Magnetic resonance imaging detects onset and association with lung disease severity of bronchial artery dilatation in cystic fibrosis

Abstract

RationaleBronchial artery dilatation (BAD) is associated with haemoptysis in advanced cystic fibrosis (CF) lung disease. Our aim was to evaluate BAD onset and its association with disease severity by magnetic resonance imaging (MRI).Materials and Methods188 CF patients (mean age 13.8±10.6 y, range 1.1–55.2 y) underwent annual chest MRI (median three exams, range one to six), contributing a total of 485 MRI examinations incl. perfusion MRI. Presence of BAD was evaluated by two radiologists in consensus. Disease severity was assessed using the validated MRI score and spirometry (ppFEV1).ResultsMRI demonstrated BAD in 71 (37.8%) CF patients consistently from the first available examination, and further ten (5.3%) patients first developed BAD during surveillance. Mean MRI global score in patients with BAD was 24.5±8.3 compared to 11.8±7.0 without BAD (p<0.001), and ppFEV1was lower in BAD compared to patients without (60.8versus82.0%, respectively, p<0.001). BAD was more prevalent in patients with chronicP. aeruginosainfectionversusin patients without (63.6%versus28.0%, p<0.001). In the ten patients who newly developed BAD the MRI global score increased from 15.1±7.8 before to 22.0±5.4 at first detection of BAD (p<0.05). Youden indices for presence of BAD were 0.57 for age (cut-off 11.2 y), 0.65 for ppFEV1(cut-off 74.2%), and 0.62 for MRI global score (cut-off 15.5) (p<0.001).ConclusionMRI detects BAD in patients with CF without radiation exposure. Onset of BAD is associated with increased MRI scores, worse lung function, and chronicP. aeruginosainfection, and may serve as a marker of disease severity.

Magnetic Resonance Imaging of Lung Perfusion

“…A broad range of patients with different lung diseases was evaluated using DCE-MRI, including CF, COPD, COVID-19 interstitial lung disease, and pulmonary hypertension. 11,14,22,[46][47][48][49] The technical validation of the DCE-MRI perfusion parameters included the assessment of its reproducibility, correlations with each other, correlations with perfusion parameters obtained using contrast-free techniques or hyperpolarized gas MRI, correlations with perfusion parameters using H 2 15 O positron emission tomography (PET) and single photon emission tomography (SPECT). 17,[20][21][22]29,39,43,46,50 The clinical validation of DCE-MRI perfusion parameters focused on the evaluation as disease progression marker (through the comparison with clinical parameters) and as response to treatment marker (evaluation in studies with investigational medicinal products).…”

“…[53][54][55] Apart from assessing pulmonary perfusion, DCE-MRI is able to detect bronchial artery dilatation induced by chronic inflammation and hypoxia in CF, which is associated with a decrease in the CF-MRI score. 11 In this context, the presence of bronchial artery dilatation may indicate irreversible perfusion alterations in the lungs, but this has not been further investigated to date. In addition, dual bronchodilation with indacaterol/glycopyrronium significantly improved pulmonary perfusion compared to placebo in 62 hyperinflated patients with COPD.…”

“…In chronic lung diseases, hypertrophy may occur due to chronic regional hypoxia and inflammation, and lead to an increased inflow from the systemic circulation to more than 30% of the left heart's output in extreme conditions such as chronic‐thromboembolic pulmonary hypertension 10 . Blood inflow from bronchial arteries usually is neglected when imaging lung perfusion, and their role in pulmonary diseases is not sufficiently understood, yet both lung circulations may potentially be discerned by their distinct temporal pattern of blood arrival 11 …”

“…DCE‐MRI‐derived pulmonary perfusion parameters assessed visually or by computer algorithms were extensively validated (clinical and technical validation) in the last years. A broad range of patients with different lung diseases was evaluated using DCE‐MRI, including CF, COPD, COVID‐19 interstitial lung disease, and pulmonary hypertension 11,14,22,46–49 . The technical validation of the DCE‐MRI perfusion parameters included the assessment of its reproducibility, correlations with each other, correlations with perfusion parameters obtained using contrast‐free techniques or hyperpolarized gas MRI, correlations with perfusion parameters using H 2 15 O positron emission tomography (PET) and single photon emission tomography (SPECT) 17,20–22,29,39,43,46,50 .…”

“…10 Blood inflow from bronchial arteries usually is neglected when imaging lung perfusion, and their role in pulmonary diseases is not sufficiently understood, yet both lung circulations may potentially be discerned by their distinct temporal pattern of blood arrival. 11 The following review aims to summarize MRI techniques based on either intrinsic properties of the lung signal (non-contrast-enhanced techniques) or on application of Gadolinium-based contrast agents (GBCA) (contrastenhanced techniques) for visualization and quantification of lung perfusion in different disease states.…”