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Idiopathic pulmonary fibrosis

Disease definition

An interstitial lung disease with a poor prognosis, that is characterized by the progressive formation of scar tissue within the lungs in the absence of any known cause.


Classification level: Disorder
  • Synonym(s):
    • IPF
  • Prevalence: 1-5 / 10 000
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: Adult
  • ICD-10: J84.1
  • OMIM: 178500  616371  616373
  • UMLS: C0085786  C1800706
  • MeSH: D054990
  • GARD: 8609
  • MedDRA: 10021240

Detailed information

Article for general public


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