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Abstract：
BACKGROUND: Although transbronchial lung cryobiopsy (TBLC) is widely used in diagnostic algorithms for various interstitial lung diseases (ILDs), its real-world utility in the therapeutic decision-making strategy for ILD patients remains unclear, in particular, when judging the time to start antifibrotic agents.
METHODS: We analyzed medical records of 40 consecutive patients with idiopathic or fibrotic hypersensitivity pneumonitis who underwent TBLC. A TBLC-based usual interstitial pneumonia (UIP) score was used to assess three morphologic descriptors: patchy fibrosis, fibroblastic foci, and honeycombing.
RESULTS: In our 40 patients with ILD, the most frequent radiological feature was indeterminate for UIP (45.0%). Final diagnosis included idiopathic pulmonary fibrosis (22.5%), fibrotic nonspecific interstitial pneumonia (5.0%), fibrotic hypersensitivity pneumonitis (35.0%), and unclassifiable ILD (37.5%). Linear mixed-effects analysis showed that declines in the slopes of %FVC and %DLCO in patients with TBLC-based UIP \"Score ≥ 2\" were significantly steeper than those of patients with \"Score ≤ 1.\" During follow-up of patients with Score ≥ 2 (n = 24), more than half of them (n = 17) received an antifibrotic agent, with most patients (n = 13) receiving early administration of the antifibrotic agent within 6 months after the TBLC procedure.
CONCLUSIONS: TBLC-based UIP Score ≥ 2 indicated the increased possibility of a progressive fibrosis course that may prove helpful in predicting progressive pulmonary fibrosis/progressive fibrosing ILD even if disease is temporarily stabilized due to anti-inflammatory agents. Patients may benefit from early introduction of antifibrotic agents by treating clinicians.
METHODS: We analyzed medical records of 40 consecutive patients with idiopathic or fibrotic hypersensitivity pneumonitis who underwent TBLC. A TBLC-based usual interstitial pneumonia (UIP) score was used to assess three morphologic descriptors: patchy fibrosis, fibroblastic foci, and honeycombing.
RESULTS: In our 40 patients with ILD, the most frequent radiological feature was indeterminate for UIP (45.0%). Final diagnosis included idiopathic pulmonary fibrosis (22.5%), fibrotic nonspecific interstitial pneumonia (5.0%), fibrotic hypersensitivity pneumonitis (35.0%), and unclassifiable ILD (37.5%). Linear mixed-effects analysis showed that declines in the slopes of %FVC and %DLCO in patients with TBLC-based UIP \"Score ≥ 2\" were significantly steeper than those of patients with \"Score ≤ 1.\" During follow-up of patients with Score ≥ 2 (n = 24), more than half of them (n = 17) received an antifibrotic agent, with most patients (n = 13) receiving early administration of the antifibrotic agent within 6 months after the TBLC procedure.
CONCLUSIONS: TBLC-based UIP Score ≥ 2 indicated the increased possibility of a progressive fibrosis course that may prove helpful in predicting progressive pulmonary fibrosis/progressive fibrosing ILD even if disease is temporarily stabilized due to anti-inflammatory agents. Patients may benefit from early introduction of antifibrotic agents by treating clinicians.
摘要：
背景：尽管经支气管肺冷冻活检（TBLC）广泛用于各种间质性肺病（ILD）的诊断算法中，其在ILD患者治疗决策策略中的实际效用仍不清楚，特别是,当判断开始抗纤维化药物的时间。
方法：我们分析了40例接受TBLC的特发性或纤维化过敏性肺炎患者的医疗记录。基于TBLC的常规间质性肺炎（UIP）评分用于评估三个形态学描述：斑片状纤维化，成纤维细胞灶,蜂蜜梳理。
结果：在我们的40例ILD患者中，UIP最常见的放射学特征是不确定的(45.0%)。最终诊断为特发性肺纤维化（22.5%），纤维化非特异性间质性肺炎（5.0%），纤维化过敏性肺炎（35.0%），和不可分类的ILD（37.5％）。线性混合效应分析表明，基于TBLC的UIP“评分≥2”的患者的％FVC和％DLCO斜率的下降明显高于“评分≤1”的患者。“在评分≥2（n=24）的患者的随访期间，其中一半以上（n=17）接受了抗纤维化药物，大多数患者（n=13）在TBLC手术后6个月内接受了抗纤维化药物的早期给药。
结论：基于TBLC的UIP评分≥2表明进行性纤维化病程的可能性增加，这可能有助于预测进行性肺纤维化/进行性纤维化ILD，即使疾病由于抗炎药暂时稳定。通过治疗临床医生，患者可能会从早期引入抗纤维化药物中受益。
方法：我们分析了40例接受TBLC的特发性或纤维化过敏性肺炎患者的医疗记录。基于TBLC的常规间质性肺炎（UIP）评分用于评估三个形态学描述：斑片状纤维化，成纤维细胞灶,蜂蜜梳理。
结果：在我们的40例ILD患者中，UIP最常见的放射学特征是不确定的(45.0%)。最终诊断为特发性肺纤维化（22.5%），纤维化非特异性间质性肺炎（5.0%），纤维化过敏性肺炎（35.0%），和不可分类的ILD（37.5％）。线性混合效应分析表明，基于TBLC的UIP“评分≥2”的患者的％FVC和％DLCO斜率的下降明显高于“评分≤1”的患者。“在评分≥2（n=24）的患者的随访期间，其中一半以上（n=17）接受了抗纤维化药物，大多数患者（n=13）在TBLC手术后6个月内接受了抗纤维化药物的早期给药。
结论：基于TBLC的UIP评分≥2表明进行性纤维化病程的可能性增加，这可能有助于预测进行性肺纤维化/进行性纤维化ILD，即使疾病由于抗炎药暂时稳定。通过治疗临床医生，患者可能会从早期引入抗纤维化药物中受益。
