Myasthenie.com

Muscle Nerve. 2022 Feb 23. doi: 10.1002/mus.27521. Online ahead of print.

ABSTRACT

INTRODUCTION: A subgroup of myasthenia gravis (MG) patients fails to respond adequately to recommended treatments, a condition referred to as refractory MG. During the refractory phase patients experience persistent debilitating symptoms with potential life-threatening events or inability to reduce immunosuppressant dosages and minimize long-term toxicities.

METHODS: We conducted a retrospective single-centre study of 113 MG patients to investigate the autoantibody profile and clinical characteristics of refractory MG patients, compared to non-refractory patients, based on predefined criteria.

RESULTS: Fifteen patients (13.3%) were classified as refractory. Double seronegative MG (DSNMG), without detectable nicotinic acetylcholine receptor (AChR) or muscle-specific tyrosine kinase (MuSK) antibodies, was identified in 6 refractory patients, significantly higher than those with non-refractory MG (40% vs 16.3%, p=0.031). None of the refractory patients had MuSK antibodies. Patients in the refractory group more frequently had an earlier disease onset, thymic pathology and thymectomy (all p-values ≤0.03).

DISCUSSION: In this study, patients with refractory MG were more likely than those with non-refractory MG to be DSN; and refractory DSNMG patients had worse MGFA classes in their recent visit compared to anti-AChR positive refractory patients. Refractory DSNMG patients may represent a distinct group that requires more individualized and targeted treatment approaches. This article is protected by copyright. All rights reserved.

PMID:35195920 | DOI:10.1002/mus.27521


Source: https://pubmed.ncbi.nlm.nih.gov/3519592 ... 1&v=2.17.5