Pathophysiology of Pulmonary Fibrosis

This week we will be going over the pathophysiology of pulmonary fibrosis, which basically means that we will be talking about the structural and functional changes that your body goes through when you develop pulmonary fibrosis. This is a little bit difficult with the disease of pulmonary fibrosis though because the cause of the most common type of the disease, idiopathic pulmonary fibrosis, is still somewhat of a mystery (idiopathic meaning developing from an unknown cause). But it has been shown that triggers such as allergens, chemicals, radiation and environmental particles cause other types of pulmonary fibrosis.

            Pulmonary fibrosis is caused from scarring so when it develops your body goes through the three phases of wound healing, which are injury, inflammation, and repair. With normal wound healing your body normally brings the tissue back to its normal functioning level or at least to somewhere pretty close, but when people develop pulmonary fibrosis something goes wrong in the healing process. Normally the problem is that there is a persistent irritant that causes your body to constantly go through the wound healing process. This then causes the scar tissue to build up inside the lungs, making oxygen exchange more difficult. The factors that contribute to how bad the scarring is are the degree or level of inflammation, the angiogenesis or the formation of new blood vessels, and the amount of cellular deposits. All of these factors come together to make the perfect storm that causes bad pulmonary fibrosis to form.

           

            So far the main contributors to the mortality and morbidity of pulmonary fibrosis are unknown. It is known that it is mostly due to how you body deals with the irritants and how much scar tissue it develops in the healing process. It has also been shown that old age, smoking, cancer treatments, and occupational hazards all increase the morbidity and mortality.  Idiopathic pulmonary fibrosis is still being studied and is a big mystery to a lot of the medical world.

References:

Wilson, M., & Wynn, T. (2009, January 7). Pulmonary fibrosis: Pathogenesis, etiology and regulation. Retrieved January 25, 2015, from http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2675823/

Pulmonary fibrosis. (n.d.). Retrieved January 25, 2015, from http://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/basics/risk-factors/con-20029091

Ley, B. (n.d.). Clinical Course and Prediction of Survival in Idiopathic Pulmonary Fibrosis. Retrieved January 25, 2015, from http://www.atsjournals.org/doi/full/10.1164/rccm.201006-0894CI#.VMRMbmTF8rM