Prognosis of Patients with Idiopathic Pulmonary Fibrosis Admitted to the Intensive Care Unit

原發性肺纖維化患者於加護病房之預後探討

簡榮彥(Jung-Yien Chien) ； 鄭之勛(Jih-Shuin Jerng) ； 余忠仁(Chong-Jen Yu) ； 楊泮池(Pan-Chyr Yang)

《胸腔醫學》 19卷3期 (2004/06) Pp. 168-177

https://doi.org/10.29806/TM.200406.0001

摘要

前言：原發性肺纖維化為一不明原因的間質性肺病，罹患此症會導致漸進性肺纖維化和呼吸衰竭。本研究的目的在探討導致患者呼吸衰竭而需要加護病房照顧的原因，其臨床表現及影響預後的因子。
材料與方法：分析台大醫院1999年一月至2003年四月之間，內科加護病房中罹患原發性肺纖維化患者的臨床資料及預後。
結果：總共有13位患者符合原發性肺纖維化的診斷，其中包括11位男性，中位數年齡為72歲（56-87歲）。九位患者在住院前已經在使用類固醇類藥物。大部分呼吸衰竭的加重因子為肺炎。七位患者(54%)在加護病房中接受類固醇治療。敗血症發生於11位患者(85%)，其中6位患者(46%)發生敗血性休克而共有七位患者(54%)產生多器官衰竭。加護病房死亡率為62%而住院死亡率增加至85%。單變數存活分析顯示住進加護病房前有較長症狀者，若發生敗血症者及較多器官衰竭則會有較高的死亡率。進一步的多變數存活分析顯示發生敗血症(hazard ratio=16.69; p=0.04)及增加器官衰竭數目者（每增加一個器官衰竭hazard ratio增加3.42; P=0.04）是影響預後的獨立因子。
結論：原發性肺纖維化患者因呼吸衰竭而需要加護病房照顧者若發生敗血症及多器官衰竭會有較差的預後。

關鍵字

肺纖維化 ； 呼吸衰竭 ； 呼吸器治療

並列摘要

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive diffuse parenchymal lung disease of unknown etiology. Most IPF patients develop a progressive restrictive lung disease as a result of progressive lung fibrosis, and respiratory failure eventually mandates mechanical ventilation. This study was aimed at studying the clinical features of patients with IPF admitted to the intensive care unit (ICU) due to acute respiratory failure, so as to investigate their mortality and prognostic factors.
Materials and Methods: We retrospectively reviewed the medical records of all patients with IPF admitted to the medical CU of National Taiwan University Hospital, Taipei, Taiwan, from January 1999 to April 2003.
Results: A total of 13 eligible patients, including 10 men, with a median age of 72(56-87) years, were identified. Nine patients had been on corticosteroids already at the time of admission. The most common precipitating factor for respiratory failure was pneumonia. Sepsis developed in 11(85%) patients, while septic shock developed in 6(46%), and multiple organ failure occurred in 7(54%). Seven patients (54%) received immunosuppressive therapy in the ICU. The ICU and hospital mortality rates were 62% and 85%, respectively. Univariate analysis showed that the development of septic shock and the increased number of cases of organ failure were associated with significantly higher mortality. Multivariate survival analysis showed that the development of septic shock (hazard ratio=16.69; p=0.04) and the increasing number of organ failure (HR=3.42 per additional dysfunctional organ system; p=0.04) were independent factors associated with significantly higher mortality.
Conclusions: These findings suggest that IPF patients admitted to the ICU with acute respiratory failure have a poor prognosis in the presence of septic shock or multiple organ failure.

並列關鍵字

pulmonary fibrosis ； respiratory failure ； mechanical ventilation