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Idiopathic pulmonary fibrosis

Disease definition

Idiopathic pulmonary fibrosis (IPF) is a nonneoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known cause.

ORPHA:2032

  • Synonym(s):
    • CFA
    • Cryptogenic fibrosing alveolitis
    • UIP
    • Usual interstitial pneumonia
  • 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

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