Refractory myasthenia gravis: Characteristics of a portuguese cohort

Muscle Nerve. 2019 Aug;60(2):188-191. doi: 10.1002/mus.26507. Epub 2019 May 15.

Abstract

Introduction: Some myasthenia gravis (MG) patients are refractory to conventional treatments.

Methods: To describe the clinical features of refractory MG (RMG) and explore the association with human leukocyte antigen HLA-DRB1 alleles, a cohort study of 114 consecutive MG patients was performed. Patients were classified as RMG based on predefined criteria.

Results: Twenty-two patients were found to have RMG (19.3%). There were no differences between non-RMG and RMG patients with respect to sex, age of onset, abnormal 3-Hz repetitive nerve stimulation, anti-acetylcholine receptor antibody positivity, thymectomy, thymoma or thymic hyperplasia, and polyautoimmunity. HLA-DRB1*03 was more frequent in the non-RMG vs. control population (P = 3 × 10-6 ). The HLA-DRB1*13 allele was less frequent in non-RMG patients compared with controls (P = 0.002), and less frequent in the non-RMG group compared with the RMG group (P = 0.003).

Discussion: HLA-DRB1*03 was more common in non-RMG, and the HLA-DRB1*13 allele appeared to have a protective role, as reported previously in other autoimmune disorders. Muscle Nerve 60: 188-191, 2019.

Keywords: human leukocyte antigens; myasthenia gravis; polyautoimmunity; refractory; thymoma MG.

MeSH terms

  • Adult
  • Age of Onset
  • Autoantibodies / immunology
  • Case-Control Studies
  • Cohort Studies
  • Female
  • Genetic Predisposition to Disease
  • HLA-DRB1 Chains / genetics*
  • Humans
  • Male
  • Middle Aged
  • Myasthenia Gravis / epidemiology
  • Myasthenia Gravis / genetics*
  • Myasthenia Gravis / immunology
  • Portugal / epidemiology
  • Protective Factors
  • Receptors, Cholinergic / immunology
  • Thymectomy / statistics & numerical data
  • Thymoma / epidemiology
  • Thymus Hyperplasia / epidemiology
  • Thymus Neoplasms / epidemiology
  • Young Adult

Substances

  • Autoantibodies
  • HLA-DRB1 Chains
  • Receptors, Cholinergic