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It is a type of interstitial lung disease. [3] People often benefit from pulmonary rehabilitation and supplemental oxygen. [1] Certain medications like pirfenidone or nintedanib may slow the progression of the disease. [2] Lung transplantation may also be an option. [1] About 5 million people are affected globally. [8]
Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), [3] is a group of respiratory diseases affecting the interstitium (the tissue) and space around the alveoli (air sacs) of the lungs. [4] It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It ...
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.
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]
Desquamative interstitial pneumonia (DIP) is a type of idiopathic interstitial pneumonia featuring elevated numbers of macrophages within the alveoli of the lung. [1] DIP is a chronic disorder with an insidious onset. Its common symptoms include shortness of breath, coughing, fever, weakness, weight loss, and fatigue.
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).
The chest x-ray is distinctive with features that appear similar to an extensive pneumonia, with both lungs showing widespread white patches. The white patches may seem to migrate from one area of the lung to another as the disease persists or progresses. Computed tomography (CT) may be used to confirm the diagnosis.
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]