Idiopathic pulmonary fibrosis

Med Clin (Barc). 2022 Aug 26;159(4):189-194. doi: 10.1016/j.medcli.2022.02.020. Epub 2022 Jun 1.
[Article in English, Spanish]


Idiopathic pulmonary fibrosis is defined as a chronic progressive fibrosing interstitial pneumonia of unknown etiology. There are intrinsic and extrinsic risk factors that could favor the development of the disease in individuals with a genetic predisposition. The diagnosis is made by characteristic radiological and/or histological findings on high-resolution computed tomography and lung biopsy, respectively, in the absence of a specific identifiable cause. The median survival of the disease for patients without treatment is 3-5years from the onset of symptoms, although its natural history is variable and unpredictable. Currently, there are two antifibrotic drugs that reduce disease progression. The multidisciplinary approach will consider the nutritional and emotional status, physical conditioning, and treatment of comorbidities, as well as lung transplantation and palliative care in advanced stages. The following article reviews the fundamental aspects for the diagnosis and treatment of idiopathic pulmonary fibrosis.

Keywords: Diagnosis; Diagnóstico; Fibrosis pulmonar idiopática; Idiopathic pulmonary fibrosis; Tratamiento; Treatment.

Publication types

  • Review

MeSH terms

  • Biopsy / adverse effects
  • Humans
  • Idiopathic Pulmonary Fibrosis* / diagnosis
  • Idiopathic Pulmonary Fibrosis* / etiology
  • Idiopathic Pulmonary Fibrosis* / therapy
  • Lung / pathology
  • Lung Diseases, Interstitial* / diagnosis
  • Lung Diseases, Interstitial* / etiology
  • Lung Diseases, Interstitial* / therapy
  • Lung Transplantation*
  • Tomography, X-Ray Computed / methods