Prognostic assessment in IPF

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal form of fibrotic interstitial lung disease (ILDs). The clinical profile and natural history are extremely heterogeneous, some patients progress rapidly, while others slowly. A proportion of patients experience acute exacerbations of IPF, an accelerated lung function decline of unknown cause that to date is still unpredictable. Moreover the natural course of IPF is influenced by different comorbidities. The intrinsic complexity of this entity makes very challenging to find reliable and standardized methods of prognostic assessment. Multidimensional indices and staging systems have been recently proposed. The advantage of multidimensional indices is that they are more accurate in predicting IPF prognosis compared to single prognostic clinical factors. Currently both functional variables and multidimensional indices are used in clinical practice to discuss prognosis with patients and to guide both treatment and monitoring decisions. Despite these recent progresses we are still unable to predict the rate of disease progression, the risk of acute exacerbations and the most frequent and lethal comorbidities, such as pulmonary hypertension and lung cancer, that may swerve disease course. Future research in the field of genotyping and biomolecular profiling of this heterogeneous entity, will hopefully allow important steps on the complex pathway of IPF prognostic assessment.