Quality of life in refractory generalized myasthenia gravis: A rapid review of the literature

Abstract

Generalized myasthenia gravis (GMG) is a neuromuscular transmission disorder that creates a fluctuating weakness of the voluntary muscles. This study is aimed at understanding the effect that refractory GMG has on the quality of life of patients who suffer from it, and the effect of eculizumab on it. A systematic literature search was conducted in MEDLINE (Ovid), EMBASE and the Cochrane Database of Systematic Reviews (Ovid). Eligibility criteria were verified via the title and summary and afterward through the full text. The risk of bias of the included randomized clinical trials was evaluated and the data were synthesized in a descriptive manner. Nine studies were identified that evaluated the quality of life of patients with GMG. Regarding the effect of eculizumab, two studies were identified. The quality of life in patients with GMG is lower compared to ocular myasthenia gravis (MG) and MG in remission, especially in the domains of physical function, physical role, bodily pain, vitality, and social function. Patients treated with eculizumab had a better perception of their quality of life compared to those who received placebo. GMG affects the quality of life more than other types of MG. This outcome is of great importance for the choice of therapeutic options in patients with refractory GMG. Eculizumab generates improvements in the perception of patients' quality of life compared to placebo, making it a relevant therapeutic option in the management of refractory GMG.

Keywords: Myasthenia gravis; generalized myasthenia gravis; quality of life; refractory generalized myasthenia gravis.

Figure 1.

Flowchart search screening and selection of evidence.

Figure 2.

Score of the physical and mental component of the SF-36 scale by type of myasthenia gravis, in Boldingh et al. (). Mean values. MG: Myasthenia gravis. Source: Adapted from Boldingh, et al. An up-date on health-related quality of life in myasthenia gravis - results from population-based cohorts. Health Qual Life Outcomes. 2015; 13:115.

Figure 3.

Score using the SF-36 scale by domain in the GMG population and reference population, in Paul et al. (). Mean ± standard deviation. GMG: Generalized myasthenia gravis, VR: Reference values, O-QOL: Overall quality of life related to health, PF: physical function, SF: social function, RDP: Role disruption-physical, RDE: Role disruption-emotional, MH: mental health, VT: vitality, BP: body pain, GH: general perception of health. Source: Adapted from Paul RH, et al. Quality of life and well-being of patients with myasthenia gravis. Muscle Nerve. 2001; 24:512-516.