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Evaluation of the Quality of Life of Patients with Myasthenia Gravis in Greece.

ABSTRACT: Myasthenia Gravis (MG) patients often report an affected quality of life (QoL). The aim of the current study was to evaluate the QoL of patients with MG in Greece using a specific tool. A cross-sectional online survey was performed. Adult patients were invited to participate. A questionnaire incorporating the MG-QOL15r scale was distributed, following its translation and cultural adaptation into Greek. Overall, 99 valid responses were submitted. The median age (interquartile range) of the participants was 48.50 (13.50) years and 76.80% were females. One third of the patients mentioned that they could not work/changed jobs after their diagnosis (28.30%) and that they face severe restriction of their everyday activities (26.30%). The mean MG-QOL15r score was 13.50 ± 7.70. Patients with important restriction of everyday activities (p < 0.01), patients with more pronounced need of emotional support (p < 0.01), patients with generalized MG (p < 0.01) and patients with myasthenic crises (p < 0.01) reported lower QoL. This study is the first to report on the affected QoL of the Greek population with MG using the MG-QoL15r scale. Further work should be done to incorporate the routine evaluation of QoL in the care of patients with MG.

SUBMITTER: Aggelina A

PROVIDER: S-EPMC10381620 | biostudies-literature | 2023 Jul

REPOSITORIES: biostudies-literature

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Evaluation of the Quality of Life of Patients with Myasthenia Gravis in Greece.

Aggelina Afrodite A Karampli Eleftheria E Mavrovounis Georgios G Boutsikos Ioannis I Pantazopoulos Ioannis I Kakavas Sotirios S Pavi Elpida E Athanasakis Kostas K

Journal of personalized medicine 20230712 7

Myasthenia Gravis (MG) patients often report an affected quality of life (QoL). The aim of the current study was to evaluate the QoL of patients with MG in Greece using a specific tool. A cross-sectional online survey was performed. Adult patients were invited to participate. A questionnaire incorporating the MG-QOL15r scale was distributed, following its translation and cultural adaptation into Greek. Overall, 99 valid responses were submitted. The median age (interquartile range) of the partic ...[more]

PMID: 37511743

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Project description:BackgroundMyasthenia gravis (MG), a chronic neuromuscular disorder, can adversely affect patients' health-related quality of life (HRQoL), especially in women. The study aimed to evaluate the difference in HRQoL of women and men MG patients and explore the factors that mediate the relationship between gender and HRQoL.MethodsA cross-sectional study was conducted among 1815 patients with MG in China. The revised 15-item MG quality of life scale (MG-QOL15r) was used to access patients' HRQoL in overall, physical, social and emotional domains. Socio-demographic information, diagnosis and treatment history, comorbidities, social support, active lifestyle and the MG activities of daily living scale (MG-ADL) were recorded and compared between women and men using the Student's t-test and Pearson's Chi-square test. Multivariable regression analyses were conducted to identify independent contributors to HRQoL, especially those affecting different gender.ResultsOn average, female patients with MG reported a lower MG-QOL15r score than the males (44.49 ± 29.10 vs 49.32 ± 29.18). The association between gender and patients' HRQoL interacted with the number of comorbidities across the overall, physical and social domains of patients. As the number of comorbidities increased, the scores of HRQoL decreased and it was faster among females than the males (p < 0.05). Moreover, unemployment, exacerbation of the disease, and active lifestyle contributed to the patients' HRQoL across all domains. Unemployment (β = - 4.99 [95%CI, - 7.80 to - 2.18], p < 0.001) and exacerbations (β = - 8.49 [95%CI, - 11.43 to - 5.54], p < 0.001) were correlated with poorer HRQoL; while an active lifestyle had a positive impact on HRQoL (β = 0.28 [95%CI, 0.16 to 0.40], p < 0.001).ConclusionsThe results indicate that the HRQoL of women MG patients was lower than that of men. The relationship between gender and HRQoL is modulated by the number of comorbidities. Thus, to improve the HRQoL of women MG patients, symptomatic treatments might not be enough, their comorbid conditions should be considered as well. Additionally, employment status, MG exacerbations, and an active lifestyle have been found as determining factors of the patients' HRQoL, which suggests future interventions should cope with these factors to improve their quality of life.

Project description:Aims To explore the quality of life (QOL) in patients with myasthenia gravis (MG) and factors associated with QOL. Methods This observational study included patients with MG diagnosed at the First Affiliated Hospital of Fujian Medical University between January 2020 and March 2022. The QOL of patients was evaluated with the 15-item Myasthenia Gravis Quality of Life (MG-QOL15). Current MG severity was evaluated with MGFA grade, MG-ADL score, MGC score, and MGFA Postintervention Status. The data about gender, age of onset, subgroup, antibodies, age, duration, education, employment state, marital status, skeletal muscle affected, thymic histology, and current treatment methods of the patient were collected. Results A total of 185 patients [72 males (38.9%), aged 45.2 years (14–77)] with MG were enrolled. Age at onset was 38.3 ± 17.9 years, and disease duration was 87.9 months (0–672). The median MG-QOL15 score was 12.5 (0–58). The item “have trouble using my eyes” was the highest scoring item in both ocular and generalized patients with MG. The MG-QOL15 score was significantly different among patients with OMG (9.2 ± 9.4, n = 63), GMG (9.0 ± 8.8, n = 22), and BMG (15.4 ± 14.2, n = 100) (P = 0.018). Patients with BMG had higher MG-QOL15 scores than OMG (P = 0.001) and GMG (P = 0.009), but there was no significant difference between OMG and GMG (P = 0.467). The MG-QOL15 score was significantly lower in patients who had undergone thymectomy (9.7 ± 9.8, n = 58) compared to those who had not (13.8 ± 13.4, n = 127, P = 0.022). MG-QOL15 score was significantly lower in patients who underwent thymectomy compared to those who did not (9.7 ± 9.8, n = 58 vs. 13.8 ± 13.4, n = 127, P = 0.022). MG-QOL15 score was different among MGFA grades (Remission: 5.2 ± 5.4, n = 41; I: 11.3 ± 10, n = 61; II: 11.6 ± 11.1, n = 40; III: 18.1 ± 12.1, n = 29; and IVa: 30.1 ± 20, n = 14, P < 0.001). There was no significant difference between patients in MGFA grade I and II (P = 0.896), and there was no significant difference between patients in MGFA grade III and IVa (P = 0.052). MG-ADL (P < 0.001) and MGC (P < 0.001) were positively correlated with MG-QOL15. Men had higher MG-QOL15 than women (P = 0.094), and LOMG had higher MG-QOL15 than EOMG (P = 0.072). Multivariate linear regression identified that higher MG-ADL (P < 0.001), higher MGC (P = 0.02), and poor employment status (P = 0.045) were independently associated with higher MG-QOL15. Conclusion Having trouble using the eyes accounted for the highest score in MG-QOL15, eye symptoms affect QOL more than limb weakness in MG. Daily life activity, disease severity, and employment status were associated with patients' QOL. Adequate treatment should be applied to improve QOL, while mild symptoms can be accepted. Men and patients over the age of 50 years of onset may need more attention.

Project description:Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction, whose clinical hallmark is muscle weakness and early fatigability. Azathioprine (AZA) is commonly used in Myasthenia Gravis therapy. AZA is a purine antagonist, which inhibits the cell cycle in the resting and DNA synthesis phases. It is usually used as an immunosuppressant to block T- and B-cell proliferation. AZA, bioconverted to 6-mercaptopurine by glutathione S-transferase (GST), can be metabolized either through the hypoxanthine phosphoribosyl transferase pathway to active 6-thioguanine nucleotides (6-TGN) or through the thiopurine S-methyltransferase (TPMT) pathway to inactive methyl-thiopurine metabolites. The incorporation of active 6-TGNs into DNA, causing breaks in DNA strands resulting in interference with RNA production and thereby protein synthesis, is responsible for the drug effect. The response to AZA is determined by which metabolic pathway is being favored. While balanced use of both HPRT and TPMT pathways results in responsiveness to AZA, hyperactivity of TPMT skews the balance towards the TPMT pathway and results in unresponsiveness to AZA with no pharmacological effect. In contrast, low TPMT activity skews the balance towards the HPRT pathway, resulting in increasing side-effects due to the accumulation of 6-TGNs. Current treatments for MG therapy are often inadequate because less than 40% of patients achieve complete remission with available drugs. This reflects the lack of drugs acting on target sites for which there is strong evidence of pathogenicity and the inability to identify responder patients. No criteria for responsiveness are available, exposing patients to unpredictable failures and unpredictable side effects. These individuals are at particular risk for adverse drug reaction (ADRs) or therapeutic failure. Genetic profiling with the Affymetrix drug metabolizing enzymes and transports genotyping array offers the ability to determine 1,931 variants and 225 genes involved in drug metabolism and disposition. Accordingly, the study of well-known drug-metabolizing genes can be involved in the specific metabolic pathway of a drug, which is more likely to define genotype-phenotype association and thereby genotype profiles relevant to drug response that can be applied as predictive biomarkers for pharmacological treatment.

Project description:IntroductionEculizumab, a complement active antibody, and efgartigimod, an Fc fragment that blocks neonatal Fc receptor, are both approved to treat generalized myasthenia gravis (gMG) patients. The objective of this study is to describe the clinical response to both treatments in a real-life setting.MethodsWe collected baseline and follow-up clinical data using the Myasthenia Gravis-Activities of Daily Living (MG-ADL), and Quantitative Myasthenia Gravis (QMG). We included 63 patients, 32 treated with eculizumab and 31 with efgartigimod. Of the efgartigimod-treated patients, 22 were anti-acetylcholine receptor antibody-positive (AChR-Ab +) and 9 were AChR-Ab- (3 MuSK-Ab + and 6 seronegative).ResultsBoth treatments showed similar efficacy relative to the MG-ADL scale reduction (p = 0.237). Efgartigimod had a similar effect on both AChR-Ab + and AChR-Ab- (p = 0.280). Eculizumab was superior to efgartigimod relative to the QMG score reduction for the entire dataset (p = 0.003) and was more likely to achieve a clinical response at the QMG compared to efgartigimod (OR 1.373; p = 0.016). Steroid-sparing effect was higher for eculizumab than efgartigimod ( - 16.7 vs - 5.2 mg of the baseline daily dose at follow-up; p = 0.001). Mean speed of prednisone reduction was - 13.1 mg of the daily dose for each month of follow-up for eculizumab-treated patients and - 3.2 for efgartigimod (p = 0.001). We found three serious events, all not related to treatment in the investigator's opinion. One eculizumab-treated patient experienced a severe pneumonia and died despite treatment.ConclusionsOur study provides evidence that eculizumab and efgartigimod can be used in clinical practice to reduce disability in gMG patients. Eculizumab-treated patients had a higher QMG response and steroid sparing effect. Efgartigimod may offer a more flexible schedule due to its cyclical use, no need for vaccination, and efficacy in AChR-Ab- patients.

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Evaluation of the Quality of Life of Patients with Myasthenia Gravis in Greece.

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Evaluation of the Quality of Life of Patients with Myasthenia Gravis in Greece.

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