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Primary ciliary dyskinesia (PCD) is characterized by functional abnormalities of the cilia, leading to chronic airway infection and remodeling. Lung function tests like inert gas washout measurements detect ventilation inhomogeneity, but cannot determine its origin. Novel magnetic resonance imaging (MRI) methods such as Matrix Pencil (MP) decomposition MRI can visualize functional changes in the lung without the administration of contrast agents and the need for breathing maneuvers. This study investigates the correlation between functional and morphological MRI and lung function indices in patients with PCD. Thirty-one patients with PCD (mean age 13.5 years, range 5–28) underwent MRI and lung function tests on the same day. Functional MP-MRI provided semi-quantitative measures of the perfusion (RQ) and ventilation (RFV) impairment as percentages of the affected lung volume. Morphological MRI was evaluated using a score derived from cystic fibrosis. Lung function tests provided information about global (lung clearance index, LCI) ventilation inhomogeneity. Both MRI and inert gas washout measurements detected functional impairment in PCD: RFV ranged from 11% to 29% and RQ ranged from 9% to 28%. RFV and RQ moderately correlated with LCI (r=0.4, p=0.04; r=0.4, p=0.03, respectively). Total morphology scores strongly correlated with LCI (r=0.6, p Functional MP-MRI is a novel, sensitive and non-invasive method to detect and visualize perfusion and ventilation abnormalities. For the first time we could show a correlation between a lung function test and a functional and structural MRI in patients with PCD.

Introduction: High resolution computed tomography (HRCT) is essential in the diagnostic algorithm for idiopathic pulmonary fibrosis (IPF). If identifiable causes of interstitial lung disease (ILD) are absent, an HRCT showing a typical usual interstitial pneumonia (UIP) pattern is diagnostic for IPF. Here we report first experiences with a teleradiology-project that offers physicians the possibility for second opinion HRCT read regarding the presence of UIP pattern in patients with suspected IPF. Methods: HRCT were uploaded through a secured internet connection. Only images in original Digital Imaging and Communication in Medicine (DICOM) format were accepted. HRCTs were assessed by a radiologist and a pulmonologist, both experienced in the diagnosis of ILD. According to current guidelines each HRCT was rated showing either typical UIP pattern, possible UIP pattern, or being inconsistent with UIP pattern. Results: Between March 2015 and January 2017 we received 160 HRCTs for a second opinion read from physicians in 10 different Eastern European countries. All images were of diagnostic quality. In 58/160 (36%) patients a typical UIP pattern was diagnosed, possible UIP pattern was detected in 27/160 (17%), and in 75/160 (47%) patients the prevailing pattern was considered as inconsistent with UIP. Conclusions: Our data demonstrate a useful application of teleradiology to provide expert reading of HRCT in patients with suspected IPF, particularly when local radiological expertise is limited. This project is not intended to replace local multidisciplinary panels, but to support local panels by providing specific expertise. More experience will be needed to define the role of teleradiology in patients with suspected IPF.

Lung function tests are commonly used to monitor lung disease in cystic fibrosis (CF). While practical, they cannot locate the exact origin of functional impairment. Contemporary magnetic resonance imaging (MRI) techniques provide information on the location of disease but the need for contrast agents constrains their repeated application. We examined the correlation between functional MRI, performed without administration of contrast agent, and lung clearance index (LCI) from nitrogen multiple-breath washout (N2-MBW).40 children with CF (median (range) age 12.0 (6–18) years) and 12 healthy age-matched controls underwent functional and structural MRI and lung function tests on the same day. Functional MRI provided semiquantitative measures of perfusion (RQ) and ventilation (RFV) impairment as percentages of affected lung volume. Morphological MRI was evaluated using CF-specific scores. LCI measured global ventilation inhomogeneity.MRI detected functional impairment in CF:RFV19–38% andRQ16–35%.RFVandRQcorrelated strongly with LCI (r=0.76, pNoninvasive functional MRI is a promising method to detect and visualise perfusion and ventilation impairment in CF without the need for contrast agents.