Prognosis of Pulmonary Fibrosis

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The natural history of fibrosing interstitial lung diseases is variable. The worst prognosis is given when the idiopathic pulmonary fibrosis progresses towards respiratory failure in an average of approximately four years after the initial diagnosis. This progression of the disease has a mean survival that varies between two and five years. In the case of fibrosing lung disease, the prognosis may be more favourable.  

In any case, there is a wide variability in the course of the disease in each patient that will depend on factors like the appearance of acute exacerbations in the progression of the disease, or the presence of other diseases.  

Chronic complications

Within the chronic complications that can appear in the course of fibrosing interstitial lung diseases are highlighted:   

Lung and heart

Pulmonary hypertension. This is defined as a mean pulmonary arterial pressure greater than 25 mmHg, developed as a result of a maintained chronic hypoxaemia (abnormal decrease of the partial pressure of oxygen in arterial blood to below 60 mmHg). Its prevalence is estimated at 30-45% in the patients assessed for lung transplant, but is much higher in those with a longer time of onset of the disease. The development of pulmonary hypertension causes a deterioration in the quality of life of the patients, higher exertion dyspnoea, reduction in the diffusing capacity for carbon monoxide (DLCO) in respiratory function tests, and less exercise capacity, which is demonstrated by a lower distance covered, and a lower desaturation (when the arterial oxygen saturation decreases by 4% or more) in the walking test. Furthermore, it is also associated with a lower survival with a mortality at one year of 28% in patients with pulmonary hypertension compared to 5.5% in those that do not have it. 

Standing person with abdominal pain

Gastro-oesophageal reflux disease. It is a disease in which the stomach contents return from the stomach towards the oesophagus. This can irritate the swallowing tube and cause gastric acidity, among other symptoms. Several studies have documented a high prevalence (66-87%) of gastro-oesophageal reflux in patients with pulmonary fibrosis, which is asymptomatic in the majority of them. The existence of gastro-oesophageal reflux and hiatus hernia, also more common in patients with pulmonary fibrosis, may be a factor in the disease and may represent its progression in association with the presence of pulmonary micro-aspirations.  

Person sleeping with a blocked nose or apnoea

Sleep Apnoea. Patients with fibrosing DILDs have a high prevalence of Sleep Apnoea - Hypopnoea Syndrome (SAHS) and other respiratory disorders during sleep.  

Heart with a blocked artery causing a heart attack

Other complications. There is also a higher risk of developing lung cancer in patients with pulmonary fibrosis. The prevalence is from 5-10% and increases with the development of the disease in time and in patients with associated emphysema. There is also a higher risk of cardiovascular diseases (coronary disease and venous thromboembolic disease). Pneumothorax (collapsed lung) can also be a cause for the worsening of the pulmonary fibrosis, with an approximate incidence of 11%. 

Substantiated information by:

Fernanda Hernandez Gonzalez
Jacobo Sellarés Torres
Joel Francesqui
Sandra Cuerpo Cardeñosa
Xavier Alsina Restoy

Published: 9 June 2020
Updated: 9 June 2020

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