scholarly journals Patients With Myasthenia Gravis With Acute Onset of Dyspnea: Predictors of Progression to Myasthenic Crisis and Prognosis

2021 ◽  
Vol 12 ◽  
Author(s):  
Yangyu Huang ◽  
Ying Tan ◽  
Jiayu Shi ◽  
Ke Li ◽  
Jingwen Yan ◽  
...  

Background: Life-threatening myasthenic crisis (MC) occurs in 10–20% of the patients with myasthenia gravis (MG). It is important to identify the predictors of progression to MC and prognosis in the patients with MG with acute exacerbations.Objective: This study aimed to explore the predictors of progression to MC in the patients with MG with acute onset of dyspnea and their short-term and long-term prognosis.Methods: This study is a retrospective cohort study. We collected and analyzed data on all the patients with MG with acute dyspnea over a 10-year period in a single center using the univariate and multivariate analysis.Results: Eighty-six patients with MG were included. In their first acute dyspnea episodes, 36 (41.9%) episodes eventually progressed to MC. A multivariate analysis showed that the early-onset MG (adjusted OR: 3.079, 95% CI 1.052–9.012) and respiratory infection as a trigger (adjusted OR: 3.926, 95% CI 1.141–13.510) were independent risk factors for the progression to MC, while intravenous immunoglobulin (IVIg) treatment prior to the mechanical ventilation (adjusted OR: 0.253, 95% CI 0.087–0.732) was a protective factor. The prognosis did not significantly differ between the patients with and without MC during the MG course, with a total of 45 (52.3%) patients reaching post-intervention status better than minimal manifestations at the last follow-up.Conclusion: When treating the patients with MG with acute dyspnea, the clinicians should be aware of the risk factors of progression to MC, such as early-onset MG and respiratory infection. IVIg is an effective treatment. With proper immunosuppressive therapy, this group of patients had an overall good long-term prognosis.

1997 ◽  
pp. 329-335
Author(s):  
B. C. G. Schalke ◽  
I. Schmitt ◽  
A. Marx ◽  
K. Toyka ◽  
H. K. Müller-Hermelink

Mediastinum ◽  
2017 ◽  
Vol 1 ◽  
pp. AB050-AB050
Author(s):  
Berthold Schalke ◽  
Christine Mayer ◽  
Rebecca Schneider ◽  
Sophie Schoetz ◽  
Roland Backhaus ◽  
...  

1987 ◽  
Vol 51 (3) ◽  
pp. 332-337 ◽  
Author(s):  
MIKIO ARITA ◽  
YUJI UENO ◽  
OSAMU MOHARA ◽  
HIDEYA NAKAMURA ◽  
YOSHIAKI TOMOBUCHI ◽  
...  

2014 ◽  
Vol 11 (7) ◽  
pp. 721-725 ◽  
Author(s):  
Guipeng An ◽  
Zhongqi Du ◽  
Xiao Meng ◽  
Tao Guo ◽  
Guishuang Li ◽  
...  

2017 ◽  
Vol 34 (11-12) ◽  
pp. 938-945 ◽  
Author(s):  
Qing Feng ◽  
Yu-Hang Ai ◽  
Hua Gong ◽  
Long Wu ◽  
Mei-Lin Ai ◽  
...  

Background: Sepsis and sepsis-associated encephalopathy (SAE) are common intensive care unit (ICU) diseases; the morbidity and mortality are high. The present study analyzed the sensitivity of different diagnostic criteria of sepsis 1.0 and 3.0, epidemiological characteristics of sepsis and SAE, and explored its risk factors for death, short-term, and long-term prognosis. Methods: The retrospective study included patients in ICU from January 2015 to June 2016. After excluding 58 patients, 175 were assigned to either an SAE or a non-SAE group (patients with sepsis but no encephalopathy). The sensitivity of the diagnostic criteria was compared between sepsis 1.0 and 3.0, respectively. Between-group differences in baseline data, Acute Physiology and Chronic Health Evaluation II score (APACHE II score), Sequential Organ Failure Assessment score (SOFA score), etiological data, biochemical indicators, and 28-day and 180-day mortality rates were analyzed. Survival outcomes and long-term prognosis were observed, and risk factors for death were analyzed through 180-day follow-up. Results: The sensitivity did not differ significantly between the diagnostic criteria of sepsis 1.0 and 3.0 ( P = .286). The 42.3% incidence of SAE presented a significantly high APACHE II and SOFA scores as well as 28-day mortality and 180-day mortality (all P < .001). The incidence of death was 37.1%. The multivariate stepwise regression analysis demonstrated that the risk of death in SAE group was significantly higher than the non-SAE group ( P < .001). Sepsis-associated encephalopathy is a risk factor for sepsis-related death (relative risk [RR] = 2.868; 95% confidence interval: 1.730-4.754; P < .001). Although males showed a significantly high rate of 28-day and 180-day mortality ( P = .035 and .045), it was not an independent risk factor for sepsis-related death ( P = .072). The long-term prognosis of patients with sepsis was poor with decreased quality of life. No significant difference was observed in prognosis between the SAE and non-SAE groups ( P > .05). Conclusion: Both diagnostic criteria cause misdiagnosis, and the sensitivity did not differ significantly. The incidence of SAE was high, and 28-day and 180-day mortality rates were significantly higher than those without SAE. Sepsis-associated encephalopathy is a risk factor for poor outcome. The overall long-term prognosis of patients with sepsis was poor, and the quality of life decreased.


Heart ◽  
2020 ◽  
Vol 107 (2) ◽  
pp. 135-141 ◽  
Author(s):  
Jung Ho Kim ◽  
Hi Jae Lee ◽  
Nam Su Ku ◽  
Seung Hyun Lee ◽  
Sak Lee ◽  
...  

ObjectiveThe treatment of infective endocarditis (IE) has become more complex with the current myriad healthcare-associated factors and the regional differences in causative organisms. We aimed to investigate the overall trends, microbiological features, and outcomes of IE in South Korea.MethodsA 12-year retrospective cohort study was performed. Poisson regression was used to estimate the time trends of IE incidence and mortality rate. Risk factors for in-hospital mortality were identified with multivariable logistic regression, and model comparison was performed to evaluate the predictive performance of notable risk factors. Kaplan-Meier survival analysis and Cox regression were performed to assess long-term prognosis.ResultsWe included 419 patients with IE, the incidence of which showed an increasing trend (relative risk 1.06, p=0.005), whereas mortality demonstrated a decreasing trend (incidence rate ratio 0.93, p=0.020). The in-hospital mortality rate was 14.6%. On multivariable logistic regression analysis, aortic valve endocarditis (OR 3.18, p=0.001), IE caused by Staphylococcus aureus (OR 2.32, p=0.026), neurological complications (OR 1.98, p=0.031), high Sequential Organ Failure Assessment score (OR 1.22, p=0.023) and high Charlson Comorbidity Index (OR 1.11, p=0.019) were predictors of in-hospital mortality. Surgical intervention for IE was a protective factor against in-hospital mortality (OR 0.25, p<0.001) and was associated with improved long-term prognosis compared with medical treatment only (p<0.001).ConclusionsThe incidence of IE is increasing in South Korea. Although the mortality rate has slightly decreased, it remains high. Surgery has a protective effect with respect to both in-hospital mortality and long-term prognosis in patients with IE.


2003 ◽  
Vol 26 (2) ◽  
pp. 170-173 ◽  
Author(s):  
L. Gogovska ◽  
R. Ljapcev ◽  
M. Polenakovic ◽  
L. Stojkovski ◽  
M. Popovska ◽  
...  

Background All patients with thymomatous Myasthenia Gravis (MG) should undergo early and total thymectomy, but controversy abounds in the choice of chronic immunosuppressive agents. The value of plasmaexchange (PE) in MG has been clearly estabilshed in preoperative preparation and treatment of myasthenic crisis. Whether PE may be used in the chronic long-term therapy of patients with thymomatous MG in addition to conventional immunosuppressive agents and cholinesterase inhibitors is yet to be answered. Case history We present a 40-year old woman with an 11 year history of MG. Thymectomy was done during the first year of the disease and the histopathologic finding was thymoma. To sustain clinical remission after thymectomy she continued with immunosuppression with methylprednisolone and cyclosporin A (or azathioprine) in addition to cholinesterase inhibitors. Despite the almost continuous immunosuppression, the disease course continued with fluctuating myasthenic weakness which few times progressed to myasthenic crisis requiring mechanical ventilation. During myasthenic crisis we performed 6–8 plasmapheresis at 2–3 day intervals in addition to conventional immunosuppressive therapy. The disease rapidly worsened in January 2000 and we started with intermittent plasmapheresis (3–6 procedures at 2–3 day intervals, every 6–8 weeks) in order to sustain remission. With this therapeutic protocol, during 20 months follow-up we managed to prevent myasthenic crisis and to avoid ventilatory support. Conclusions Plasmaexchange could be used as a successful and safe therapeutic tool in chronic long-term therapy in addition to conventional immunosuppressive agents to sustain remission in patients with MG. This is particularly important in the treatment of patients with thymomatous MG because they have an increased frequency of myasthenic crisis and often respond poorly an to immunosuppression with steroids or other immunosuppressants.


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