Medical gases to treat idiopathic pulmonary fibrosis
Publication Date: 2011-Feb-01
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The lung serves the vital function of providing access for oxygen to the pulmonary circulation, allowing its delivery to all other organs.
To achieve this, the lung has evolved to comprise a series of tubes — the bronchial tree — that lead in a branching network to numerous air sacs — alveoli — that together form a structure that is similar to a bee’s honeycomb.
The bronchial tree provides the conduit through which inspired gases travel to the alveoli. The extremely thin walls of the alveoli allow the diffusion of oxygen into the adjacent blood capillaries and the opposite movement of carbon dioxide1.
These delicate alveolar structures are exquisitely sensitive to injury and subsequent lung scarring. When the degree of lung injury is mild, damaged tissue will normally be repaired, whereas excess cell death may lead to unrepairable lung damages and pulmonary fibrosis.
Excessive, uncontrolled fibrosis can lead to impairment of gas exchange and ultimately respiratory failure, organ dysfunction and death.
Idiopathic pulmonary fibrosis (IPF) is one of a family of idiopathic pneumonias, sharingthe clinical features of shortness of breath, radiographicallyevident diffuse pulmonary infiltrates, and varying degrees ofinflammation, fibrosis, or both on biopsy.
It is a progressive disease with a devastating prognosis and a median survival from the time of diagnosis of only 2-3 years. IPF is characterized by sequential acute lunginjury that results in a progressive accumulation of fixed fibrosiswith architectural distortion and loss of lung function2.
Newer insights into the pathogenesis of idiopathic pulmonary fibrosis suggest a crucial role for epithelial apoptosis as a key profibrotic event in lung fibrogenesis3.
Moreover, in the lungs ofIPF patients, increased epithelial apoptosis represents a persistentfinding (particularly in areas of collagen deposition) supportingan interaction between altered apoptosis and the pathogenesisof the disease4.
Furthermore, carbon monoxide (CO) has been shown to suppress e...