Pulmonary Fibrosis, Idiopathic

Alternative Names

  • IPF
  • Idiopathic Pulmonary Fibrosis, Familial
  • Fibrosing Alveolitis, Cryptogenic
  • Fibrocystic Pulmonary Dysplasia
  • Interstitial Pneumonitis, Usual
  • UIP
  • Hamman-Rich Disease, Included
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WHO-ICD-10 version:2010

Diseases of the respiratory system

Other respiratory diseases principally affecting the interstitium

OMIM Number



Idiopathic pulmonary fibrosis is one of a family of idiopathic pneumonias sharing clinical features of shortness of breath, radiographically evident diffuse pulmonary infiltrates, and varying degrees in inflammation, fibrosis, or both on lung biopsy. In some cases, the disorder can be rapidly progressive and characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease. [From OMIM]

Epidemiology in the Arab World

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Other Reports


Kanj et al. 2006 described the first instance of familial idopathic pulmonary fibrosis in 3 Lebanese siblings with similar clinical, radiological, and histological features.

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