ESR/ERS - Novel strategies in idiopathic interstitial pneumonia
1. To appreciate the rationale for the roles of pathology, radiology, and pneumology.
2. To understand strengths and weaknesses of pathology.
3. To become familiar with novel developments in histopathology.
Updated clinical practice guidelines for classification. The 2011 ATS/ERS/JRS/ALAT guidelines for the diagnosis of idiopathic pulmonary fibrosis (IPF) serve to identify a large sub-group of IPF patients in whom a confident diagnosis can be made with, based upon a) typical HRCT findings of usual interstitial pneumonia (UIP) or b) UIP at surgical biopsy when HRCT appearances are atypical. However, guideline criteria fail to diagnose at least 50% of IPF patients, due to concerns about mortality associated with diagnostic surgical biopsy. Developments related to classification and the diagnostic tools by which disease is classified include: 1) in the 2013 ATS/ERS updated classification of the idiopathic interstitial pneumonia, a category of “unclassifiable disease” is formalised and a disease behaviour classification is proposed as a guide to treatment goals and their achievement. This category includes a large sub-group of IPF patients not diagnosed using current criteria. 2) The development of cryobiopsy should allow increased use of histologic data in the formulation of an IPF diagnosis with particular reference to older patients with greater pulmonary function impairment. 3) A current ERS task force has proposed an algorithm allowing the formulation of a working diagnosis of IPF in multidisciplinary discussion in cases not meeting guideline criteria.
1. To consolidate knowledge about the anatomical and pathological correlates.
2. To learn about the criteria for usual interstitial pneumonia (UIP).
3. To learn about the criteria for non-specific interstitial pneumonia (NSIP).
In 2013, the consensus statement on idiopathic interstitial pneumonias (IIPs) was updated, with the aim of adding further precision to the diagnosis/management of the IIPs. Radiologists are central to the evaluation of patients with IIPs. Radiologists should first distinguish fibrosing from non-fibrosing entities. The main computed tomography (CT) differential of fibrosing lung disease is between the usual interstitial pneumonia (UIP) pattern and other fibrotic patterns such as non-specific interstitial pneumonia (NSIP). When definite UIP pattern is not fulfilled, the differential between UIP and NSIP should rely on CT characteristics of reticular opacities and clinical features, notably age. An important addition is a disease behaviour classification, which provides a framework for management of cases that are unclassifiable or where a histologic diagnosis cannot be obtained.
1. To learn about the clinical course and prognosis.
2. To consolidate knowledge about the standard therapy.
3. To become familiar with novel therapies.
IPF is a disorder with a sort of unpredictable behaviour with, broadly speaking, rapid progressions and slow progressions. Biomarkers such as specific autoantibodies, morphologic features might be valid indicators of the incoming lung function decline. The use of less invasive procedures such as transbronchial cryobiopsy may strengthen the clinical-radiologic correlation and allow identification of new imaging markers of the disorder that could have diagnostic and prognostic impact (presence of upper lobe subpleural thickening-elastotic fibrosis, nodular calcifications, peripheral bronchiolar proliferation, etc.). Also genomic studies on lung samples or even culture of cells obtained by this method could increment the "personalized diagnostic and therapeutic" approach. So far, however, specific antifibrotic drugs are identified as efficacious in reducing the functional decline in IPF subject and in reducing the mortality risk. Clinicians' task is to clearly define patients with a high probability diagnosis of IPF (not only according to rigid guidelines but also taking into account the clinical profile/clinical behaviour, data provided by biomarkers, CT scan and morphology) to adjust the therapy for the single patient. Learning points: clinical diagnosis of IPF, treatment of patients with a high probability diagnosis of IPF, identification of new effective endpoints to design clinical trials and identification of new efficacious drugs.
1. To consolidate knowledge about the rationale for therapy response imaging.
2. To learn about quantitative imaging biomarkers.
3. To become familiar with novel options of imaging inflammation.
Being an integral tool in the multidisciplinary team approach of idiopathic interstitial pneumonias (IIPs), CT helps to make the diagnosis and may predict prognosis and survival. However, since it is clear now that less typical CT patterns may also be predictive of pulmonary fibrosis and especially since the recent successful introduction of antifibrotic therapy, there is a growing need for a more accurate prognostic evaluation and for an accurate assessment of longitudinal behaviour and response to treatment. While monitoring prognosis and therapy response is predominantly based on clinical data (pulmonary function tests), many methods to quantify disease with CT (and few with MRI) ranging from visual estimates to the use of sophisticated software were developed and used although mostly in study circumstances. In this presentation, the need for prognostic and therapy response imaging and the potentials and pitfalls of (semi)quantitative imaging biomarkers will be discussed. Using imaging biomarkers is challenging since both qualitative and (semi)quantitative measurement results reflect the effect of different processes like inflammation, fibrosis and lung destruction that interact with each other, vary in time and may be different in different parts of the lung. In addition, some of these processes are not well understood. Especially, estimating the amount of inflammation may be difficult, although novel techniques to image inflammation have been proposed. A short review of these techniques will be given.