Progression of fibrosing interstitial lung disease

Abstract

Fibrotic interstitial lung diseases (ILDs) are often challenging to diagnose and classify, but an accurate diagnosis has significant implications for both treatment and prognosis. A subset of patients with fibrotic ILD experience progressive deterioration in lung function, physical performance, and quality of life. Several risk factors for ILD progression have been reported, such as male sex, older age, lower baseline pulmonary function, and a radiological or pathological pattern of usual interstitial pneumonia. Morphological similarities, common underlying pathobiologic mechanisms, and the consistently progressive worsening of these patients support the concept of a progressive fibrosing (PF)-ILD phenotype that can be applied to a variety of ILD subtypes. The conventional approach has been to use antifibrotic medications in patients with idiopathic pulmonary fibrosis (IPF) and immunosuppressive medications in patients with other fibrotic ILD subtypes; however, recent clinical trials have suggested a favourable treatment response to antifibrotic therapy in a wider variety of fibrotic ILDs. This review summarizes the literature on the evaluation and management of patients with PF-ILD, and discusses questions relevant to applying recent clinicial trial findings to real-world practice.

Keywords: Disease classification; Interstitial lung disease; Outcomes; Progression; Pulmonary fibrosis; Pulmonary function tests.

Fig. 1

Computed tomography imaging of the chest in a patient with progressive unclassifiable interstitial lung disease. Serial apical (a, c, e) and basal (b, d, f) axial images at baseline (a, b); at 36 months (c, d); and at 42 months (e, f). Images show upper lobe predominant pulmonary fibrosis with progressive reticulation, traction bronchiectasis, and honeycombing