Pathophysiology Of Interstitial Lung Disease

Interstitial lung diseases (ILD) are a group of lung disorders that involve scarring or thickening of the tissues between the air sacs in the lungs known as the interstitium. The interstitium helps provide support for the thin walls of the air sacs and contains blood vessels, the lymphatic system, nerves and connective tissues. When the interstitium becomes damaged, scarred or thickened, normal gas exchange between the air sacs and blood vessels becomes impaired. This makes it difficult for oxygen to pass from the air sacs into the blood and for carbon dioxide to pass out from the blood into the air sacs.

There are over 200 different conditions that fall under the classification of ILD. While the specific causes may differ, the end result is similar - the formation of scar tissue or fibrosis in the lungs that stiffens the lungs and reduces their ability to transport oxygen into the bloodstream. Some of the more common forms of ILD include idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis, nonspecific interstitial pneumonia and connective tissue disease-associated Interstitial Lung Diseases.

Idiopathic Pulmonary Fibrosis

IPF is a progressive fibrotic lung disease of unknown cause that primarily affects older adults. It occurs when lung tissue deep in the lungs becomes thick and stiff over time due to abnormal scarring. This process, called fibrosis, makes it increasingly difficult for the lungs to work properly. With IPF, this scarring process happens without a known cause.

The signs and symptoms of IPF mainly involve shortness of breath and a dry cough. As the disease progresses, the symptoms tend to get worse over time. Physical activity becomes increasingly difficult and exhausting for patients. Eventually, the scarring can become severe enough that the lungs can no longer provide an adequate oxygen supply to the blood. Breathing may also become labored due to the stiffening of the lung tissue. IPF has no cure and gradually worsens over time. Current treatment focuses on slowing disease progression.

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