page_title: Idiopathic pulmonary fibrosis
section_title: Prognosis
hierarchical_section_title: Idiopathic pulmonary fibrosis / Prognosis
caption_attribution_description: English: Comparison of the 5-year survival rate for Idiopathic Pulmonary Fibrosis (IPF) and common malignancies.
context_page_description: Idiopathic pulmonary fibrosis is a type of chronic scarring lung disease characterized by a progressive and irreversible decline in lung function. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other changes may include feeling tired, and abnormally large and dome shaped finger and toenails. Complications may include pulmonary hypertension, heart failure, pneumonia, or pulmonary embolism.
  The cause is unknown. Risk factors include cigarette smoking, certain viral infections, and a family history of the condition. The underlying mechanism involves scarring of the lungs. Diagnosis requires ruling out other potential causes. It may be supported by a CT scan or lung biopsy which show usual interstitial pneumonia. It is a type of interstitial lung disease.
  People often benefit from pulmonary rehabilitation and supplemental oxygen. Certain medications like pirfenidone or nintedanib may slow the progression of the disease. Lung transplantation may also be an option.
  About 5 million people are affected globally. The disease newly occurs in about 12 per 100,000 people per year. Those in their 60s and 70s are most commonly affected.
context_section_description: The clinical course of IPF can be unpredictable. IPF progression is associated with an estimated median survival time of 2 to 5 years following diagnosis.
  The 5-year survival for IPF 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 IPF. 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 IPF. 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 IPF 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 IPF 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 IPF treatments rather than survival.
  In addition to clinical and physiological parameters to predict how rapidly patients with IPF might progress, genetic and molecular features are also associated with IPF mortality. For example, it has been shown that IPF 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.
caption: Comparison of the 5-year survival rate for IPF and common malignancies. Adapted from Bjoraker et al. 1998.[57]
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