Prognosis of Patients with DM/PM Requiring Intensive Care Due to Acute Respiratory Failure

皮肌炎及多發性肌炎病人因急性呼吸衰竭需要加護醫療之預後

陳欣怡(Hsin-Yi Chen) ； 葉步盛(Pu-Sheng Yeh) ； 余忠仁(Chong-Jen Yu) ； 楊泮池(Pan-Chyr Yang) ； 陸坤泰(Kwen-Tay Luh)

《胸腔醫學》 21卷5期 (2006/10) Pp. 422-432

https://doi.org/10.29806/TM.200610.0004

摘要

背景：目前為止，尚沒有關於皮肌炎(dermatomyositis)及多發性肌炎(polymyositis)病人因急性呼吸衰竭需要加護醫療之預後之報告。本文以回溯性分析此類病人的臨床病程及預後。
方法：我們回顧自1985年1月至2004年12月間，曾住進加護病房病人的醫療紀錄，將診斷符合皮肌炎多發性肌炎，並且發生呼吸衰竭而住到加護病房的病人納入研究。皮肌炎／多發性肌炎之診斷，是根據Bohan及Peter二人所訂定的診斷條件。
結果：在研究期間，共有19位診斷為皮肌炎／多發性肌炎病人，因呼吸衰竭住到加護病房。其中有8位病人被診斷有皮肌炎／多發性肌炎相關之間質性肺病。呼吸衰竭的發生，平均在皮肌炎／多發性肌炎診斷後的14個月發生。導致呼吸衰竭的原因有：肺炎(n=14)，間質性肺病(n=5)，通氣衰竭(ventilatory failure)(n=2)，及急性肺水腫(n=1)。最常發生的併發症為敗血性休克(n=16)，其次為急性呼吸窘迫症候群(n=13)及急性腎衰竭(n=9)。共有12位病患死亡，死亡率63%。其死因包括：肺炎(n=7)、敗血性休克(n=3)、間質性肺病併發呼吸衰竭(n=2)、肝衰竭(n=1)。死亡病人中，92%發生急性呼吸窘迫症候群；而所有間質性肺病之患者，均發生急性呼吸窘迫症候群。後者常對免疫抑制治療無效；反之那些因通氣衰竭的病患，對治療則有不錯的反應。
結論：皮肌炎／多香性肌炎病人，併發呼吸衰竭而需要加護醫療照顧者，死亡率極高，最常見的死因為肺炎。

關鍵字

皮肌炎 ； 多發性肌炎 ； 呼吸衰竭

並列摘要

Background: The prognosis of patients with dermatomyositis/polymyositis (DM/PM) requiring intensive care for acute respiratory failure has not been reported. A retrospective study was conducted to analyze the clinical course and outcome of these patients.
Methods: Medical records were reviewed for patients admitted to the intensive care unit from January 1985 to December 2004. The diagnosis of DM/PM was based upon Bohan and Peter's criteria, and those patients with respiratory failure as the indication for ICU admission were enrolled.
Results: Nineteen patients with DM/PM were admitted to the ICU because of respiratory failure during the study period. Eight patients were diagnosed with interstitial lung disease. Respiratory failure developed within a mean of 14 months after the diagnosis of DM/PM. The causes of respiratory failure were: pneumonia (n=14), interstitial lung disease (ILD) (n=5), ventilatory failure (n=2), and acute lung edema (n=1). The most common complications were septic shock (n=16), followed by ARDS (n=13), and acute renal failure (n=9). Twelve patients died, with a mortality rate of 63%. The causes of death were: pneumonia (n=6), septic shock (n=3), ILD with respiratory failure (n=2), and hepatic failure (n=1). Ninety-two percent of the expired patients and all of those with ILD developed ARDS. ILD patients were often refractory to immunosuppressive treatment while patients with ventilatory failure had a good response to therapy.
Conclusion: Patients with DM/PM and respiratory failure requiring ICU admission had an extremely high mortality rate, and most died of pneumonia.

並列關鍵字

dermatomyositis ； polymyositis ； respiratory failure