Biopsy in interstitial lung disease: specific diagnosis and the identification of the progressive fibrotic phenotype.

Abstract

The evaluation of progression in fibrotic interstitial lung diseases (ILDs) may require a multidimensional approach. This review will cover the role and usefulness of lung biopsy in diagnosis and assessment of the progressive fibrotic phenotype. The identification of specific findings and the balance between inflammation and fibrosis on lung biopsy may help distinguishing different disease entities and may likely determine the effect of treatment and possibly prognosis. The fibrotic morphological patterns potentially associated with a progressive phenotype include usual interstitial pneumonia (UIP), fibrotic nonspecific interstitial pneumonia, pleuroparenchymal fibroelastosis, desquamative interstitial pneumonia, fibrotic hypersensitivity pneumonitis and other less common fibrotic variants, with histopathological findings of UIP at the time of diagnosis being predictive of worse outcome compared with other patterns. The prognostic significance of lung biopsy findings has been assessed after both surgical lung biopsy (SLB) and transbronchial lung cryobiopsy (TBLC), the latter becoming a valid alternative to SLB, if performed in experienced centres, due to significantly lower morbidity and mortality. Lung biopsy plays an important role in diagnosis and identification of the progressive fibrotic phenotype. The introduction of less invasive procedures could potentially expand the role of lung sampling, including for example patients with a known diagnosis of ILD or at an earlier stage of the disease.