Idiopathic pulmonary fibrosis clinical features and diagnosis

摘要

Idiopathic pulmonary fibrosis (IPF) is one of many conditions that are grouped together under the term "interstitial lung diseases". Despite considerable heterogeneity in the cause, presentation, treatment options and outcomes of these conditions, the interstitial lung diseases are grouped together because of similarities in clinical and radiological presentation (see Box 1, p221). The complex and historically inconsistent terminology, multitude and relative rarity of individual subtypes of interstitial lung disease contribute to confusion in their diagnosis and description. IPF, previously termed "cryptogenic fibrosing alveolitis" in the UK, is a chronic, progressive and fibrosing idiopathic interstitial pneumonia of unknown cause that occurs in older adults.' It is irreversible and has a poor prognosis - five-year survival is around 20% and median survival after diagnosis is around two and a half years.