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Life expectancy is generally less than five years. [3] Signs and symptoms ... The mean age of all pulmonary fibrosis patients is between 65 and 70 years, making age a ...
The disease newly occurs in about 12 per 100,000 people per year. [4] Those in their 60s and 70s are most commonly affected. [4] Males are affected more often than females. [4] Average life expectancy following diagnosis is about four years. [1]
The median survival in idiopathic pulmonary fibrosis is 3-3.5 years. In those who receive a lung transplant, the medial survival in idiopathic pulmonary fibrosis is 5.2 years, as compared to 6.7 years in those with other types of ILD. [8] ILD is associated with a 3-fold increased risk of lung cancer. [8]
Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. [1] The scarring (pulmonary fibrosis) involves the pulmonary interstitium (the supporting framework of the lung). UIP is thus classified as a form of interstitial lung disease.
Many cases of restrictive lung disease are idiopathic (have no known cause). Still, there is generally pulmonary fibrosis. [5] Examples are: Idiopathic pulmonary fibrosis; Idiopathic interstitial pneumonia, of which there are several types; Sarcoidosis; Eosinophilic pneumonia; Lymphangioleiomyomatosis; Pulmonary Langerhans' cell histiocytosis
The development of PMF is usually associated with a restrictive ventilatory defect on pulmonary function testing. PMF can be mistaken for bronchogenic carcinoma and vice versa. PMF lesions tend to grow very slowly, so any rapid changes in size, or development of cavitation, should prompt a search for either alternative cause or secondary disease.
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