Clinical characteristics of anti-AChR-MuSK-LRP4 antibody-negative myasthenia gravis in China

Muscle Nerve. 2023 Jun;67(6):481-488. doi: 10.1002/mus.27822. Epub 2023 Apr 3.

Abstract

Introduction/aims: Descriptions of the clinical characteristics of anti-AChR-MuSK-LRP4 antibody-negative myasthenia gravis (triple-negative myasthenia gravis, TNMG) are lacking in the current literature. Therefore, we investigated the clinical characteristics of TNMG in Chinese patients.

Methods: We retrospectively analyzed 925 patients with MG registered in the Department of Neuroimmunology, Henan Institute of Medical and Pharmaceutical Sciences from January 2015 to March 2021.

Results: One hundred six patients diagnosed with TNMG were included in the study. The average age of onset was 32.4 y, with a male-to-female ratio of 1:1. The age of onset showed a bimodal distribution: 0-9 y and 40-49 y. Adult patients were more likely to have weakness of limb and bulbar muscles (p < .05). Thymic hyperplasia was found in 20.2% of the patients. Younger patients were more likely to relapse. The rate of adult early-onset myasthenia gravis reaching complete stable remission and pharmacological remission was 47.6%, and the prognosis was better than that in juvenile-onset myasthenia gravis (p = .019). Older age of onset was the only risk factor for the development of generalized TNMG from ocular TNMG (R = 1.046, p = .002, 95% confidence interval 1.017-1.077).

Discussion: This study showed that the clinical characteristics of patients with TNMG varied among the different age groups. Significant findings included a bimodal distribution of onset age, coexisting thymic hyperplasia, and a generally favorable prognosis.

Keywords: anti-acetylcholine receptor antibody; late-onset; myasthenia gravis; prognosis; thymic hyperplasia; triple seronegative (TSN).

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Autoantibodies
  • China / epidemiology
  • Female
  • Humans
  • LDL-Receptor Related Proteins
  • Male
  • Myasthenia Gravis* / diagnosis
  • Myasthenia Gravis* / epidemiology
  • Neoplasm Recurrence, Local
  • Receptor Protein-Tyrosine Kinases
  • Receptors, Cholinergic
  • Retrospective Studies
  • Thymus Hyperplasia*

Substances

  • Receptors, Cholinergic
  • Receptor Protein-Tyrosine Kinases
  • Autoantibodies
  • LRP4 protein, human
  • LDL-Receptor Related Proteins