Conditions with 
Pulmonary Fibrosis

There are many different conditions associated with pulmonary fibrosis. Some are referred to as interstitial lung diseases (ILDs). ILDs with pulmonary fibrosis are a challenging group of diseases that affect the interstitium of the lung (the space between the air sacs in the lung) and lead to the formation of scar tissue. The following diagram shows normal lung air sacs on the left and the thickened air sacs in ILDs on the right. 

ILDs may cause scar tissue to build up in your lungs. This scar tissue is known as pulmonary fibrosis and affects the tiny air sacs and blood vessels responsible for providing oxygen to all parts of the body. In some cases, there is excessive scarring with an increasing extent of the lung lost to fibrosis and it becomes harder for oxygen to pass through the lungs into the bloodstream, which can result in shortness of breath. 

There are many different ILDs, and pulmonary fibrosis is an important factor in some of them. ILDs with pulmonary fibrosis can be caused by certain medications or drugs, occupational or environmental exposure, autoimmune conditions or, many times, the cause is never known. Some of the autoimmune conditions that are associated with ILD include scleroderma, rheumatoid arthritis, sarcoidosis and Sjögren's syndrome.