Interstitial lung disease (ILD), also known as diffuse parenchymal lung disease (DPLD), is an umbrella term for many different lung diseases.1 The key area of damage is the interstitial (or parenchymal) area of the lung, which is the area between the microscopic-air or alveolar sacs. Diffuse simply means scattered widely. There are estimated to be over 200 identified causes1 of ILD and its prevalence has been reported as between one and three in 10,000 people.2
One of the challenges involved in diagnosing ILD, is the wide range of potential causes, the complexity of diagnostic tests, and their interpretation. Although some ILDs are acute onset, many people with ILD present late for diagnosis. When a patient presents late on a progressive disease trajectory, their care can often be about symptom control and become palliative in nature, rather than curative.3
Irritation of the tissue area surrounding the alveoli, the air sacs (alveolar epithelium, pulmonary endothelium, perivascular and perilymphatic tissues) can stem from many causes, which can often be prolonged. This can result in stiff or scarred tissue, known as fibrosis. Extensive alteration occurs to both the alveolar and airway architecture as a result of inflammation and fibrosis and, in many cases, this damage is irreversible. Fibrosis stops the lungs from working properly as the lungs become smaller and stiffer, affecting the abilty to breathe and carry ox
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