Diagnosis and Treatment of Fibrotic Hypersensitivity Pneumonia Where We Stand and Where We Need to Go
Diagnosis and Treatment of Fibrotic Hypersensitivity Pneumonia Where We Stand and Where We Need to Go
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DOI：
10.1164/rccm.201608-1675pp
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发表时间：
2017-09-15
影响因子：
24.7
通讯作者：
Flaherty, Kevin R.
中科院分区：
文献类型：
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作者：
Salisbury, Margaret L.;Myers, Jeffrey L.;Flaherty, Kevin R.
过敏性肺炎(HP)是一种间质性肺部疾病，由吸入抗原致敏引起。虽然已报道的激发抗原很多，但大多数被归类为禽类、微生物(环境真菌或细菌)或自然界中的化学物质(通常是包括异氰酸酯在内的有机化合物)，但经过彻底评估后，可能仍未确定。真正的影响是未知的，而且可能因当地气候和工业的不同而不同；农业似乎与暴露风险增加和不成比例的死亡负担有关(1)。在英国，幽门螺杆菌的发病率为每10万人年0.9例，美国的年龄调整死亡率为0.19‰(1，2)，而特发性肺纤维化(IPF)的发病率约为每10万人年4.6-16.3例(3)。怀疑惠普被低估了(4-7)。Morell和他的同事强调了IPF和纤维性HP之间的诊断重叠，描述了相当大比例的最初被诊断为IPF的患者后来被诊断为Hp(5)。区分纤维性HP和IPF是很重要的，因为IPF有特定的治疗方法，而纤维性HP的最佳治疗方法并不明确(8，9)。广泛的症状表现、高分辨率计算机断层扫描(HRCT)和组织病理学结果，以及缺乏有效的诊断标准，使诊断变得困难，并阻碍了研究治疗方式的进展。这篇肺学透视回顾了幽门螺杆菌的表现和表型，以及诊断和治疗策略，重点是纤维性幽门螺杆菌。
Hypersensitivity pneumonia (HP) is an interstitial lung disease (ILD) caused by sensitization to an inhaled antigen. Although the reported inciting antigens are numerous, most are classified as either avian, microbial (environmental fungi or bacteria), or chemical (usually organic compounds including isocyanates) in nature, but may remain unidentified after thorough evaluation. The true impact is unknown and likely varies geographically due to local climate and industry; agriculture appears to be associated with increased risk of exposure and disproportionate mortality burden (1). The incidence of HP is 0.9 cases per 100,000 person-years in the United Kingdom, with an age-adjusted death rate 0.19 per million overall in the United States (1, 2), compared with the idiopathic pulmonary fibrosis (IPF) incidence of approximately 4.6–16.3 per 100,000 person-years (3). HP is suspected to be underrecognized (4–7). Morell and colleagues highlighted the diagnostic overlap between IPF and fibrotic HP, describing a sizeable proportion of patients initially diagnosed with IPF later receiving a diagnosis of HP (5). Distinguishing fibrotic HP from IPF is important, as specific therapy is available for IPF whereas optimal treatment for fibrotic HP is poorly defined (8, 9). The broad range of presenting symptoms, high-resolution computed tomography (HRCT) and histopathologic findings, and lack of validated diagnostic criteria make diagnosis difficult and have hindered the progress of research investigating treatment modalities. This Pulmonary Perspective reviews HP manifestations and phenotypes along with diagnostic and treatment strategies, focusing on fibrotic HP.