Transbronchial lung biopsy for the diagnosis of lymphangioleiomyomatosis: the severity of cystic lung destruction assessed by the modified Goddard scoring system as a predictor for establishing the diagnosis

Abstract

Background A guide of patient selection for establishing the diagnosis of lymphangioleiomyomatosis (LAM) by transbronchial lung biopsy (TBLB) has not been established, although the pathological confirmation of LAM by lung biopsy is desirable, particularly when patients have no additional test results except typical findings of computed tomography (CT) of the chest. Methods We retrospectively reviewed the medical records of LAM patients who visited at our hospital from January 2010 to September 2018. We found 19 patients who underwent TBLB and collected the following data to investigate which parameters could predict the TBLB diagnostic positivity for LAM: age, degree of exertional dyspnea, pulmonary function test, cystic lung destruction visually assessed by the modified Goddard scoring system (MGS), serum level of vascular endothelial growth factor-D, and TBLB-related data. Results The diagnosis of LAM was established by TBLB in 15 of 19 patients (78.9%) and no serious complications occurred. MGS was significantly higher in the TBLB-positive group than the TBLB-negative group. In LAM patients without pulmonary lymphatic congestion on CT (N = 16), multivariable logistic regression analysis revealed that MGS and FEV1/FVC were independent contributing parameters for TBLB diagnostic positivity. However, the analysis of Bayesian inference demonstrated that MGS is a better predictor than FEV1/FVC; the probability of establishing diagnosis exceeds 80% if MGS is > 2 (i.e., area of cystic destruction occupies > 25% of lung parenchyma on CT). Conclusions MGS may be a helpful and convenient tool to select candidates for TBLB to establish the diagnosis of LAM pathologically.

The value of transbronchial lung biopsy in the diagnosis of lymphangioleiomyomatosis

“…Our results offered similar experience of the value of TBLB in LAM. The positive rate of TBLB in our results was similar to those previously reported [ 5 , 6 , 12 ]. In addition, the complications of TBLB were also similar to previous reports.…”

“…found that TBLB was positive in 6 of 10 patients and had a yield of approximately 60% in LAM, with 52/217 (14%) complications, including pneumothorax (6%), bleeding (4%), chest pain (2%) and pneumonia (2%) [ 11 ]. In recent years, some researchers reported that the positive rate of TBLB in patients with LAM was 70–78%, and there were no serious adverse events such as pneumothorax or uncontrollable bleeding due to TBLB [ 5 , 6 ]. Our results offered similar experience of the value of TBLB in LAM.…”

“…found the yield of TBLB was higher in patients with a reduced diffusing capacity [ 5 ]. Okamoto et al reported the severity of LAM on CT scans affected the positive rate of TBLB [ 6 ].In our study Grade I LAM patients had a lower positive rate of TBLB than Grade II/III LAM patients although this was not statistically significant. The lack of statistical significance may have resulted from the small sample sizes in each group.…”

“…Current guidelines for LAM recommend TBLB before surgical lung biopsy [ 3 ]. However, the experience of this application has seldom been reported since the publication of this recommendation [ 5 , 6 ]. We aimed to report our experience using TBLB on patients at the LAM Clinic in Peking Union Medical College Hospital (PUMCH), Beijing, China.…”

The Value of Transbronchial Lung Biopsy in the Diagnosis of Lymphangioleiomyomatosis

“…However, the experience of this application has seldom been reported since the publication of this recommendation (5,6). We aimed to report our experience using TBLB on patients at the LAM Clinic in Peking Union Medical College Hospital (PUMCH), Beijing, China.…”