Office-based respiratory assessment in patients with generalized myasthenia gravis

• Published in: Neuromuscular disorders : NMD 2024-07, Vol.40, p.1-6
• Main Authors: Alcantara, Monica, Barnett-Tapia, Carolina, Bril, Vera, Mannan, Shabber, Shabanpour, Jafar, Riaz, Sarah, Ng, Eduardo, Ryan, Clodagh, Katzberg, Hans
• Format: Article
• Language: English
• Subjects: Adult; Aged; Female; Forced vital capacity; Humans; Male; Maximal inspiratory pressure; Middle Aged; Myasthenia gravis; Myasthenia Gravis - diagnosis; Myasthenia Gravis - physiopathology; Quality of Life; Respiratory dysfunction; Respiratory Function Tests; Surveys and Questionnaires; Vital Capacity
• ISSN: 0960-8966; 1873-2364; 1873-2364
• DOI: 10.1016/j.nmd.2024.05.005

•In a large cohort of myasthenia gravis patients with mild respiratory symptoms and disability, portable respiratory function tests were useful to screen those at risk of deterioration.•MIP and SNIPs were good indicators of respiratory muscle weakness and MIP best correlated with measures of disability and quality of life.•Females had a higher likelihood of presenting with an abnormal FVC and MIP than males. Patients with myasthenia gravis (MG) can present with respiratory dysfunction, ranging from exercise intolerance to overt respiratory failure, increased fatigue, or sleep-disordered breathing. To investigate the value of multiple respiratory tests in MG, we performed clinical and respiratory assessments in patients with mild to moderate generalized disease. One-hundred and thirty-six patients completed the myasthenia gravis quality-of-life score(MG-QOL-15), myasthenia gravis impairment index(MGII), Epworth sleepiness scale(ESS), University of California-San Diego Shortness of Breath Questionnaire(UCSD-SOB), Modified Medical Research Council Dyspnea Scales(MRC-DS), supine and upright forced vital capacity(FVC), maximal inspiratory pressures(MIPs) and sniff nasal inspiratory pressures(SNIP). Seventy-three (54 %) had respiratory and/or bulbar symptoms and 45 (33 %) had baseline abnormal FVC, with no significant postural changes (p = 0.89); 55 (40.4 %) had abnormal MIPs and 50 (37 %) had abnormal SNIPs. Overall, there were low scores on respiratory and disability scales. Females had increased odds of presenting with abnormal FVC (OR 2.89, p = 0.01) and MIPs (OR 2.48, p = 0.022). There were significant correlations between MIPs, FVC and SNIPs; between MGII/MG-QOL15 and UCSD-SOB/MRC-DS and between ESS and respiratory scales in the whole group. Our data suggests that office-based respiratory measurements are a useful screening method for stable MG patients, even when presenting with minimal respiratory symptoms and no significant disability.