Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive irreversible fibrotic lung disease of unknown cause. It occurs in older patients and is limited to the lungs. The prognosis is dismal with a median survival of 3-5 years after diagnosis. The diagnosis is based on a definite pattern of usual interstitial pneumonia on high resolution computed tomography or specific combinations of radiological and histopathological patterns. Early diagnosis and referrral is recommended as anti-fibrotic treatment with pirfenidone or nintedanib that can slow down progression has become available. All patients should be evaluated for lung transplantation.
Highlights
Idiopathic pulmonary fibrosis (IPF) is a progressive irreversible fibrotic lung disease of unknown cause
The diagnosis is based on a definite pattern of usual interstitial pneumonia on high resolution computed tomography or specific combinations of radiological and histopathological patterns
Diagnosis and referrral is recommended as anti-fibrotic treatment with pirfenidone or nintedanib that can slow down progression has become available
Summary
Idiopathic pulmonary fibrosis (IPF) is a progressive irreversible fibrotic lung disease of unknown cause. New international definitions, classifications, guidelines and treatment possibilities have developed in interstitial lung disease (ILD) and in IPF. The first pathologic classification was described in the 1960ies, and in the following 20-30 years no clear distinction was made between the inflammatory and fibrotic ILDs which led to an exaggerated optimism of the effect of steroid treatment. The first guideline for IPF was published in 2011 and provided a new definition of the disease based on the exclusion of all known causes for ILD and the identification of specific combinations of radiological and histological patterns of UIP [1]. The incidence of IPF seems to have increased in recent years, probably due to improved and faster diagnostic procedures.
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