The value of transbronchial lung biopsy in the diagnosis of lymphangioleiomyomatosis

Wenshuai Xu,Ruie Feng,Xinlun Tian,Han Cui,Hongrui Liu,Yanli Yang,Kai-Feng Xu

doi:10.1186/s12890-021-01518-2

Abstract

BackgroundTransbronchial lung biopsy (TBLB) in the diagnosis of lymphangioleiomyomatosis (LAM) is not a common approach, although TBLB is often performed in diffuse lung diseases. We aimed to examine the diagnostic value and safety of TBLB in LAM patients based on the data collected in our center.MethodsWe reviewed LAM patients registered in our LAM Clinic from December 8, 2006, to December 31, 2019. All patients with definite or probable diagnosis of LAM who had been examined using TBLB were included. All available pathology slides were reviewed by an experienced LAM pathologist. All complications were reviewed by the medical records and confirmed using telephone interviews.ResultsThe pathology results of 86 patients (including 74 definite LAM and 12 probable LAM) were available. The positive rate of TBLB in LAM patients was 49/86 (57.0%). The positive rates of SMA, HMB-45, ER, and PR in LAM patients were 97.6%, 93%, 84.6%, and 78.4% respectively. The positive rate of TBLB was 40%, 60% and 60.8% in patients with CT Grade I, Grade II, and Grade III respectively, and the difference was not significant. Patients who had 3–4 or 5–6 biopsied specimens had a higher rate of diagnosis than those with 1–2 biopsied specimens. Four patients (5.6%) reported pneumothorax. No major hemoptysis was reported.ConclusionsTBLB is a feasible and safe procedure for obtaining a pathological diagnosis of LAM. Taking more than 2 samples during the biopsy procedure increased the rate of diagnosis.

Highlights

Lymphangioleiomyomatosis (LAM) is a rare disease that predominantly affects females with clinical features of progressive cystic destruction of the lungs and the accumulation of LAM cells within the lungs and axial lymphatics [1]
Materials and methods We reviewed LAM patients registered in the LAM center in Peking Union Medical College Hospital (PUMCH) from December 8, 2006, to December 31, 2019, in our database to determine which patients had undergone Transbronchial lung biopsy (TBLB)
The following data were collected: [1] diagnosis-related information: history of chylous effusion, high resolution computed tomography (HRCT) of the chest, Computed tomography (CT) or magnetic resonance imaging (MRI) or ultrasound of abdomen, and serum VEGF-D; [2] number of samples biopsied during bronchoscopy; [3] pathological results including immunohistochemical examination; and [4] complications during bronchoscopy or within 24 h after the procedure

Summary

Introduction

Lymphangioleiomyomatosis (LAM) is a rare disease that predominantly affects females with clinical features of progressive cystic destruction of the lungs and the accumulation of LAM cells within the lungs and axial lymphatics [1]. Sporadic LAM affects 1 in 400,000 adult females, while it is common in adult females with tuberous sclerosis complex (TSC) [2]. The diagnostic criteria of definite LAM include characteristic or compatible. We aimed to report our experience using TBLB on patients at the LAM Clinic in Peking Union Medical College Hospital (PUMCH), Beijing, China. Transbronchial lung biopsy (TBLB) in the diagnosis of lymphangioleiomyomatosis (LAM) is not a com‐ mon approach, TBLB is often performed in diffuse lung diseases. We aimed to examine the diagnostic value and safety of TBLB in LAM patients based on the data collected in our center

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