Cause/etiology/symptoms
The cause of IPF is unknown; it may arise either from an autoimmune disorder or as a result of infection. Cigarette smoking has been identified as a potential risk factor and one case control study has suggested an assocaition between antidepressants and the risk of development of pulmonary fibrosis, alhtough this inding in the pathogenesis of IPF is unknown. (Am J Respir Grit Care Med 161: 646-664)
IPF is defined as a specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the histologic appearance of usual interstitial pneumonia (UIP) on surgical (thoracoscopic or open) lung biopsy. (Am J Respir Grit Care Med 161: 646-664).
Symptoms include dyspenea (shortness of breach) and dry cough. Death can result from hypoxemia, right heart failure, heart attack, lung embolism, stroke or lung infection.
Idiopathic pulmonary fibrosis (IPF): is characterized by inflammation and eventually fibrosis of lung tissue.
Treatment
Treatment includes corticosteroids (e.g., prednisone), penicillamine, and various anti-neoplastics. Other treatments include oxygen administration and in extreme cases, lung transplantation.
A small population of bone marrow stem cells which have the CXCR4 receptor are believed to move to the lung in the pathology of IPF in response to the chemokine stromal-dervied factor-1 (SDF-1) which is secreted by neutrophils and macrophages. These stem cells then differentiate into fibrocytes and elaborate collagen, contributing to fibrotic damage.