Independent risk factors for in-hospital outcome of myasthenic crisis: a prospective cohort study.

IF 5.4 3区材料科学 Q2 CHEMISTRY, PHYSICAL ACS Applied Energy Materials Pub Date : 2024-02-09 eCollection Date: 2024-01-01 DOI:10.1177/17562864241226745

Yuan Wang, Xiao Huan, Xinfang Zhu, Jie Song, Chong Yan, Lei Yang, Caihua Xi, Yafang Xu, Jianying Xi, Chongbo Zhao, Rong Xia, Sushan Luo

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Abstract

Background: Myasthenic crisis (MC) is a life-threatening condition for myasthenia gravis (MG). Therapeutic plasma exchange (TPE) and intravenous immunoglobulin (IVIg) efficaciously treat patients with MC. However, not every MC responds well to rescue therapies, and the determinants for outcome with the evidence from prospective cohorts are still lacking.

Objectives: To explore the risk factors for in-hospital outcomes in patients with MC.

Methods: Using a national neuromuscular center-based cohort of MG with prospective follow-ups from the crisis to the post-crisis phase, we finally included 90 MC episodes from 76 independent patients who received a standard regimen of rescue therapies.

Results: The mean admission age was 52.89 ± 15.72 years. With a female predominance of 63.16% (48/76) and a high proportion of thymoma-associated MG (TMG) of 63.16% (48/76), the overall in-hospital mortality was 2.63% (2/76) and the average duration for mechanical ventilation (MV) use was 17.09 ± 13.36 days (0-53 days). In contrast to the patients with anti-acetylcholine receptor (AChR) antibodies, muscle-specific tyrosine kinase (MuSK)-associated MC exhibited a shorter MV support (5.20 ± 5.07 versus 17.40 ± 13.24 days, p = 0.023), length of intensive care units (ICU) stay (6.00 ± 4.64 versus 19.16 ± 17.54 days, p = 0.046), and hospital stay (16.00 ± 4.12 versus 34.43 ± 20.48 days, p = 0.011). Thymoma [odds ratio (OR): 0.200, 95% confidence interval (CI): 0.058-0.687, p = 0.011], partial pressure of carbon dioxide (PCO₂) in blood gas before MV (OR: 1.238, 95% CI: 1.015-1.510, p = 0.035), and pneumonia (OR: 0.204, 95% CI: 0.049-0.841, p = 0.028) were identified as independent risk factors for prolonged MV use. TMG patients with thymoma burden exhibited a notable longer MV use (22.08 ± 17.54 versus 8.88 ± 6.79 days, p = 0.001), a prolonged hospital stay (40.40 ± 26.13 versus 23.67 ± 13.83 days, p = 0.009) compared with non-TMG. Even with complete thymoma resection (R0), TMG exhibited an unfavorable outcome versus non-TMG.

Conclusion: With timely rescue therapies and prospective follow-ups, the in-hospital outcome of MCs was substantially improved. Thymoma, PCO₂ in blood gas before MV, and pneumonia were identified as independent risk factors for prolonged MV use.

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肌无力危象院内预后的独立风险因素：一项前瞻性队列研究。

背景：肌无力危象（MC）是一种危及重症肌无力（MG）患者生命的疾病。治疗性血浆置换（TPE）和静脉注射免疫球蛋白（IVIg）可有效治疗 MC 患者。然而，并不是每一位重症肌无力患者都能对抢救疗法做出良好反应，而且目前仍缺乏前瞻性队列研究的证据来确定治疗结果的决定因素：目的：探讨MC患者院内预后的风险因素：我们使用了一个基于全国神经肌肉中心的 MG 队列，从危机到危机后阶段进行了前瞻性随访，最终纳入了 76 名独立患者的 90 次 MC 事件，这些患者接受了标准的抢救治疗方案：平均入院年龄为（52.89 ± 15.72）岁。女性占 63.16%（48/76），胸腺瘤相关 MG（TMG）占 63.16%（48/76），院内总死亡率为 2.63%（2/76），使用机械通气（MV）的平均时间为 17.09 ± 13.36 天（0-53 天）。与抗乙酰胆碱受体（AChR）抗体患者相比，肌肉特异性酪氨酸激酶（MuSK）相关 MC 的机械通气支持时间更短（5.20 ± 5.07 对 17.40 ± 13.36 天，P = 0.0.0）。24 天，p = 0.023）、重症监护室（ICU）住院时间（6.00 ± 4.64 对 19.16 ± 17.54 天，p = 0.046）和住院时间（16.00 ± 4.12 对 34.43 ± 20.48 天，p = 0.011）。胸腺瘤[几率比（OR）：0.200，95% 置信区间（CI）：0.058-0.687，p = 0.011]、MV 前血气中二氧化碳分压（PCO2）（OR：1.238，95% CI：1.015-1.510，p = 0.035）和肺炎（OR：0.204，95% CI：0.049-0.841，p = 0.028）被确定为延长 MV 使用时间的独立风险因素。与非胸腺瘤患者相比，有胸腺瘤负担的 TMG 患者使用 MV 的时间明显更长（22.08 ± 17.54 对 8.88 ± 6.79 天，p = 0.001），住院时间也更长（40.40 ± 26.13 对 23.67 ± 13.83 天，p = 0.009）。即使胸腺瘤完全切除（R0），TMG与非TMG相比也显示出不利的预后：结论：通过及时的抢救治疗和前瞻性随访，MC 的院内预后得到了显著改善。胸腺瘤、中风前血气中的 PCO2 和肺炎被确定为中风使用时间延长的独立风险因素。

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来源期刊

ACS Applied Energy Materials Materials Science-Materials Chemistry

CiteScore

10.30

自引率

6.20%

发文量

1368

期刊介绍： ACS Applied Energy Materials is an interdisciplinary journal publishing original research covering all aspects of materials, engineering, chemistry, physics and biology relevant to energy conversion and storage. The journal is devoted to reports of new and original experimental and theoretical research of an applied nature that integrate knowledge in the areas of materials, engineering, physics, bioscience, and chemistry into important energy applications.