Progressive pulmonary fibrosis: all roads lead to Rome (but not all at the same speed)

Extract

Fibrosing interstitial lung diseases (ILDs) encompass a number of diverse conditions, overlapping in their clinical presentations, and imaging and histopathological patterns [1]. Idiopathic pulmonary fibrosis (IPF) is the prototype of fibrosing ILD, and is characterised by irreversible progressive pulmonary disease, accounting for loss of lung function, exercise intolerance and complications, especially acute exacerbation and respiratory failure leading to early mortality [2]. A significant proportion of patients with fibrosing ILDs other than IPF will develop a progressive phenotype comparable to untreated IPF [3]. Such progression can occur despite conventional treatment which, depending on the underlying condition, may include close monitoring, antigen eviction, glucocorticoids, immunosuppressive therapy and pulmonary rehabilitation. Progressive pulmonary fibrosis (PPF) [1, 4], also referred to as “progressive fibrosing ILDs” (PF-ILD) or fibrosing ILDs with a progressive phenotype [5], is characterised by a disease course similar to that of IPF, with worsening respiratory symptoms, decline in lung function and early mortality [6, 7].