Interstitial Pulmonary Fibrosis

Excerpt

Nonidiopathic interstitial pulmonary fibrosis (non-IPF) describes a group of interstitial lung diseases (ILD) that cause inflammation and fibrosis of the lung interstitium, leading to impaired gas exchange due to a known cause. Depending on the specific disorder, it can also affect the trachea, bronchi, bronchioles, alveoli, and pleura. Most of these diseases are characterized by clinical, radiographic, pathologic, and physiologic findings. The classic features often include progressive shortness of breath and cough, chest imaging abnormalities, and inflammatory and fibrotic changes on histology. A restrictive pattern with a decreased diffusing capacity for carbon monoxide (DLCO) is often seen in pulmonary function testing (PFT).