背景:这项研究的主要目的是评估慢性过敏性肺炎(HP)患者的生存率和肺功能变化,纤维化(f-HP)和非纤维化(nf-HP),并与特发性肺纤维化(IPF)患者进行比较。
方法:根据抗原暴露诊断HP,HRCT(高分辨率CT扫描),BAL(支气管肺泡灌洗),和组织学。根据HRCT,HP分为纤维化和非纤维化表型。在大多数情况下,根据HRCT结果诊断IPF。
结果:我们确定了84例患者:46例IPF,18与f-HP,和20与NF-HP。IPF的5年生存率为23.9%,72%的f-HP,和100%的nf-HP(p<0.0001)。蜂巢与IPF(p<0.001)和f-HP(p<0.0001)的生存率降低有关。平均FVC(强迫肺活量)损失%pred。IPF(预测百分比)为-18.3%(p=0.001),-4.8%的f-HP,nf-HP为-6.0%。DLCO(一氧化碳扩散能力)%pred的平均变化。IPF中为-10.2%(p<0.002),-0.5%的f-HP,nf-HP+1.9%。HP的放射学表型和组织学之间的一致性为89.6%。
结论:我们发现IPF的生存期较短,其次是f-HP,nf-hp随着时间的推移,我们没有发现FVC%pred的显著变化.或DLCO%pred。在HP中,同时注意到IPF的显着下降。在HP中,我们发现放射学表型和组织学之间有很强的一致性。提示HP肺纤维化的放射学征象对于f-HP的诊断是可靠的,并且似乎具有内在的预后价值。
BACKGROUND: The main objective of this study was to estimate survival and changes in lung function in patients with chronic hypersensitivity pneumonitis (HP), both fibrotic (f-HP) and nonfibrotic (nf-HP), and to compare them with those in patients with idiopathic pulmonary fibrosis (IPF).
METHODS: HP was diagnosed based on antigen exposure, HRCT (high-resolution CT scan), BAL (bronchoalveolar lavage), and histology. According to HRCT, HP was classified into fibrotic and non-fibrotic phenotypes. In most cases, IPF was diagnosed based on HRCT findings.
RESULTS: We identified 84 patients: 46 with IPF, 18 with f-HP, and 20 with nf-HP. Five-year survival was 23.9% in IPF, 72% in f-HP, and 100% in nf-HP (p <0.0001). Honeycombing was associated with decreased survival in IPF (p <0.001) and in f-HP (p <0.0001). The mean loss of FVC (forced vital capacity) % pred. (percent predicted) was -18.3% in IPF (p =0.001), -4.8% in f-HP, and -6.0% in nf-HP. The mean change in DLCO (diffusion capacity for carbon monoxide) % pred. was -10.2% in IPF (p <0.002), -0.5% in f-HP, and +1.9% in nf-HP. The agreement between radiological phenotypes and histology in HP was 89.6%.
CONCLUSIONS: We found shorter survival in IPF, followed by f-HP, and nf-HP. Over time, we did not find significant changes in FVC% pred. or DLCO% pred. in HP, while a significant decline in IPF was noted. In HP, we found strong agreement between radiological phenotypes and histology. Radiological signs suggestive of lung fibrosis in HP were reliable for the diagnosis of f-HP and seem to have intrinsic prognostic value.