OBJECTIVE: To investigate the level and influencing factors of health-related quality of life in myasthenia gravis (MG) patients with myasthenia gravis quality of life-15 (MGQOL-15) Chinese version and to provide corresponding measures in one tertiary hospital of Sichuan Province. METHODS: We collected the general data (gender, age, body mass index BMI, marital status, educational level and employee status), clinical data [Osserman type, myasthenia gravis composite (MGC), other immunopathies, disease duration, frequency of outpatient visits per month, ratio of disease cost to income each month and frequency of symptoms during the past month] and the MGQOL-15 Chinese version from 168 myasthenia gravis patients in one tertiary hospital of Sichuan Province. RESULTS: The mean score of MGQOL-15 was 17.67±12.78. The score of the item "My occupational skills and job status have been negatively affected." was the highest, followed by "I have trouble using my eyes." and "I am frustrated by my MG." Single factor analysis showed that MG patients' QOL were different with different disease severity MGC (F=19.353, P<0.001), ratio of disease cost to income each month (F=5.831, P<0.001) and the frequency of symptoms during the past month (F=9.128,P<0.001). Multiple regression analysis showed that disease severity MGC (β=0.743,P<0.001), ration of disease cost to income each month (β=3.347,P<0.001) and the frequency of symptoms during the past month (β=2.216,P<0.003) were the main predictors of HRQOL in the MG patients. CONCLUSION Our study showed that the MGQOL-15 is helpful for clinicians to evaluate MG patients' QOL regularly, investigate the influencing factors and implement corresponding interventions the so as to improve the patients' quality of life. Disease severity MGC, ratio of disease cost to income each month and the frequency of symptoms during the past month were the main predictors of MG patients' QOL. Clinicians should pay more attention to MG patients' disease severity MGC and the frequency of symptoms during the past month.
Adult ; Aged ; Cost of Illness ; Female ; Humans ; Income ; Male ; Marital Status ; Middle Aged ; Myasthenia Gravis/psychology* ; Quality of Life

OBJECTIVETo evaluate a scale of patient-reported outcomes for the assessment of myasthenia gravis patients (MG-PRO) in China.

METHODSA total of 100 MG patients were interviewed for the field testing. Another 56 MG patients were selected and assessed with the MG-PRO scale before treatment and at 1, 2 and 4 weeks after treatment. The classical test theory and item response theory (IRT) were used to assess the psychometric characteristics of the MG-PRO scale.

RESULTSThe MG-PRO scale included 4 dimensions: physical, psychological, social environment, and treatment. Confirmatory factor analysis showed that each dimension was consistent with the theoretical construct. The scores of the physical and psychological dimensions increased significantly at 1 week after treatment (P<0.05). All the dimension scores and the MG-PRO score increased significantly at 2 and 4 weeks after treatment (P<0.05). IRT showed that person separation indices were greater than 0.8, most of the item fit residual statistics were within ± 2.5, and no item had uniform or non-uniform differential item functioning (DIF) between gender and age (<40, [Symbol: see text]40).

CONCLUSIONSThe MG-PRO scale is valid for measuring the quality of life (QOL) of MG patients, with good reliability, validity, responsiveness, and good psychometric characteristics from IRT. It can be applied to evaluate the QOL of MG patients and to assess treatment effects in clinical trials.

Adolescent ; Adult ; Aged ; China ; Female ; Humans ; Male ; Middle Aged ; Myasthenia Gravis ; psychology ; therapy ; Social Environment ; Surveys and Questionnaires ; Treatment Outcome ; Young Adult

Azathioprine is an immunosuppressant that has been widely used in the prevention of organ post-transplantation rejection, rheumatoid arthritis, inflammatory bowel disease and myasthenia gravis. The adverse effects reported with this drug have been classified as early events that include fever, hypotension, vasculitis and erythema nodosum-like eruptions or as late events that include bone marrow depression, nausea and vomiting. Although erythema nodosum-like eruptions could be useful for recognition of azathioprine hypersensitivity, this eruption has not yet been reported in Korean dermatological literature. We report, herein, a 68-year-old female with myasthenia gravis, who presented with tender, erythema nodosum-like eruptions on both upper and lower extremities 14 days after the first administration of azathioprine. Her symptoms improved 2 days after discontinuation of azathioprine and healed with no scarring change.
Aged ; Arthritis, Rheumatoid ; Azathioprine ; Bone Marrow ; Cicatrix ; Depression ; Erythema ; Erythema Nodosum ; Female ; Fever ; Humans ; Hypersensitivity ; Hypotension ; Inflammatory Bowel Diseases ; Lower Extremity ; Myasthenia Gravis ; Nausea ; Rejection (Psychology) ; Vasculitis ; Vomiting

PURPOSE: Myasthenia gravis(MG) is relatively rare in childhood and shows some distinct epidemiologic and clinical features according to ages and races. The role of immunosuppressive therapy such as steroid treatment is not well established. The objective of this study is to characterize clinical features of childhood MG and to evaluate the outcomes of steroid treatment. METHODS: We studied 23 MG patients(7 male and 16 females) with symptom onsets from 1 to 14 years of age(mean 4.1 years). RESULTS: Fifteen patients were ocular type and 6 patients were generalized type. Sero-positivity to acetylcholine receptor antibodies was found in 85% of the patients. Two patients who showed initial ocular manifestations experienced secondary generalization without steroid treatment. Alopecia totalis and moyamoya disease were associated with systemic MG in two cases. Eighteen patients were treated with both anticholine esterase and steroid while 2 patients were treated with steroid only. Complete remission was observed in 6 patients(30%) while 11 patients(55%) had one or more recurrences and only partial responses were acquired in 4 patients(20%). CONCLUSION: We found some different epidemiologic features from western countries including a relatively high proportion of ocular MG and earlier onset age especially in ocular MG. Further long-term follow up study on responses to steroid treatment will be necessary to evaluate clinical benefits in the disease progression.
Acetylcholine ; Age of Onset ; Alopecia ; Antibodies ; Continental Population Groups ; Disease Progression ; Follow-Up Studies ; Generalization (Psychology) ; Humans ; Male ; Moyamoya Disease ; Myasthenia Gravis* ; Recurrence

BACKGROUND & PURPOSE: Thymic tumors are frequently found in patients with myasthenia gravis(MG); the patients with thymoma seem to be more associated with severe MG. This study was performed to analyse the clinical characteristics of thymic tumor-associated MGs and to compare the patients with invasive and noninvasive thymomas. METHODS: We retrospectively reviewed the medical records of 305 MG subjects(M:F = 112:193, age = 30.4 ? 8.1 years) who had been diagnosed to have MG from January 1986 to June 1996, and analysed the clinical characteristics of MG subjects with thymic tumors. Forty seven patients were confirmed to have thymic tumor by the postoperative histopathologic findings(thymic tumor(TT) group) and 191 were known to have nonneoplastic thymus by chest CT or postoperative histopathologic findings(nonneoplastic thymus(NN) group). RESULTS: The incidence of thymic tumor-associated MG was 25.2%. The male to female ratio for the TT and the NN groups were 1:1 and 1:2, and the mean age of onset were 42.9 ? 11.2 years and 28.3 ? 17.5 years, respectively. When we evaluated the disease severity by Osserman's classification, 85% of the TT group and 51% of NN group were severely affected(Iib, III, IV). The generalization of symptoms occurred in 98% of the TT group and 66% of the NN group. The disease severity and the clinical outcome were not significantly different between the MGs with invasive thymoma, noninvasive thymoma and thymic carcinoma. CONCLUSION: Compared with those of MGs with nonneoplastic thymus, the thymic tumor-associated MGs showed several different clinical phenotypes, as in the followings; they showed no sex predominance, the onset age was older, more common generalization of myasthenic symptoms, and more patients were severely affected. The severity and outcome were not different between the MGs with thymic tumor.
Age of Onset ; Classification ; Female ; Generalization (Psychology) ; Humans ; Incidence ; Male ; Medical Records ; Myasthenia Gravis* ; Phenotype ; Retrospective Studies ; Thymectomy ; Thymoma ; Thymus Gland ; Thymus Neoplasms ; Tomography, X-Ray Computed