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Idiopathic pulmonary fibrosis is interstitial lung disease for which no obvious cause can be identified (idiopathic) and is associated with typical findings both radiographic (basal and pleural-based fibrosis with honeycombing) and pathologic (temporally and spatially heterogeneous fibrosis, histopathologic honeycombing, and fibroblastic foci).
It is a type of interstitial lung disease. [3] People often benefit from pulmonary rehabilitation and supplemental oxygen. [1] Certain medications like pirfenidone (Esbriet) or nintedanib (Ofev) may slow the progression of the disease. [2] Lung transplantation may also be an option. [1] About 5 million people are affected globally. [8]
The differential diagnosis includes other types of lung disease that cause similar symptoms and show similar abnormalities on chest radiographs. Some of these diseases cause fibrosis, scarring or honeycomb change. The most common considerations include: chronic hypersensitivity pneumonitis; non-specific interstitial pneumonia; sarcoidosis
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). There are seven recognized distinct subtypes of IIP. [2]
Causes include environmental pollution, certain medications, connective tissue diseases, infections, and interstitial lung diseases. [1] [3] [6] But in most cases the cause is unknown (idiopathic pulmonary fibrosis). [1] [3] Diagnosis may be based on symptoms, medical imaging, lung biopsy, and lung function tests. [1]
Acute interstitial pneumonitis (also known as acute interstitial pneumonia) is a rare, severe lung disease that usually affects otherwise healthy individuals. There is no known cause or cure. Acute interstitial pneumonitis is often categorized as both an interstitial lung disease and a form of acute respiratory distress syndrome (ARDS).
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