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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 ...
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]
It is the main cause of restrictive lung disease that is intrinsic to the lung parenchyma. In contrast, quadriplegia [16] and kyphosis [17] are examples of causes of restrictive lung disease that do not necessarily involve pulmonary fibrosis.
End stage pulmonary disease (ESPD) [2] is the result of chronic progressive lung diseases like COPD, [3] idiopathic pulmonary fibrosis, or systemic progressive diseases that affect the lungs such as cystic fibrosis or granulomatosis with polyangiitis. It is defined as when the lungs can no longer or barely remove enough carbon dioxide or supply ...
Restrictive lung diseases are a category of extrapulmonary, pleural, or parenchymal respiratory diseases that restrict lung expansion, [2] resulting in a decreased lung volume, an increased work of breathing, and inadequate ventilation and/or oxygenation. Pulmonary function test demonstrates a decrease in the forced vital capacity.
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.
The morbidity associated with DIPNECH is due to the associated obstructive lung disease. The lung disease tends to be slowly progressive, but given enough time can lead to significant disability and require supplemental oxygen therapy. [8] There have been reports of lung transplantation in the setting of end-stage DIPNECH. [11]
Lymphangioleiomyomatosis (LAM) is a rare, progressive and systemic disease that typically results in cystic lung destruction. It predominantly affects women, especially during childbearing years. [1]