Diagnosis of interstitial pulmonary fibrosis-an update.

ABSTRACT

The most common and most lethal type of idiopathic interstitial pneumonia (IIP) is idiopathic pulmonary fibrosis (IPF), which accounts for 55% of lung diseases classified as IIPs. Diagnosis of IPF requires precision and a multidisciplinary approach .Indeed, an early and accurate diagnosis of IPF is critical for a better outcome, especially with the advent of new specific treatments for this disease. The previous guidelines using major and minor criteria for the clinical (i.e. non-pathological) diagnosis of IPF have been discarded, as it is now clear that, in an appropriate clinical setting, the presence of a classical UIP pattern on the HRCT scan is sufficient for a diagnosis of IPF to be made. In the presence of the four classical features, that together accurately identify a Usual interstitial pneumonia (UIP) pattern, a definitive diagnosis of IPF can be made. Guidelines emphasizes the importance of multidisciplinary discussion between clinicians, radiologists and pathologists to improve diagnostic confidence. The course of disease in IPF is unpredictable, but the importance of an early diagnosis is clear, as individuals with less severe lung function abnormalities have a better prognosis.