Pulmonary fibrosis (PF), the most common type of interstitial lung disease, is characterized by thick and stiff tissue that turns into scar tissue over time. Because of the increasing presence of scar tissue in the lungs, the organ is not able to properly transport oxygen into the bloodstream. When the blood does not get enough oxygen, symptoms result including shortness of breath; persistent, dry and hacking cough; fatigue; unexplained weight loss; aching muscles and joints; and clubbing of the fingertips.

While the majority of pulmonary fibrosis cases are classified as idiopathic, meaning the cause is not fully understood, there are numerous reasons known that contribute to the disease development. Included are: occupational and environmental exposure to pollutants, specific medication or drugs, radiation therapy, some connective tissue diseases and interstitial lung disease.

Treatments for pulmonary fibrosis include medicines, oxygen therapy, pulmonary rehabilitation, or a lung transplant.

Average Prognosis and Life Expectancy for Pulmonary Fibrosis

Pulmonary fibrosis is a progressive and life-threatening disease with a poor prognosis.

According to the study “Clinical Course and Prediction of Survival in Idiopathic Pulmonary Fibrosis,” median survival of patients who suffer from pulmonary fibrosis is 2.5 years, but some patients live much longer than that. The most common cause of death among patients is respiratory failure.

A lack of reliable prediction models that can be used in clinical practice makes it difficult to access individual risk of mortality.

From the study: “Although IPF has an overall poor prognosis, the clinical course of individual patients varies from slow progression to acute decompensation and death. Several individual clinical variables have been shown to correlate with survival; however, there is no established method of combining these predictors to accurately determine prognosis or define the stage of disease. More accurate prediction of survival in IPF would be useful to facilitate counseling patients and their families, to aid communication among providers, and to guide optimal timing of transplantation.”

Risk Factors that Impact Pulmonary Fibrosis Prognosis

A series of factors impact the prognosis of a patient with pulmonary fibrosis.

Death rate increases as the patient gets older; rates are consistently higher among men than women. Interestingly, deaths occur most in the winter. The study demonstrated that five-year survival rates are only 20 to 30% and the majority of the cases are related to progression of lung fibrosis instead of commonly occurring comorbid (co-existing) conditions.

Patients with pulmonary fibrosis frequently need to be hospitalized with respiratory problems and often experience subacute deterioration before dying.

Ischemic heart disease, heart failure, bronchogenic carcinoma, infection and pulmonary embolism are also common causes of mortality among these patients.

Some suggest that individual predictors of survival can include ethnicity, smoking, dyspnea, physical features and other comorbidities.

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Note: Pulmonary fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.