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Pulmonary fibrosis is not a single disease, but a group of diseases causing interstitial lung damage. This eventually results in lung elasticity loss and fibrosis. These conditions are chronic and usually affect patients between 50 and 70 years of age.
There are several different causes, such as exposure to certain substances, certain medications, tuberculosis, idiopathic pulmonary fibrosis, Hermansky Pudlak syndrome, sarcoidosis, radiation, connective tissue diseases and fibrosing alveolitisi.
Pulmonary fibrosis prognosis differs greatly from patient to patient, but overall the prognosis is poor. It is estimated that as many as 500,000 people in the United States have an interstitial lung disease, with 200,000 of these people having idiopathic pulmonary fibrosis. On average, 40,000 Americans die due to idiopathic pulmonary fibrosis every year.
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Factors Affecting Prognosis
For many patients suffering from this condition, their prognosis is poor. The disease is progressive and the symptoms continuously worsen. This reduces the patient's quality of life overall as well. A poor prognosis is associated with the following:
- Older age
- Severe dyspnoea
- Severe lung function loss
- Not responding to treatment and therapies
- Being male
- History of smoking cigarettes
- Severity and appearance of fibrosis on imaging or radiological studies
- On histopathological evaluation, prominent fibroblastic foci
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Cause of Death
Most patients with this condition eventually die as a result of respiratory failure. Other patients develop infections that are secondary to right-heart failure or steroid therapy and will not survive these infections.
Complications associated with this condition can also influence a patient's pulmonary fibrosis prognosis. Such complications may include:
Hypoxemia: Also referred to as low-blood oxygen levels, this complication may result due to this condition decreasing how much oxygen a patient can take in, therefore, the amount of oxygen in their bloodstream is reduced. This can interfere with basic body functions and be life-threatening when blood-oxygen levels are severely low.
Pulmonary hypertension: This complication is characterized by high blood pressure within the lungs. It is serious, progressively gets worse and can eventually become fatal.
Respiratory failure: Respiratory failure is often the last step in chronic lung diseases. When a patient's blood-oxygen levels become dangerously low, it occurs. When blood-oxygen levels are low, it can result in unconsciousness and heart arrhythmias.
Right-side heart failure: This occurs when the right ventricle in the heart has to work much harder in order to pump blood through pulmonary arteries that are blocked.
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On average, biopsy proven idiopathic pulmonary fibrosis has a median survival rate of three years. A five-year survival rate is experienced by 30 to 50 percent of patients. At this time, several studies are being done to work on new medications and treatment methods to improve quality of life and extend survival rate.
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MayoClinic.com. (2009). Pulmonary Fibrosis. Retrieved on October 10, 2010 from MayoClinic.com: http://www.mayoclinic.com/health/pulmonary-fibrosis/DS00927
Pulmonary Fibrosis Foundation. (2010). What is Pulmonary Fibrosis? Retrieved on October 10, 2010 from the Pulmonary Fibrosis Foundation: http://www.pulmonaryfibrosis.org/education
International Scleroderma Network. (2010). Overview of Pulmonary Fibrosis. Retrieved on October 10, 2010 from the International Scleroderma Network: http://sclero.org/medical/symptoms/pulmonary/fibrosis/a-to-z.html