Noda Yoshimi, Shiroyama Takayuki, Amiya Saori, Adachi Yuichi, Enomoto Takatoshi, Hara Reina, Niitsu Takayuki, Miyake Kotaro, Hirata Haruhiko, Takeda Yoshito, Kumanogoh Atsushi
Department of Respiratory Medicine and Clinical Immunology, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita City, Osaka, 565-0871, Japan.
Respir Med Case Rep. 2021;34:101505. doi: 10.1016/j.rmcr.2021.101505. Epub 2021 Sep 3.
Coronavirus disease 2019 (COVID-19) is an emerging viral disease with a mortality that depends on the individual's condition. Underlying comorbidities are major risk factors for COVID-19-related morbidity and mortality. However, information regarding the clinical course of COVID-19 in patients with rare respiratory system diseases is lacking. Here, we present a case of severe COVID-19 in a patient with advanced sporadic lymphangioleiomyomatosis (LAM) who was awaiting lung transplantation. She experienced a marked worsening of her respiratory status despite the limited size of the infiltrations seen on chest computed tomography. She responded to treatment with dexamethasone and remdesivir, and did not require mechanical ventilation. She recovered her pre-COVID-19 respiratory function. This case illustrates that patients with severe lung parenchymal destruction due to advanced LAM are at risk of worsening hypoxemia, but may not have a bad outcome if managed appropriately. Prevention and early diagnosis of COVID-19 are crucial in patients with advanced LAM. Future studies are needed to improve understanding of the clinical features and optimal treatment of COVID-19 in patients with LAM.
2019冠状病毒病（COVID-19）是一种新出现的病毒性疾病，其死亡率取决于个体状况。基础合并症是COVID-19相关发病和死亡的主要危险因素。然而，关于罕见呼吸系统疾病患者中COVID-19临床病程的信息尚缺。在此，我们报告一例晚期散发性淋巴管平滑肌瘤病（LAM）且正在等待肺移植的患者发生重症COVID-19的病例。尽管胸部计算机断层扫描显示的浸润范围有限，但她的呼吸状况仍显著恶化。她对用地塞米松和瑞德西韦治疗有反应，且不需要机械通气。她恢复到了感染COVID-19之前的呼吸功能。该病例表明，因晚期LAM导致严重肺实质破坏的患者有低氧血症恶化的风险，但如果处理得当可能不会有不良结局。对于晚期LAM患者，COVID-19的预防和早期诊断至关重要。需要开展进一步研究以增进对LAM患者中COVID-19临床特征及最佳治疗方法的了解。
