IPF acute exacerbations

The clinical course of idiopathic pulmonary fibrosis (IPF) may include periods of acute deterioration in respiratory function, which are termed acute IPF exacerbations. Patients who experience an acute exacerbation show a worsened prognosis, and acute exacerbations are believed to reflect disease progression in IPF. Reducing the risk of acute exacerbations is a key treatment goal in the management of all IPF patients.

Risk factors for acute exacerbations in IPF are unclear, but there is evidence that a number of factors may increase risk. Lower total lung capacity, lower forced vital capacity (FVC) and/or lower diffusing capacity of the lung for carbon monoxide (DLco) have been shown to increase the risk of AEx-IPF,1,2 as has a greater decline in FVC over time.3 GERD, a common comorbidity in patients with IPF, may also contribute to the pathogenesis of IPF through introduction of gastric acid into the respiratory tree.4,5

References

  1. Mura M, Porretta MA, Bargagli E, Sergiacomi G, Zompatori M, Sverzellati N, Taglieri A, Mezzasalma F, Rottoli P, Saltini C, Rogliani P: Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study. Eur Respir J 2012, 40:101–109.
  2. Song JW, Hong SB, Lim CM, Koh Y, Kim DS: Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J 2011, 37:356–363.
  3. Kondoh Y, Taniguchi H, Katsuta T, Kataoka K, Kimura T, Nishiyama O, Sakamoto K, Johkoh T, Nishimura M, Ono K, Kitaichi M: Risk factors of acute exacerbation of idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2010, 27:103–110.
  4. Lee JS, Song JW, Wolters PJ, Elicker BM, King TE Jr, Kim DS, Collard HR: Bronchoalveolar lavage pepsin in acute exacerbation of idiopathic pulmonary fibrosis. Eur Respir J 2012, 39:352–358.
  5. Tobin RW, Pope CE, Pellegrini CA, Emond MJ, Sillery J, Raghu G: Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1998, 158:1804–1808.