Abstract

BackgroundMyasthenia gravis (MG) is an autoimmune disease involving the neuromuscular junction. Myasthenic crisis (MC), which is characterized by respiratory failure and the requirement of mechanical ventilation in patients with MG, is still a medical emergency despite the decrease in mortality with the advances in acute management. Hemogram is a cost-effective test for evaluating hematological complications and systemic inflammation, and hemogram data have been used to predict various clinical outcomes of several diseases. The relationship between hemogram and MG has been discussed, but the role of hemogram data in predicting the prognosis of MC patients has not been established.MethodsTo identify whether hemogram data can predict in-hospital mortality in patients with MC, we retrospectively investigated 188 myasthenic crisis events from the Chang Gung Research Database between April 2001 and March 2019. Demographic and clinical characteristics were collected, as well as hemogram data before intubation and extubation. The endpoints were mortality during mechanical ventilation and mortality after extubation.ResultsThe overall in-hospital mortality rate was 22%. Multivariate logistic regression analysis for predicting mortality during mechanical ventilation showed that old age at MC onset (OR = 1.039, p = 0.022), moderate-to-severe anemia (OR = 5.851, p = 0.001), and extreme leukocytosis (OR = 5.659, p = 0.022) before intubation were strong predictors of mortality, while acute management with plasma exchange or double-filtration plasmapheresis (PE/DFPP) significantly decreased mortality (OR = 0.236, p = 0.012). For predicting mortality after extubation, moderate-to-severe anemia before extubation (OR = 8.452, p = 0.017) and non-treated with disease-modifying therapy before MC (OR = 5.459, p = 0.031) were crucial predictive factors.ConclusionThis study demonstrated that both old age at MC onset and moderate-to-severe anemia are important predictors of in-hospital mortality in patients with MC, and extreme leukocytosis is another crucial predictor of mortality during mechanical ventilation. The suggested mechanism is that anemia-induced hypoxia may enhance the release of proinflammatory cytokines, exacerbate systemic inflammation, and lead to multiple organ dysfunction syndrome and, finally, mortality.

Highlights

  • Myasthenia gravis (MG) is an autoimmune disease caused by autoantibodies against nicotinic acetylcholine receptors or related molecules on the postsynapticHsu et al BMC Neurol (2021) 21:388 muscle membrane of the neuromuscular junction [1]

  • Forty-five cases of Myasthenic crisis (MC) occurred at the moment of MG diagnosis; they did not receive any medication for MG before hospitalization

  • Reviewing the medical treatment for MG before hospitalization in the other 143 cases, only symptomatic treatment with pyridostigmine was administered for 33 cases, and disease-modifying therapy (DMT) with or without prednisolone was administered for 110 cases

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Summary

Introduction

Myasthenia gravis (MG) is an autoimmune disease caused by autoantibodies against nicotinic acetylcholine receptors or related molecules on the postsynapticHsu et al BMC Neurol (2021) 21:388 muscle membrane of the neuromuscular junction [1]. Myasthenic crisis (MC) is a medical emergency characterized by respiratory failure and the requirement of mechanical ventilation because of acute deterioration of respiratory dysfunction and bulbar muscle weakness in MG patients [3]. Myasthenia gravis (MG) is an autoimmune disease involving the neuromuscular junction. Myasthenic crisis (MC), which is characterized by respiratory failure and the requirement of mechanical ventilation in patients with MG, is still a medical emergency despite the decrease in mortality with the advances in acute management. Hemo‐ gram is a cost-effective test for evaluating hematological complications and systemic inflammation, and hemogram data have been used to predict various clinical outcomes of several diseases. The relationship between hemogram and MG has been discussed, but the role of hemogram data in predicting the prognosis of MC patients has not been established

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