Respiratory muscle training in patients with moderate to severe myasthenia gravis

Abstract

Background: Myasthenia gravis (MG) is a specific autoimmune disease characterized by weakness and fatigue. MG may affect also the respiratory muscles causing symptoms that may vary from dyspnea on severe exertion to dyspnea at rest. This study was undertaken in order to determine the effects of respiratory muscle training on respiratory muscle performance, spirometry data and the grade of dyspnea in patients with moderate to severe generalized MG. Methods: Eighteen patients with MG were studied and divided into 2 groups: Group A included 10 patients (3 males and 7 females aged 29-68) with moderate MG, and Group B that included 8 patients (5 males and 3 females aged 21-74) with severe MG. Patients in Group A received both inspiratory and expiratory muscle training for 1/2 h/day, 6 times a week, for 3 months, while patients in Group B followed the same protocol but had inspiratory muscle training only. Results: Mean PI(max) increased significantly from 56.6 ± 3.9 to 87.0 ± 5.8 cm H2O (p < 0.001) in Group A, and from 28.9 ± 5.9 to 45.5 ± 6.7 cm H2O (p < 0.005) in Group B. The mean PE(max) also increased significantly in patients in Group A, but remained unchanged in the patients in Group B. The respiratory muscle endurance also increased significantly, from 47.9 ± 4.0 to 72.0 ± 4.2%, p < 0.001, in patients of Group A, and from 26.0 ± 2.9 to 43.4 ± 3.8, p < 0.001, in patients in Group B. The improved respiratory muscle performance was associated with a significant increase in the FEV1 values, and in the FVC values, in patients of both groups. Mean dyspnea index score also increased significantly from 2.6 ± 0.8 to 3.6 ± 0.4 (p < 0.005) in Group A, and from 0.7 ± 0.2 to 2.0 ± 0.2 (p < 0.001) in Group B. Conclusions: Specific inspiratory threshold loading training alone, or combined with specific expiratory training, markedly improved respiratory muscle strength and endurance in patients with MG. This improvement in respiratory muscle performance was associated with improved lung function and decreased dyspnea. Respiratory muscle training may prove useful as a complementary therapy with the aim of reducing dyspnea symptoms, delay the breathing crisis and the need for mechanical ventilation in patients with MG.