Magnetic Resonance Imaging Detects Onset and Association with Lung Disease Severity of Bronchial Artery Dilatation in Cystic Fibrosis

Overview

Journal ERJ Open Res

Specialty Pulmonary Medicine

Date 2023 Apr 3

PMID 37009019

Authors

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 Wielputz

Affiliations

Soon will be listed here.

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 (FEV) % 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 (p0.001) and FEV % pred was lower in patients with BAD compared with patients without BAD (60.8% 82.0%; p<0.001). BAD was more prevalent in patients with chronic infection in patients without infection (63.6% 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 (p0.05). Youden indices for the presence of BAD were 0.57 for age (cut-off 11.2 years), 0.65 for FEV % pred (cut-off 74.2%) and 0.62 for MRI global score (cut-off 15.5) (p0.001).

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 infection, and may serve as a marker of disease severity.

References

Stahl M, Steinke E, Graeber S, Joachim C, Seitz C, Kauczor H . Magnetic Resonance Imaging Detects Progression of Lung Disease and Impact of Newborn Screening in Preschool Children with Cystic Fibrosis. Am J Respir Crit Care Med. 2021; 204(8):943-953. DOI: 10.1164/rccm.202102-0278OC. View

Wielputz M, Eichinger M, Wege S, Eberhardt R, Mall M, Kauczor H . Midterm Reproducibility of Chest Magnetic Resonance Imaging in Adults with Clinically Stable Cystic Fibrosis and Chronic Obstructive Pulmonary Disease. Am J Respir Crit Care Med. 2019; 200(1):103-107. DOI: 10.1164/rccm.201812-2356LE. View

Kuo W, Ciet P, Tiddens H, Zhang W, Guillerman R, van Straten M . Monitoring cystic fibrosis lung disease by computed tomography. Radiation risk in perspective. Am J Respir Crit Care Med. 2014; 189(11):1328-36. DOI: 10.1164/rccm.201311-2099CI. View

Antonelli M, Midulla F, Tancredi G, Salvatori F, Bonci E, Cimino G . Bronchial artery embolization for the management of nonmassive hemoptysis in cystic fibrosis. Chest. 2002; 121(3):796-801. DOI: 10.1378/chest.121.3.796. View

Dabo H, Gomes R, Marinho A, Madureira M, Paquete J, Morgado P . Bronchial artery embolisation in management of hemoptysis--A retrospective analysis in a tertiary university hospital. Rev Port Pneumol (2006). 2015; 22(1):34-8. DOI: 10.1016/j.rppnen.2015.09.001. View

Fruchter O, Schneer S, Rusanov V, Belenky A, Kramer M . Bronchial artery embolization for massive hemoptysis: long-term follow-up. Asian Cardiovasc Thorac Ann. 2014; 23(1):55-60. DOI: 10.1177/0218492314544310. View

Wielputz M, Puderbach M, Kopp-Schneider A, Stahl M, Fritzsching E, Sommerburg O . Magnetic resonance imaging detects changes in structure and perfusion, and response to therapy in early cystic fibrosis lung disease. Am J Respir Crit Care Med. 2014; 189(8):956-65. DOI: 10.1164/rccm.201309-1659OC. View

CAULDWELL E, SIEKERT R . The bronchial arteries; an anatomic study of 150 human cadavers. Surg Gynecol Obstet. 1948; 86(4):395-412. View

Stahl M, Wielputz M, Graeber S, Joachim C, Sommerburg O, Kauczor H . Comparison of Lung Clearance Index and Magnetic Resonance Imaging for Assessment of Lung Disease in Children with Cystic Fibrosis. Am J Respir Crit Care Med. 2016; 195(3):349-359. DOI: 10.1164/rccm.201604-0893OC. View

10.

Mall M, Mayer-Hamblett N, Rowe S . Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications. Am J Respir Crit Care Med. 2019; 201(10):1193-1208. PMC: 7233349. DOI: 10.1164/rccm.201910-1943SO. View

11.

Barben J, Ditchfield M, Carlin J, Robertson C, Robinson P, Olinsky A . Major haemoptysis in children with cystic fibrosis: a 20-year retrospective study. J Cyst Fibros. 2004; 2(3):105-11. DOI: 10.1016/S1569-1993(03)00066-3. View

12.

Flume P, Mogayzel Jr P, Robinson K, Rosenblatt R, Quittell L, Marshall B . Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax. Am J Respir Crit Care Med. 2010; 182(3):298-306. DOI: 10.1164/rccm.201002-0157OC. View

13.

Ley-Zaporozhan J . [Thoracic imaging in cystic fibrosis : Radiography]. Radiologe. 2020; 60(9):802-812. DOI: 10.1007/s00117-020-00735-w. View

14.

Soares Pires F, Teixeira N, Coelho F, Damas C . Hemoptysis--etiology, evaluation and treatment in a university hospital. Rev Port Pneumol. 2011; 17(1):7-14. DOI: 10.1016/s2173-5115(11)70004-5. View

15.

Wielputz M, Eichinger M, Biederer J, Wege S, Stahl M, Sommerburg O . Imaging of Cystic Fibrosis Lung Disease and Clinical Interpretation. Rofo. 2016; 188(9):834-45. DOI: 10.1055/s-0042-104936. View

16.

Woods J, Wild J, Wielputz M, Clancy J, Hatabu H, Kauczor H . Current state of the art MRI for the longitudinal assessment of cystic fibrosis. J Magn Reson Imaging. 2019; 52(5):1306-1320. PMC: 7297663. DOI: 10.1002/jmri.27030. View

17.

Wucherpfennig L, Triphan S, Wege S, Kauczor H, Heussel C, Schmitt N . Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis. J Cyst Fibros. 2022; 21(6):1053-1060. DOI: 10.1016/j.jcf.2022.03.011. View

18.

Graeber S, Vitzthum C, Pallenberg S, Naehrlich L, Stahl M, Rohrbach A . Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two Alleles. Am J Respir Crit Care Med. 2021; 205(5):540-549. DOI: 10.1164/rccm.202110-2249OC. View

19.

Pontana F, Moureau D, Schmidt B, Duhamel A, Faivre J, Yasunaga K . CT pulmonary angiogram with 60% dose reduction: Influence of iterative reconstructions on image quality. Diagn Interv Imaging. 2015; 96(5):487-93. DOI: 10.1016/j.diii.2014.08.006. View

20.

Kuo W, Kemner-Van de Corput M, Perez-Rovira A, De Bruijne M, Fajac I, Tiddens H . Multicentre chest computed tomography standardisation in children and adolescents with cystic fibrosis: the way forward. Eur Respir J. 2016; 47(6):1706-17. DOI: 10.1183/13993003.01601-2015. View