Transbronchial lung cryobiopsy in the diagnosis of fibrotic interstitial lung diseases

PLoS One. 2014 Feb 28;9(2):e86716. doi: 10.1371/journal.pone.0086716. eCollection 2014.

Abstract

Background: Histology is a key element for the multidisciplinary diagnosis of fibrotic diffuse parenchymal lung diseases (f-DPLD) when the clinical-radiological picture is nondiagnostic. Transbronchial lung cryobiopsy (TBLC) have been shown to be useful for obtaining large and well-preserved biopsies of lung parenchyma, but experience with TBLC in f-DPLD is limited.

Objectives: To evaluate safety, feasibility and diagnostic yield of TBLC in f-DPLD.

Method: Prospective study of 69 cases of TBLC using flexible cryoprobe in the clinical-radiological setting of f-DPLD with nondiagnostic high resolution computed tomography (HRCT) features.

Safety: pneumothorax occurred in 19 patients (28%). One patient (1.4%) died of acute exacerbation. Feasibility: adequate cryobiopsies were obtained in 68 cases (99%). The median size of cryobiopsies was 43.11 mm(2) (range, 11.94-76.25). Diagnostic yield: among adequate TBLC the pathologists were confident ("high confidence") that histopathologic criteria sufficient to define a specific pattern in 52 patients (76%), including 36 of 47 with UIP (77%) and 9 nonspecific interstitial pneumonia (6 fibrosing and 3 cellular), 2 desquamative interstitial pneumonia/respiratory bronchiolitis-interstitial lung disease, 1 organizing pneumonia, 1 eosinophilic pneumonia, 1 diffuse alveolar damage, 1 hypersensitivity pneumonitis and 1 follicular bronchiolitis. In 11 diagnoses of UIP the pathologists were less confident ("low confidence"). Agreement between pathologists in the detection of UIP was very good with a Kappa coefficient of 0.83 (95% CI, 0.69-0.97). Using the current consensus guidelines for clinical-radiologic-pathologic correlation 32% (20/63) of cases were classified as Idiopathic Pulmonary Fibrosis (IPF), 30% (19/63) as possible IPF, 25% (16/63) as other f-DPLDs and 13% (8/63) were unclassifiable.

Conclusions: TBLC in the diagnosis of f-DPLD appears safe and feasible. TBLC has a good diagnostic yield in the clinical-radiological setting of f-DPLD without diagnostic HRCT features of usual interstitial pneumonia. Future studies should consider TBLC as a potential alternative to SLBx in f-DPLD.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Biopsy / methods*
  • Genetic Diseases, Inborn / diagnosis*
  • Humans
  • Lung / pathology*
  • Lung Diseases, Interstitial / diagnosis*
  • Prospective Studies
  • Risk Factors
  • Sensitivity and Specificity

Supplementary concepts

  • Interstitial Pneumonitis, Desquamative, Familial

Grants and funding

This study is supported by Associazione Morgagni Malattie Polmonari. The sponsor covers only the publication's costs. The entire study was realized without any financial support. In particular the funder had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.