Extract
The efficacy and approval of two antifibrotic drugs [1, 2], the increased mortality of idiopathic pulmonary fibrosis (IPF) from corticosteroids and anticoagulants [3, 4], and the availability of data from multiple drug trials have tremendously increased the need for an accurate diagnosis of interstitial lung disease (ILD). As stated in international guidelines [5], in the appropriate clinical setting with all possible causes of ILD ruled out, the presence of a definite usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT) is sufficient for the diagnosis of IPF. In the absence of a UIP pattern on CT, establishing a secure diagnosis of IPF requires a surgical lung biopsy [5]. This approach, therefore, relies heavily on the CT appearance of ILD that is stratified into three categories, namely UIP, possible UIP and inconsistent with UIP. In theory, a lung biopsy should be contemplated in patients with a pattern of possible UIP or inconsistent with UIP. All possibly relevant information available is then synthesised during the multidisciplinary discussion among clinicians, radiologists and pathologists, all being experts in the field of ILD, a process that increases the accuracy of the diagnosis [6] and impacts management [7]. The multidisciplinary discussion also allows the diagnosis to be made with higher confidence than by clinicians or radiologists alone (although reproducibility between different teams needs to be optimised for diagnoses other than IPF [8]), and has become the gold standard for the diagnosis of ILD.
Abstract
Diagnostic guidelines now need to be amended to better correspond to the pragmatic management of ILD http://ow.ly/97M8304aylG
Footnotes
This article has been revised according to the correction published in the December 2016 issue of the European Respiratory Journal.
Conflict of interest: Disclosures can be found alongside this article at erj.ersjournals.com
- Received August 16, 2016.
- Accepted August 16, 2016.
- Copyright ©ERS 2016