Acute exacerbation of idiopathic pulmonary fibrosis

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic disorder and ultimately fatal disease. Acute exacerbation of IPF (AEIPF) is defined as a rapid deterioration with new radiographic opacities in patients with IPF with no identifiable cause such as infection, pulmonary embolism, or heart failure. Reported incidence of this disease entity is highly variable and the etiology is unknown. Research into the pathobiology of this process has implicated possible causes including microaspirations and occult viral infection. AE-IPF manifests histopathologically as diffuse alveolar damage and can have features of organizing pneumonia in the later phase. High-resolution computed tomography of the chest demonstrates new ground-glass opacities and can have prognostic value based on the pattern and extent of opacities. The mortality of this condition is very high and currently there is no proven effective treatment other than lung transplantation. AE-IPF is a major cause of morbidity and mortality in patients with IPF and further research is needed to elucidate the pathobiology and develop effective treatments.

Original languageEnglish (US)
Pages (from-to)262-268
Number of pages7
JournalClinical Pulmonary Medicine
Volume21
Issue number6
DOIs
StatePublished - Jan 1 2014

Fingerprint

Idiopathic Pulmonary Fibrosis
Lung Transplantation
Mortality
Virus Diseases
Pulmonary Embolism
Research
Glass
Pneumonia
Thorax
Heart Failure
Tomography
Morbidity
Incidence
Therapeutics
Infection

Keywords

  • acute exacerbation
  • Biomarkers
  • Histopathology
  • Idiopathic pulmonary fibrosis
  • Incidence
  • Prognosis
  • Risk factors

Cite this

Acute exacerbation of idiopathic pulmonary fibrosis. / Perlman, David M; Bhargava, Maneesh; Kim, Hyun J; Ritter, Jon H.; Tomic, Rade.

In: Clinical Pulmonary Medicine, Vol. 21, No. 6, 01.01.2014, p. 262-268.

Research output: Contribution to journalArticle

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