ABSTRACT
Idiopathic pulmonary fibrosis (IPF) is possibly the most rapidly progressive, chronic fibrosing condition in any organ. It is associated with a median survival of 3 years from diagnosis. The overall incidence of diagnosed IPF is increasing, in part as awareness of the diagnosis increases. Indeed, recent epidemiologic findings from the Respiratory Health of the Nation study in the United Kingdom indicate that IPF is twice as common as previously believed, with around 0.5 cases diagnosed per 1,000 of the UK population (1). Moreover, although it is considered a rare disease, IPF is strongly associated with ageing. In patients ≥70 years in age, its incidence is estimated between 1 and 4 per 1,000, and it is responsible for 1% of deaths in the United Kingdom (1–3). Clinical need remains under-served in terms of its diagnosis and effective treatment. Nevertheless, the direction of travel over recent years in both these aspects is encouraging. The evolution of guidelines and algorithms for multidisciplinary diagnosis of IPF (4) has been associated with increased consistency between specialist centres internationally (5). This appears to be accompanied by good identification of cases in which typically ‘IPF-like’ disease behaviour – progressive decline despite therapeutic intervention – is predicted, allowing proactive management planning. Within this ‘typical’ picture of disease progression, heterogeneity is increasingly recognized at both clinical and molecular levels, allowing the possibility of more personalized care in the future (6).
