Rare Disease Highlight: Idiopathic Pulmonary Fibrosis

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Rare Disease Highlight

Idiopathic pulmonary fibrosis (IPF) is the most commonly observed form of idiopathic interstitial pneumonia, an aging-associated, progressive, chronic, and irreversible lung disease with very limited therapeutic options 1,2. IPF is characterized by progressive scarring of the lungs causing respiratory failure and death. The exact cause of IPF is unclear, although there are some environmental and genetic risk factors to the disease including middle-to-older age, smoking, and chronic inflammation 1,3. Symptoms of IPF include shortness of breath after exertion combined with dry cough. As the disease progresses, discoloration of the skin and signs of right ventricular failure may occur together with respiratory failure, eventually leading to death 4.  Currently there are no approved therapies for the treatment of IPF, however supportive therapies are available to manage symptoms. IPF is rare, with an estimated incidence rate of 2.8-9.3 per 100,000 per year in North America and a low survival of only 20% for five years 5.

References used
  1. Pardo, A. & Selman, M. Lung fibroblasts, aging, and idiopathic pulmonary fibrosis. Ann. Am. Thorac. Soc. 13, S417–S421 (2016).
  2. Zieliński, M., Sitek, P. & Ziora, D. Idiopathic pulmonary fibrosis coexisting with lung cancer — a review. Adv. Respir. Med. (2015). doi:10.5603/ARM.a2018.0052
  3. Kaunisto, J., Salomaa, E., Hodgson, U., Kaarteenaho, R. & Myllärniemi, M. Idiopathic pulmonary fibrosis – a systematic review on methodology for the collection of epidemiological data. 1–11 (2013). doi:10.1186/1471-2466-13-53
  4. Sgalla, G. I., Biffi, A. L. & Richeldi, L. U. C. A. Idiopathic pulmonary fibrosis : Diagnosis , epidemiology and. 427–437 (2016). doi:10.1111/resp.12683
  5. Barratt, S. L., Creamer, A., Hayton, C. & Chaudhuri, N. Idiopathic Pulmonary Fibrosis ( IPF ): An Overview. 1–21 (2018). doi:10.3390/jcm7080201

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