3 November 2021
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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.
Acute complications
During the long-term progression of these diseases, acute exacerbation episodes can occur, characterised by a rapid deterioration of the disease from at least one month of onset, or a respiratory deterioration that leads to significant mortality rates for the patients.
It may be differentiated between acute exacerbation of the disease due to an identifiable cause, such as an infection, a heart failure, or a pulmonary thromboembolism, as well as an idiopathic exacerbation in which it has not been able to find a clear trigger.
Several studies have established possible risk factors for the development of acute exacerbations, like the maximum amount of air you can forcibly exhale from your lungs after fully inhaling (Forced Vital Capacity); the increased lung capacity (ILC); low lung diffusing capacity (DLCO); low distance covered in the walk test; deficient oxygenation; baseline level of dyspnoea increased, as well as having presented previous exacerbation episodes or have comorbidities such as pulmonary hypertension, emphysema, or coronary artery disease.
Chronic complications
Within the chronic complications that can appear in the course of fibrosing interstitial lung diseases are highlighted:
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.
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.
Sleep Apnoea. Patients with fibrosing DILDs have a high prevalence of Sleep Apnoea - Hypopnoea Syndrome (SAHS) and other respiratory disorders during sleep.
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%.
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Substantiated information by:
Fernanda Hernandez Gonzalez
Pulmonologist
Pneumology Department
Jacobo Sellarés Torres
Pneumologist
Pneumology and Respiratory Allergy Service
Joel Francesqui
Pulmonologist
Pneumology Department
Sandra Cuerpo Cardeñosa
Pulmonologist
Pneumology Department
Xavier Alsina Restoy
Advanced Nurse Practitioner
Pneumology Department
Published: 9 June 2020
Updated: 9 June 2020
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