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急性间质性肺炎-哈曼-里奇综合征:临床特征及诊断与治疗考量

Acute interstitial pneumonia-Hamman-Rich syndrome: clinical characteristics and diagnostic and therapeutic considerations.

作者信息

Avnon Lone S, Pikovsky Oleg, Sion-Vardy Neta, Almog Yaniv

机构信息

Division of Pulmonary Medicine, Soroka University Medical Center, Ben Gurion University of the Negev, Beer-Sheva, Israel.

出版信息

Anesth Analg. 2009 Jan;108(1):232-7. doi: 10.1213/ane.0b013e318188af7a.

DOI:10.1213/ane.0b013e318188af7a
PMID:19095855
Abstract

BACKGROUND

Acute interstitial pneumonia is a rapidly progressive disease frequently leading to respiratory failure and mechanical ventilation. The prognosis is usually poor despite aggressive diagnostic and treatment efforts.

METHODS

In this retrospective cohort survey, we enrolled patients with hypoxemic respiratory failure who met predefined criteria of acute idiopathic interstitial pneumonia. Patients' records, radiologic studies, and pathologic specimens were reviewed. All data were recorded in each patient's study file and subsequently analyzed.

RESULTS

Our cohort consisted of 5 men and 4 women with a mean age of 69.4 yr (55-80 yr). The chest radiograph in all patients progressed to diffuse bilateral infiltrates over a 12-day course. All nine patients had histological proof of diffuse alveolar damage consistent with acute interstitial pneumonia, obtained by either transbronchial biopsy or open lung biopsy. All patients required admission to the medical intensive care unit and mechanical ventilation. The mortality rate was 100%, and patients died within 5-26 days of their admission to the unit.

CONCLUSIONS

Acute interstitial pneumonia (Hamman-Rich syndrome) is an idiopathic, rapidly progressive and, at times, fatal form of interstitial lung disease. A transbronchial biopsy is a logical first diagnostic step, to be followed by an open lung biopsy, if necessary. Response to corticosteroids in our series was minimal. In patients who fail to respond to conventional therapy and are otherwise appropriate candidates, lung transplantation may be considered as an additional alternative.

摘要

背景

急性间质性肺炎是一种迅速进展的疾病,常导致呼吸衰竭和机械通气。尽管进行了积极的诊断和治疗,其预后通常较差。

方法

在这项回顾性队列研究中,我们纳入了符合急性特发性间质性肺炎预定义标准的低氧性呼吸衰竭患者。对患者的病历、影像学检查和病理标本进行了回顾。所有数据均记录在每位患者的研究档案中,随后进行分析。

结果

我们的队列包括5名男性和4名女性,平均年龄为69.4岁(55 - 80岁)。所有患者的胸部X线片在12天内进展为双侧弥漫性浸润。所有9例患者均通过经支气管活检或开胸肺活检获得了与急性间质性肺炎一致的弥漫性肺泡损伤的组织学证据。所有患者均需入住医学重症监护病房并接受机械通气。死亡率为100%,患者在入住病房后5 - 26天内死亡。

结论

急性间质性肺炎(Hamman - Rich综合征)是一种特发性、迅速进展且有时致命的间质性肺疾病形式。经支气管活检是合理的首要诊断步骤,必要时可继以开胸肺活检。在我们的系列研究中,患者对皮质类固醇的反应极小。对于对传统治疗无反应且其他方面合适的患者,可考虑将肺移植作为一种额外的选择。

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