Acute Interstitial Pneumonia-Hamman-Rich Syndrome: Clinical Characteristics and Diagnostic and Therapeutic Considerations
Acute Interstitial Pneumonia-Hamman-Rich Syndrome: Clinical Characteristics and Diagnostic and Therapeutic Considerations
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DOI：
10.1213/ane.0b013e318188af7a
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发表时间：
2009-01-01
影响因子：
5.7
通讯作者：
Almog, Yaniv
中科院分区：
文献类型：
--
作者：
Avnon, Lone S.;Pikovsky, Oleg;Almog, Yaniv
背景：急性间质性肺炎是一种进展迅速的疾病，常导致呼吸衰竭和机械通气。尽管积极的诊断和治疗efforts.METHODS：在这项回顾性队列调查中，我们招募了符合急性特发性间质性肺炎预定标准的低氧性呼吸衰竭患者。回顾病人的病历、放射学检查和病理标本。所有数据记录在每个病人的研究文件，随后analysed.RESULTS：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）。所有患者的胸片在12天的病程中进展为弥漫性双侧浸润。所有9例患者均有弥漫性肺炎的组织学证据，通过与急性间质性经支气管活检或开放性肺活检一致的肺泡损伤获得。所有患者均需入住重症监护室和机械通气。死亡率为100%，患者在5-26天内死亡，他们入院的unit.CONCLUSIONS：急性间质性肺炎（哈曼-里奇综合征）是在特发性，迅速进行性，有时，致命的形式间质性疾病。经支气管活检是合理的第一诊断步骤，如有必要，随后进行所有开放性肺活检。在我们的系列中，对皮质类固醇的反应很小。对于那些对常规治疗无反应的患者，肺移植可能被认为是一种额外的选择。
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 surveys, we enrolled patients with hypoxemic respiratory failure who met predefined criteria of acute idiopathic interstitial pneumonia. Patients' records, radiologic studies, and pathologic specimens were In 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 pneumonia, obtained by either alveolar damage consistent with acute interstitial 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 In idiopathic, rapidly progressive and, at times, fatal form of interstitial long disease. A transbronchial biopsy is a logical first diagnostic step, to be followed by all 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.