Idiopathic pulmonary fibrosis: phenotypes and comorbidities

Clin Chest Med. 2012 Mar;33(1):51-7. doi: 10.1016/j.ccm.2011.12.005.


Idiopathic pulmonary fibrosis (IPF) is a progressive fatal disease of the lung with an unknown etiology and limited treatment options. Three distinct phenotypes of IPF have been proposed: combined pulmonary fibrosis and emphysema, disproportionate pulmonary hypertension in IPF, and rapidly progressive IPF. Although treatment options for IPF are limited, much can be done to identify and alleviate symptoms from comorbidities, potentially improving the overall quality of life and well-being of these patients. This article describes emerging evidence to support the hypothesis that there is more than one phenotype for IPF and describes the common comorbidities seen in this disease.

Publication types

  • Review

MeSH terms

  • Canada / epidemiology
  • Cardiovascular Diseases / complications
  • Comorbidity
  • Depression / etiology
  • Disease Progression
  • Gastroesophageal Reflux / complications
  • Humans
  • Hypertension, Pulmonary / complications*
  • Hypertension, Pulmonary / epidemiology
  • Idiopathic Pulmonary Fibrosis / complications*
  • Idiopathic Pulmonary Fibrosis / diagnosis
  • Idiopathic Pulmonary Fibrosis / epidemiology*
  • Idiopathic Pulmonary Fibrosis / genetics
  • Idiopathic Pulmonary Fibrosis / psychology
  • Kaplan-Meier Estimate
  • Lung Neoplasms
  • Phenotype*
  • Prognosis
  • Pulmonary Emphysema / complications*
  • Pulmonary Emphysema / epidemiology
  • Pulmonary Fibrosis / complications*
  • Pulmonary Fibrosis / epidemiology
  • Quality of Life
  • Risk Factors
  • Tomography, X-Ray Computed
  • Venous Thromboembolism / complications