Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis
Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis
复制标题
DOI：
10.1016/j.rmed.2018.03.031
复制
发表时间：
2018-05-01
影响因子：
4.3
通讯作者：
Wells, Athol U.
中科院分区：
文献类型：
--
作者：
Jacob, Joseph;Odink, Arlette;Wells, Athol U.
背景：胸膜实质弹力纤维增生症（PPFE）已在过敏性肺炎（HP）中被描述，但其功能意义尚不清楚。偶尔在从不吸烟的 HP 患者中发现合并肺纤维化和肺气肿 (CPFE)，但有关其患病率的流行病学数据很少。因此，对大型 HP 队列中的 CT 进行了检查，以确定 PPFE 和肺气肿的患病率和影响。方法：233 名 HP 患者的间质性肺疾病 (ILD) 和肺气肿的 CT 范围量化至最接近的 5%。 PPFE 的肺叶胸膜受累百分比按 4 点分类量表进行量化：0 = 不存在，1 = 影响 < 10%，2 = 影响 10-33%，3 = 影响 > 33%。标记的 PPFE 反映肺部总评分 >= 3/18。根据 FVC、DLco 和死亡率对结果进行评估。结果：显着 PPFE 患病率为 23%，而从不吸烟者中有 23% 患有肺气肿。对患者年龄、性别、吸烟状况以及 ILD 和肺气肿程度进行调整后，显着的 PPFE 与基线 FVC (p = 0.0002) 和 DLco (p = 0.002) 降低独立相关，并且与相同协变量一起检查时，与生存恶化独立相关 (p = 0.01)。 HP 中的 CPFE 表现出人工肺容量保留和不成比例的 DLco 减少的特征性功能特征。与无肺气肿的 HP 患者相比，除了 ILD 和肺气肿的 CT 范围所解释的情况外，CPFE 并未表现出更差的结果。结论：PPFE 在 HP 中并不罕见，并且与肺功能受损和死亡率增加独立相关。 23% 的 HP 从不吸烟者发现患有肺气肿。 CPFE 似乎与恶性微血管表型无关，因为结果是通过 ILD 和肺气肿程度来解释的。
BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) has been described in hypersensitivity pneumonitis (HP) yet its functional implications are unclear. Combined pulmonary fibrosis and emphysema (CPFE) has occasionally been described in never-smokers with HP, but epidemiological data regarding its prevalence is sparse. CTs in a large HP cohort were therefore examined to identify the prevalence and effects of PPFE and emphysema.Methods: 233 HP patients had CT extents of interstitial lung disease (ILD) and emphysema quantified to the nearest 5%. Lobar percentage pleural involvement of PPFE was quantified on a 4-point categorical scale: 0 = absent, 1 = affecting < 10%, 2 = affecting 10-33%, 3 = affecting > 33%. Marked PPFE reflected a total lung score of >= 3/18. Results were evaluated against FVC, DLco and mortality.RESULTS: Marked PPFE prevalence was 23% whilst 23% of never-smokers had emphysema. Following adjustment for patient age, gender, smoking status, and ILD and emphysema extents, marked PPFE independently linked to reduced baseline FVC (p = 0.0002) and DLco (p = 0.002) and when examined alongside the same covariates, independently linked to worsened survival (p = 0.01).CPFE in HP demonstrated a characteristic functional profile of artificial lung volume preservation and disproportionate DLco reduction. CPFE did not demonstrate a worsened outcome when compared to HP patients without emphysema beyond that explained by CT extents of ILD and emphysema.CONCLUSIONS: PPFE is not uncommon in HP, and is independently associated with impaired lung function and increased mortality. Emphysema was identified in 23% of HP never-smokers. CPFE appears not to link to a malignant microvascular phenotype as outcome is explained by ILD and emphysema extents.