Acute Exacerbations of Fibrotic Hypersensitivity Pneumonitis A Case Series
Acute Exacerbations of Fibrotic Hypersensitivity Pneumonitis A Case Series
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DOI：
10.1378/chest.08-0428
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发表时间：
2008-10-01
期刊：
影响因子：
9.6
通讯作者：
Frankel, Stephen K.
中科院分区：
文献类型：
--
作者：
Olson, Amy L.;Huie, Tristan J.;Frankel, Stephen K.
背景资料：现在认识到，相当一部分特发性肺纤维化（IPF）患者在病程中可具有不能由感染、心力衰竭或血栓栓塞性疾病解释的突然和快速恶化。这些事件通常是致死性的，被称为基础疾病的急性加重（AE）。虽然在IPF患者中描述最好，但在其他形式的间质性肺病患者中也有报告。我们试图确定过敏性肺炎（HP）患者是否也会出现同样的现象。方法：我们回顾性分析了我们在国家犹太医学研究中心的临床经验，这些患者经手术肺活检证实为纤维化HP，呼吸状态急性下降，符合IPF AE诊断标准。结果：在2年期间，我们确定了4例纤维化HP AE患者。所有患者的临床病程与IPF AE中最常见的相似：需要辅助通气的呼吸衰竭、对高剂量皮质类固醇治疗缺乏临床应答和预后不良（所有病例均导致死亡或紧急肺移植）。当时的肺活检。AE，外植体，或尸检发现组织弥漫性肺泡损伤叠加纤维化肺diseases.Conclusions：纤维化HP，像其他形式的纤维化肺疾病，可以与AE的疾病。进一步研究各种纤维化肺病AE的相似性和常见途径可能会对这种最近认识到的综合征产生更多的见解。
Background: it is now recognized that a significant portion of patients with idiopathic pulmonary fibrosis (IPF) can have sudden and rapid deteriorations in disease course that cannot be explained by infection, heart failure, or thromboembolic disease. These events are often fatal and have been termed acute exacerbations (AEs) of underlying disease. While best described in patients with IPF, they have also been reported in patients with other Forms of interstitial lung disease. We sought to determine if this same phenomenon occurs in patients with hypersensitivity pneumonitis (HP).Methods: We retrospectively reviewed our clinical experience at National Jewish Medical and Research Center For patients with surgical lung biopsy-proven fibrotic HP who had an acute decline in respiratory status and met criteria similar to those proposed for the diagnosis of an AE of IPF.Results: Over a 2-year period, we identified four patients with in AE of fibrotic HP. All patients had a clinical course similar to that most frequently described in AEs of IPF: respiratory failure requiring assisted ventilation, lack of clinical response to high-dose corticosteroid therapy, and a poor prognosis (all cases resulted in death or emergent lung transplantation). Lung biopsy at the time of the. AE, explant, or autopsy revealed organizing diffuse alveolar damage superimposed on fibrotic lung disease.Conclusions: Fibrotic HP, like other forms of fibrotic lung disease, can be associated with AEs of disease. Further investigation into similarities and pathways common in AEs of various fibrotic lung diseases may yield additional insight into this recently recognized syndrome.