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Pulmonary fibrosis is a condition in which the lungs become scarred over time. [1] Symptoms include shortness of breath , a dry cough, feeling tired, weight loss, and nail clubbing . [ 1 ] Complications may include pulmonary hypertension , respiratory failure , pneumothorax , and lung cancer .
The yearly incidence of AE-IPF is between 10 and 15% of all patients. The prognosis of AE-IPF is poor, with mortality ranging from 78% to 96%. [63] Other causes of AE-IPF such as pulmonary embolism, congestive heart failure, pneumothorax, or infection need to be excluded. Pulmonary infection have to be ruled out by endotracheal aspirate or BAL.
Appearance of usual interstitial pneumonia (UIP) in a surgical lung biopsy at low magnification. The tissue is stained with hematoxylin (purple dye) and eosin (pink dye) to make it visible. The pink areas in this picture represent lung fibrosis (collagen stains pink). Note the "patchwork" (quilt-like) pattern of the fibrosis.
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]
Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia [1] are a class of diffuse lung diseases.These diseases typically affect the pulmonary interstitium, although some also have a component affecting the airways (for instance, cryptogenic organizing pneumonitis).
Combined pulmonary fibrosis and emphysema (CPFE), describes a medical syndrome involving both pulmonary fibrosis and emphysema. [ 1 ] [ 2 ] The combination is most commonly found in male smokers. Pulmonary function tests typically show preserved lung volume with very low transfer factor.
Differential diagnosis includes non-specific interstitial pneumonia, pulmonary Langerhans cell histiocytosis, respiratory bronchiolitis-associated interstitial lung disease, and hypersensitivity pneumonia. DIP is primarily treated by quitting smoking, but it may not be enough in all cases. In moderate to severe cases, corticosteroids are used.
Pulmonary function testing in people with organizing pneumonia, either cryptogenic or due to secondary causes, shows a restrictive defect with a decrease in the gas absorptive capacity of the lungs (seen as a decrease in the diffusion capacity of carbon monoxide). [9] Airflow obstruction is usually not seen on pulmonary function testing. [9]