Efficacy and safety of transbronchial lung biopsy for the diagnosis of lymphangioleiomyomatosis: A report of 24 consecutive patients
Efficacy and safety of transbronchial lung biopsy for the diagnosis of lymphangioleiomyomatosis: A report of 24 consecutive patients
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DOI：
10.1111/resp.13190
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发表时间：
2018-03-01
期刊：
影响因子：
6.9
通讯作者：
Inoue, Yoshikazu
中科院分区：
文献类型：
--
作者：
Koba, Taro;Arai, Toru;Inoue, Yoshikazu
背景与目的淋巴管肌瘤病是一种常见于育龄妇女的弥漫性囊性肺病。LAM可以在具有典型的高分辨率计算机断层扫描(HRCT)模式和至少一种其他确证疾病特征的患者的临床基础上被诊断，例如乳糜胸、血管肌脂肪瘤、结节性硬化症或血清血管内皮生长因子(VEGF)-D升高。然而，不符合这些标准的患者需要组织确认才能做出明确的诊断，而比外科肺活检侵入性更小的方法，如经纤维支气管镜肺活检(TB-B)，其实用性还没有得到很好的研究。方法1991年1月至2015年8月，前瞻性登记了131例LAM患者，其中24例进行了TBLB检查。结果24例患者均为女性，中位年龄42岁。HRCT显示肺内弥漫分布多个圆形薄壁囊肿。血清VEGF-D中位数为2109pg/mL。两位专家病理学家发现17例(70.8%)LAM的特征性病理表现。17例结核分枝杆菌阳性的LAM患者一氧化碳弥散能力的预测百分率显著低于7例结核分枝杆菌阴性的LAM患者(P=0.046)。结论经纤维支气管镜活检对淋巴管肌瘤病的诊断准确率为70.8%，是一种安全、有效的诊断方法。在弥散能力降低的患者中，TBLB的产量更高，这表明这种方法需要足够的疾病负担。该手术似乎是安全的，因为没有观察到严重的不良事件，如气胸或出血过多。
Background and objectiveLymphangioleiomyomatosis (LAM) is a diffuse cystic lung disease that occurs in women of childbearing age. LAM can be diagnosed on a clinical basis in patients with typical high-resolution computed tomography (HRCT) patterns and at least one other corroborating disease feature, such as chylothorax, angiomyolipoma, tuberous sclerosis complex or elevated serum vascular endothelial growth factor (VEGF)-D. However, patients who do not meet these criteria require tissue confirmation for a definitive diagnosis, and the utility of methods that are less invasive than surgical lung biopsy, such as transbronchial lung biopsy (TBLB), are not well studied. We retrospectively studied the efficacy and safety of TBLB for the diagnosis of LAM.MethodsFrom January 1991 to August 2015, 131 consecutive LAM patients were prospectively registered in our study, and a TBLB was conducted for 24 patients. We retrospectively studied the yield and safety of TBLB in this cohort.ResultsAll 24 patients were women; the median age was 42years. HRCT showed multiple round thin-walled cysts diffusely scattered throughout the lungs. The median level of serum VEGF-D was 2109pg/mL. Characteristic pathological findings for LAM were identified in 17 patients (70.8%) by two expert pathologists. The %predicted value for diffusing capacity of carbon monoxide was significantly lower in the 17 TBLB-positive LAM patients compared to the seven TBLB-negative LAM patients (P=0.046). There were no serious adverse events such as pneumothorax or uncontrollable bleeding due to TBLB.ConclusionTBLB is a safe and effective method for the pathological diagnosis of LAM.The diagnosis yield for lymphangioleiomyomatosis by transbronchial lung biopsy (TBLB) was 70.8%. The yield of TBLB was higher in patients with a reduced diffusing capacity, suggesting that an adequate disease burden is required for this approach. The procedure seems safe, as no serious adverse events such as pneumothorax or excessive bleeding were observed.