Gazing into the Proteomic Crystal Ball: Predicting Survival in Idiopathic Pulmonary Fibrosis

摘要

In many respects, idiopathic pulmonary fibrosis (IPF) is a predictable disease. It is a condition characterized by inevitable and usually inexorable progression with an untreated median survival of between 3 and 5?years (1). However, a look beyond population-level statistics and examination of the disease behavior of individual patients reveals considerable heterogeneity. Some individuals have rapidly progressive disease and die within months of diagnosis, whereas others have relatively indolent disease, with a small proportion living with IPF for 10?years or longer (2). Furthermore, this heterogeneity of disease behavior has almost certainly been amplified by the availability of antifibrotic therapy (3). Some of the interindividual differences in disease behavior and clinical outcomes can be ascribed to demographic and physiological factors. Men and older individuals with IPF have worse survival, as do those with greater physiological impairment. These observations have been encapsulated in the GAP (Gender-Age-Physiology Index) score, which provides a reasonable prediction of prognosis for individuals with IPF (4). Prognosis prediction, such as that provided by the GAP score, may be helpful in clinical practice (e.g., timing of transplant referral) and may, in the future, help guide the initiation and management of pharmacologic therapy in IPF.