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Myasthenia gravis: descriptive analysis of life-threatening events in a recent nationwide registry
A Ramos-Fransi1, R Rojas-García1,2,3, S Segovia1
1Neuromuscular Disorders Unit, Neurology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Background And Purpose:
Myasthenia gravis (MG) may become life-threatening if patients have respiratory insufficiency or dysphagia. This study aimed to determine the incidence, demographic characteristics, risk factors, response to treatment and outcome of these life-threatening events (LTEs) in a recent, population-based sample of MG patients.
Methods:
A retrospective analysis of MG patients who presented with an LTE between 2000 and 2013 was performed. Participants were identified from a neuromuscular diseases registry in Spain that includes 648 patients with MG (NMD-ES).
Results:
Sixty-two (9.56%) patients had an LTE. Thirty-two were classified as class V according to the MG Foundation of America, and 30 as class IVB. Fifty per cent were previously diagnosed with MG and median duration of the disease before the LTE was 24 months (3-406). The most common related factor was infection (n = 18). All patients received intravenous human immunoglobulin; 11 had a second infusion and six had plasma exchange. Median time to feeding tube removal was 13 days (1-434). Median time to weaning from ventilation was 12 days (3-176), and it was significantly shorter in late onset MG (≥50 years) (P = 0.019). LTEs improved <2 weeks in 55.8% but did not improve until after 1 month in 20% of patients. Four patients died. No other factors influenced mortality or duration of LTEs.
Conclusions:
The percentage of LTEs in MG patients was low, particularly amongst those previously diagnosed and treated for the disease. The significant percentage of treatment-resistant LTEs indicates that more effective treatment approaches are needed for this vulnerable sub-population.
Insights
Life-threatening events (LTEs) in myasthenia gravis (MG) patients occurred in 9.56%, often due to infection. While many improved with treatment, some were resistant, highlighting the need for better therapeutic strategies for this vulnerable group.
Area of Science:
- Neurology
- Clinical Medicine
- Immunology
Background:
- Myasthenia gravis (MG) can lead to life-threatening respiratory insufficiency or dysphagia.
- Understanding the incidence and characteristics of these life-threatening events (LTEs) is crucial for patient management.
Purpose of the Study:
- To determine the incidence, demographics, risk factors, treatment response, and outcomes of LTEs in a population-based sample of MG patients.
- To identify factors influencing LTEs and their management.
Main Methods:
- Retrospective analysis of MG patients experiencing LTEs between 2000 and 2013.
- Data sourced from a Spanish neuromuscular diseases registry (NMD-ES) including 648 MG patients.
Main Results:
- 9.56% of MG patients experienced LTEs, most commonly linked to infection.
- Intravenous immunoglobulin was a primary treatment; median recovery times for ventilation and feeding tubes were 12 and 13 days, respectively.
- 55.8% improved within 2 weeks, but 20% required over a month for improvement; mortality was low (4 patients).
Conclusions:
- LTEs in MG patients are relatively uncommon, especially in those previously diagnosed and treated.
- A significant proportion of LTEs were treatment-resistant, indicating a need for enhanced therapeutic strategies for this high-risk population.
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