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

Patricia Leutz-Schmidt; Daiva-Elzbieta Optazaite; Olaf Sommerburg; Monika Eichinger; Sabine Wege; Eva Steinke; Simon Y Graeber; Michael U Puderbach; Jens-Peter Schenk; Abdulsattar Alrajab; Simon M F Triphan; Hans-Ulrich Kauczor; Mirjam Stahl; Marcus A Mall; Mark O Wielpütz

doi:10.1183/23120541.00473-2022

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

ERJ Open Res. 2023 Mar 27;9(2):00473-2022. doi: 10.1183/23120541.00473-2022. eCollection 2023 Mar.

Authors

Patricia Leutz-Schmidt^{1

2

3}, Daiva-Elzbieta Optazaite^{1

2

3}, Olaf Sommerburg^{2

4

5}, Monika Eichinger^{1

2

3}, Sabine Wege⁶, Eva Steinke^{7

8

9}, Simon Y Graeber^{7

8

9}, Michael U Puderbach^{1

2

3

10}, Jens-Peter Schenk¹¹, Abdulsattar Alrajab¹¹, Simon M F Triphan^{1

2

3}, Hans-Ulrich Kauczor^{1

2

3}, Mirjam Stahl^{7

8

9}, Marcus A Mall^{7

8

9

12}, Mark O Wielpütz^{1

2

3

12}

Affiliations

¹ Diagnostic and Interventional Radiology, Subdivision of Pulmonary Imaging, University Hospital of Heidelberg, Heidelberg, Germany.
² Translational Lung Research Center Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany.
³ Department of Diagnostic and Interventional Radiology with Nuclear Medicine, Thoraxklinik at the University Hospital of Heidelberg, Heidelberg, Germany.
⁴ Division of Pediatric Pulmonology and Allergy, and Cystic Fibrosis Center, Department of Pediatrics, University of Heidelberg, Heidelberg, Germany.
⁵ Department of Translational Pulmonology, University Hospital Heidelberg, Heidelberg, Germany.
⁶ Department of Pulmonology and Respiratory Medicine, Thoraxklinik at the University Hospital of Heidelberg, Heidelberg, Germany.
⁷ Department of Pediatric Respiratory Medicine, Immunology and Intensive Care Medicine, Charité - Universitätsmedizin Berlin, Berlin, Germany.
⁸ German Center for Lung Research (DZL) associated partner site, Berlin, Germany.
⁹ Berlin Institute of Health at Charité, Berlin, Germany.
¹⁰ Department of Diagnostic and Interventional Radiology, Hufeland Hospital, Bad Langensalza, Germany.
¹¹ Diagnostic and Interventional Radiology, Subdivision of Pediatric Radiology, University Hospital of Heidelberg, Heidelberg, Germany.
¹² These authors contributed equally.

Abstract

Background: Bronchial 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).

Methods: 188 CF patients (mean±sd age 13.8±10.6 years, range 1.1-55.2 years) underwent annual chest MRI (median three exams, range one to six exams), contributing a total of 485 MRI exams including perfusion MRI. Presence of BAD was evaluated by two radiologists in consensus. Disease severity was assessed using the validated MRI scoring system and spirometry (forced expiratory volume in 1 s (FEV₁) % pred).

Results: MRI demonstrated BAD in 71 (37.8%) CF patients consistently from the first available exam and a further 10 (5.3%) patients first developed BAD during surveillance. Mean MRI global score in patients with BAD was 24.5±8.3 compared with 11.8±7.0 in patients without BAD (p<0.001) and FEV₁ % pred was lower in patients with BAD compared with patients without BAD (60.8% versus 82.0%; p<0.001). BAD was more prevalent in patients with chronic Pseudomonas aeruginosa infection versus in patients without infection (63.6% versus 28.0%; p<0.001). In the 10 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 the presence of BAD were 0.57 for age (cut-off 11.2 years), 0.65 for FEV₁ % pred (cut-off 74.2%) and 0.62 for MRI global score (cut-off 15.5) (p<0.001).

Conclusions: MRI detects BAD in patients with CF without radiation exposure. Onset of BAD is associated with increased MRI scores, worse lung function and chronic P. aeruginosa infection, and may serve as a marker of disease severity.