina. Background: Acetylcholine receptor antibody (AChR-Ab) is the most common antibody found in MG. In AChR-Ab negative patient, a percentage which varies in different series, presents anti-muscle specific kinase antibodies (MuSK-Ab), anti-LPR4 antibodies, or are considered d...
Objective: To study the serological status amongst generalized Myasthenia Gravis (MG) patients and their relationship with clinical features and the outcome of the disease in a hospital-based study from Buenos Aires, Argentina. Background: Acetylcholine receptor antibody (AChR-Ab) is the most common antibody found in MG. In AChR-Ab negative patient, a percentage which varies in different series, presents anti-muscle specific kinase antibodies (MuSK-Ab), anti-LPR4 antibodies, or are considered double seronegative patients. Design/Methods: We retrospectively reviewed the medical records of patients affected by generalized MG attending at Ramos Mejia Hospital in Buenos Aires City, and related their serogical status with clinical characteristics of MG. Results: Amongst 98 generalized MG patients, 82 were AChR-Ab positive(83.7%). 4/12(33.3%) AChR-Ab negative patients were MuSK-Ab positive and 8/12 double seronegative-MG. No differences were found related to gender, age at onset and autoimmmune comorbidity between the three groups. Clinical features showed an initial ocular involvement more frequently in AChR-Ab negative groups: 75%(3/4) in Musk-Ab positive, 87.5%(7/8) in double seronegative-MG and 48.1%(39/81) in AChR-Ab positive MG(p<0,05). However, bulbar impairment at maximal worsening was found in all MuSK-Ab positive patients vs 42.7% AChR-Ab positive and 12.8% double seronegative-MG. Thymic abnormalities were seen in 22/85 patients(68.2% hyperplasia and 31.8% thymoma), all of them AChR-Ab positive. Severity of MG was no different between the groups according to MGFA clinical grading, but MuSK-Ab patients more frequently required immunosuppressive treatment (4/4). At the end of follow-up, the AChR-Ab positive group had better outcome than the other two groups, achieving minimal manifestation and remission stages in 59.5%. AChR level didn`t correlate with clinical severity. Conclusions: In our population, the AChR-Ab seroprevalence was 83.7%. Musk-MG represented 33.3% of AChR-Ab negative patients. 75% of Musk-MG showed exclusive ocular involvement at the beginning, although all of them subsequently generalized affecting bulbar muscles. AChR-Ab positive patients evidenced a better outcome than AChR-Ab negative patients.
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Key Words
Myasthenia GravisMuSK-AbAChR-Ab