Comparison of the 5-year survival rate for<unk>  and common malignancies. Adapted from Bjoraker et al. 1998. The clinical course of<unk>  can be unpredictable.<unk>  progression is associated with an estimated median survival time of 2 to 5 years following diagnosis. The 5-year survival for<unk>  ranges between 20–40%, a mortality rate higher than that of a number of malignancies, including colon cancer, multiple myeloma and bladder cancer. Recently a multidimensional index and staging system has been proposed to predict mortality in<unk> . The name of the index is GAP and is based on gender G, age A, and two lung physiology variables P (FVC and DLCO that are commonly measured in clinical practice to predict mortality in<unk> . The highest stage of GAP (stage III) has been found to be associated with a 39% risk of mortality at 1 year. This model has also been evaluated in<unk>  and other ILDs and shown good performance in predicting mortality in all main ILD subtypes. A modified ILD-GAP Index has been developed for application across ILD subtypes to provide disease-specific survival estimates. In<unk>  patients, the overall mortality at 5 years rate is high but the annual rate of all-cause mortality in patients with mild to moderate lung impairment is relatively low. This is the reason why change in lung function (FVC) is usually measured in 1-year clinical trials of<unk>  treatments rather than survival. In addition to clinical and physiological parameters to predict how rapidly patients with<unk>  might progress, genetic and molecular features are also associated with<unk>  mortality. For example, it has been shown that<unk>  patients who have a specific genotype in the mucin MUC5B gene polymorphism (see above) experience slower decline in FVC and significantly improved survival. Even if such data are interesting from a scientific point of view, the application in the clinical routine of a prognostic model based on specific genotypes is still not possible.
Idiopathic pulmonary fibrosis
Sarcoidosis
Mineral dust airway disease
Hypersensitivity pneumonitis
Allergic bronchopulmonary aspergillosis
Interstitial lung disease
Rheumatoid lung disease
Idiopathic interstitial pneumonia