Pulmonary fibrosis (PF) is a serious lung disease characterized by lung inflammation and fibrosis, or scarring. Scar tissue makes it harder for lungs to expand and also impairs their ability to take in oxygen. In its most common form, called idiopathic pulmonary fibrosis (IPF), the underlying cause of fibrosis is unclear.
All forms of PF are progressive, meaning that symptoms start off mild and progressively worsen over time. The most common cause of death is respiratory failure, which happens when the lungs are unable to supply the body with enough oxygen.
Factors affecting prognosis
A number of factors can influence the prognosis for someone with PF.
PF cannot be cured. However, anti-fibrotic treatments that slow the development of lung scarring can significantly slow disease progression. As such, proper treatment can improve the condition’s prognosis.
For treatment and management to begin, a person must have the correct diagnosis. As such, an early and accurate diagnosis is generally tied to better long-term outcomes.
Lung function can be evaluated with a number of tests, such as spirometry. Poorer lung function, and a more rapid lung function decline over time, are indicative of a worse prognosis. Worsening dyspnea, or shortness of breath, is also indicative of worse lung function and associated with a poorer prognosis.
Certain imaging tests may also be used to detect and evaluate the degree of lung scarring. More pronounced scarring generally is tied to a worse prognosis. A specific pattern of lung damage called usual interstitial pneumonia (UIP), which is characteristic of IPF, has also been associated with a particularly poor prognosis.
Smoking is one of the main risk factors for PF and is also a major prognostic factor: current or former smokers generally have a worse prognosis than people who have never smoked.
Age, sex, and body mass index
Older people with PF generally have a worse prognosis than their younger peers.
PF is more commonly diagnosed in males than in females. Some studies have suggested that females are also less likely to die from the condition, though other studies have found no difference in survival based on sex.
Comorbidities, or other co-occurring health problems, can negatively influence PF prognosis. For example, many patients with PF also develop pulmonary hypertension — a condition characterized by abnormally high blood pressure in the blood vessels connecting the heart to the lungs. That is tied to a worse prognosis.
Last updated: Dec. 30, 2021, by Marisa Wexler MS
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