Kistik Fibrozis Hastalarında Akciğer Tutulumunun Manyetik Rezonans Görüntüleme ile Değerlendirilmesi

Abstract

Amaç: Kistik Fibrozis (KF) hastalığında pulmoner yapısal değişikliklerin erken dönemde noninvaziv olarak gösterilmesi ile tedaviye daha erken başlanarak ve mevcut tedavi gözden geçirilerek komplikasyonların önüne geçilebilir. Erken dönem pulmoner yapısal değişiklikleri, pulmoner alevlenme öncesi ve sonrası tedavi yanıtını göstermede bilgisayarlı toraks tomografisi (BT) ile beraber mediastinal manyetik rezonans görüntüleme (MRG) kullanılmaya başlanmıştır. Bu çalışmanın amacı, kliniğimizde KF ile takip edilen ve kliniği stabil hastalarda eş zamanlı olarak toraks BT ve MRG çekilen hastalardaki radyolojik bulguların birbirleri ile karşılaştırılması ve hastaların bazı klinik ve laboratuar bulguları ile korelasyon yapılarak; takiplerinde uygun yöntemle görüntüleme yapmaktır.

Gereç ve Yöntemler: Ağustos 2018 - Şubat 2019 tarihleri arasında, hastanemiz Çocuk Göğüs Hastalıkları Kliniği’nde KF tanısı ile takip edilmekte olan, klinik olarak stabil 14 hastanın aynı gün çekilen BT ve MRG’leri geriye dönük olarak Helbich ve Eichinger skorlama sistemine göre incelendi. Hastaların demografik verileri, klinik ve laboratuar bulguları kronik kolonizasyon durumu ve genetik mutasyonları kaydedildi.

Bulgular: KF tanısı ile takip edilmekte olan, pulmoner alevlenme şikayeti olmayan 14 hastanın aynı gün çekilen BT ve MRG’leri geriye dönük olarak değerlendirildi. BT ve MRG bulguları karşılaştırıldığında Helbich skorlama sistemine göre sadece mozaik atenüasyon paterninde BT ile daha iyi tanımlandığı gösterildi (p = 0.003). Helbich skorlama sistemine göre BT için ortalama skor 6.6 (1-17), MRG için 4.7 (0-15)’di. Eichinger skorlamasına göre MRG için ortalama skor 3 (0-16)’dı.Hastaların klinik ve demografik bulguları karşılaştırıldığında Phe508del homozigot mutasyonu olup P.aeruginosa ile kronik kolonize olan hastaların BT ve MRG skorları diğer hastalara göre anlamlı olarak daha yüksekti (p = 0.002).

Sonuç: KF hastalarında pulmoner etkilenmeyi göstermek için mediastinal MRG toraks BT kadar güvenilir; radyasyon içermediği için de tercih edilebilir bir yöntemdir. Mozaik atenüasyon gibi erken dönem akciğer bulgularının değerlendirilmesinde ise BT ile beraber kullanılabilir. Yakın zamanda KF hastalarındaki akciğer bulgularını göstermede deneyimli personel ve yeni çekim teknikleri ile MRG altın standart haline gelebilir.

Keywords

• Kistik fibrozis
• bilgisayarlı tomografi
• manyetik rezonans görüntüleme
• pulmoner tutulum

The Evaluation of Lung Involvement in Patients with Cystic Fibrosis By Using Mediastinal Magnetic Resonance Imaging

Abstract

Objective: In Cystic Fibrosis (CF) disease, complications can be avoided by noninvasively demonstrating pulmonary structural changes in the early period by starting treatment earlier and reviewing the current treatment. Computed thoracic tomography (CT) and mediastinal magnetic resonance imaging (MRI) have been used to show early pulmonary structural changes and treatment response before and after pulmonary exacerbation.The aim of this study is to compare the CT and MRI findings of CF patients with some clinical and laboratory findings, and to perform imaging with an appropriate method in their follow-up.

Material and Methods: CT and MRIs of 14 clinically stable CF patients August 2018 and February 2019 were retrospectively analyzed according to the Helbich and Eichinger scoring system. Patient’s laboratory findings, chronic colonization status and genetic mutations were recorded.

Results: According to Helbich scoring, the mean score in CT was 6.6 (score range 1-17), while MRI was 4.7 (score range 0-15). The mean score in the MRI was 3 (score range 0-16) according to the Eichinger score. There was a statistically significant difference between CT and MRI findings according to Helbich scoring (p=0.003). CT was superior to MRI in demonstrating mosaic attenuation. Four patients who had Phe508del homozygous mutation chronic colonised with p.aeruginosa and had higher CT and MRI scores than rest of them (p=0.002).

Conclusion: Mediastinal MRI is as reliable as thoracic CT; to show pulmonary involvement in CF patients and can be preferred to reduce radiation damage. MRI can be used together with CT in the evaluation of early lung findings such as mosaic attenuation. Recently, MRI may become the gold standard with experienced staff and new imaging techniques in demonstrating lung findings in CF patients.

Keywords

• Cystic Fibrosis
• Computed Tomography
• Magnetic Resonance İmaging
• Lung İnvolvement

References

1. 1. Ratjen F, Bell SC, Rowe SM, Goss CH, Quittner AL, Bush A. Cystic fibrosis. Nat Rev Dis Primers. 2015 May 14;1:15010.
2. 2. Paranjape SM, Mogayzel PJ Jr. Cystic fibrosis. Pediatr Rev. 2014 May;35(5):194-205.
3. 3. Horsley A. Book review: Hodson and Geddes’ Cystic Fibrosis. Breathe 2016; 12: 91–92.
4. 4.Cystic Fibrosis Lung Disease: An OvervieW Nelson L Turcios Respiratory Care Feb 2020, 65 (2) 233-251
5. 5. Kołodziej M, de Veer MJ, Cholewa M, Egan GF, Thompson BR. Lung function imaging methods in Cystic Fibrosis pulmonary disease. Respir Res. 2017 May 17;18(1):96.
6. 6. Villanueva G, Marceniuk G, Murphy MS, Walshaw M, Cosulich R; Guideline Committee. Diagnosis and management of cystic fibrosis: summary of NICE guidance. BMJ. 2017 Oct 26;359:j4574
7. 7. Newbegin K, Pilkington K, Shanthikumar S, Ranganathan S. Clinical utility of surveillance computed tomography scans in infants with cystic fibrosis. Pediatr Pulmonol. 2018 Oct;53(10):1387-1390.
8. 8. Sanders DB, Li Z, Brody AS. Chest computed tomography predicts the frequency of pulmonary exacerbations in children with cystic fibrosis. Ann Am Thorac Soc. 2015 Jan;12(1):64-9.

9. 9. .Wielpütz MO, Puderbach M, Kopp-Schneider A, Stahl M, Fritzsching E, Sommerburg O. et al. . 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 Apr 15;189(8):956-65.
10. 10. Theilmann RJ, Darquenne C, Elliott AR, Bailey BA, Conrad DJ. Characterizing Lung Disease in Cystic Fibrosis with Magnetic Resonance Imaging and Airway Physiology. PLoS One. 2016 Jun 23;11(6):e0157177.
11. 11. Anjorin A, Schmidt H, Posselt HG, Smaczny C, Ackermann H, Deimling M et al. Comparative evaluation of chest radiography, low-field MRI, the Shwachman-Kulczycki score and pulmonary function tests in patients with cystic fibrosis. Eur Radiol. 2008 Jun;18(6):1153-61.
12. 12. Thia LP, Calder A, Stocks J, Bush A, Owens CM, Wallis C et al. London Cystic Fibrosis Collaboration. Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age? Thorax. 2014 Apr;69(4):320-7.
13. 13. Loeve M, van Hal PT, Robinson P, de Jong PA, Lequin MH, Hop WC et al. The spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease. Thorax. 2009 Oct;64(10):876-82.
14. 14. Loeve M, Hop WC, de Bruijne M, van Hal PT, Robinson P, Aitken ML et al. Computed Tomography Cystic Fibrosis Survival Study Group. Chest computed tomography scores are predictive of survival in patients with cystic fibrosis awaiting lung transplantation. Am J Respir Crit Care Med. 2012 May 15;185(10):1096-103.
15. 15. Brody AS, Sucharew H, Campbell JD, Millard SP, Molina PL, Klein JS, Quan J. Computed tomography correlates with pulmonary exacerbations in children with cystic fibrosis. Am J Respir Crit Care Med. 2005 Nov 1;172(9):1128-32.
16. 16. Sanders DB, Li Z, Brody AS. Chest computed tomography predicts the frequency of pulmonary exacerbations in children with cystic fibrosis. Ann Am Thorac Soc. 2015 Jan;12(1):64-9.
17. 17. Jacobsen LE, Houston CS, Habbick BF, Genereux GP, Howie JL. Cystic fibrosis: a comparison of computed tomography and plain chest radiographs. Can Assoc Radiol J. 1986 Mar;37(1):17-21.
18. 18. .Stick SM, Brennan S, Murray C, Douglas T, von Ungern-Sternberg BS, Garratt LW et al. Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF). Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. J Pediatr. 2009 Nov;155(5):623-8.e1.
19. 19. Mott LS, Park J, Murray CP, et al. Progression of early structural lung disease in young children with cystic fibrosis assessed using CT. Thorax. 2012 Jun;67(6):509-516.
20. 20. Wainwright CE, Vidmar S, Armstrong DS, Byrnes CA, Carlin JB, Cheney J et al. ACFBAL Study Investigators. Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: a randomized trial. JAMA. 2011 Jul 13;306(2):163-71.
21. 21.Tepper LA, Utens EM, Caudri D, Bos AC, Gonzalez-Graniel K, Duivenvoorden HJ, van der Wiel EC, Quittner AL, Tiddens HA. Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis. Eur Respir J. 2013 Aug;42(2):371-9.
22. 22. Yang C, Montgomery M. Dornase alfa for cystic fibrosis. Cochrane Database Syst Rev. 2018 Sep 6;9(9):CD001127.
23. 23. Kerem E, Conway S, Elborn S, Heijerman H; Consensus Committee. Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros. 2005 Mar;4(1):7-26.
24. 24. Hansell DM, Strickland B. High-resolution computed tomography in pulmonary cystic fibrosis. Br J Radiol. 1989 Jan;62(733):1-5.
25. 25 Hansell DM. Bronchiectasis. Radiol Clin North Am. 1998 Jan;36(1):107-28.
26. 26. Arakawa H, Webb WR. Air trapping on expiratory high-resolution CT scans in the absence of inspiratory scan abnormalities: correlation with pulmonary function tests and differential diagnosis. AJR Am J Roentgenol. 1998 May;170(5):1349-53.
27. 27 Gilchrist FJ, Buka R, Jones M, Ho SA, Lenney W, Carroll WD. Clinical indications and scanning protocols for chest CT in children with cystic fibrosis: a survey of UK tertiary centres. BMJ Paediatr Open. 2018 Oct 27;2(1):e000367.
28. 28. de González AB, Kim KP, Samet JM. Radiation-induced cancer risk from annual computed tomography for patients with cystic fibrosis. Am J Respir Crit Care Med. 2007 Nov 15;176(10):970-3.
29. 29. Ferris H, Twomey M, Moloney F, O'Neill SB, Murphy K, O'Connor OJ, Maher M. Computed tomography dose optimisation in cystic fibrosis: A review. World J Radiol. 2016 Apr 28;8(4):331-41.
30. 30. Roach DJ, Crémillieux Y, Fleck RJ, Brody AS, Serai SD, Szczesniak RD et al. Ultrashort Echo-Time Magnetic Resonance Imaging Is a Sensitive Method for the Evaluation of Early Cystic Fibrosis Lung Disease. Ann Am Thorac Soc. 2016 Nov;13(11):1923-1931. 31. Walkup LL, Thomen RP, Akinyi TG, Watters E, Ruppert K, Clancy JP et al. Feasibility, tolerability and safety of pediatric hyperpolarized 129Xe magnetic resonance imaging in healthy volunteers and children with cystic fibrosis. Pediatr Radiol. 2016 Nov;46(12):1651-1662.
31. 32. Thomen RP, Walkup LL, Roach DJ, Cleveland ZI, Clancy JP, Woods JC. Hyperpolarized 129Xe for investigation of mild cystic fibrosis lung disease in pediatric patients. J Cyst Fibros. 2017 Mar;16(2):275-282. 33. Sileo C, Corvol H, Boelle PY, Blondiaux E, Clement A, Ducou Le Pointe H. HRCT and MRI of the lung in children with cystic fibrosis: comparison of different scoring systems. J Cyst Fibros. 2014 Mar;13(2):198-204.
32. 34. Wielpütz MO, Puderbach M, Kopp-Schneider A, Stahl M, Fritzsching E, Sommerburg O, Ley S, Sumkauskaite M, Biederer J, Kauczor HU, Eichinger M, Mall MA. 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 Apr 15;189(8):956-65.
33. 35. Failo R, Wielopolski PA, Tiddens HA, Hop WC, Mucelli RP, Lequin MH. Lung morphology assessment using MRI: a robust ultra-short TR/TE 2D steady state free precession sequence used in cystic fibrosis patients. Magn Reson Med. 2009 Feb;61(2):299-306.
34. 36. Stahl M, Wielpütz MO, Graeber SY, Joachim C, Sommerburg O, Kauczor H U et l. 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. 2017 Feb 1;195(3):349-359.
35. 37. Couch MJ, Munidasa S, Rayment JH, Voskrebenzev A, Seethamraju RT, Vogel-Claussen J et al. Comparison of Functional Free-Breathing Pulmonary 1H and Hyperpolarized 129Xe Magnetic Resonance Imaging in Pediatric Cystic Fibrosis. Acad Radiol. 2020 Jun 10:S1076-6332(20)30284-1.
36. 38. Couch MJ, Thomen R, Kanhere N, Hu R, Ratjen F, Woods J, Santyr G. A two-center analysis of hyperpolarized 129Xe lung MRI in stable pediatric cystic fibrosis: Potential as a biomarker for multi-site trials. J Cyst Fibros. 2019 Sep;18(5):728-733
37. 39. Thia LP, Calder A, Stocks J, Bush A, Owens CM, Wallis C et al. London Cystic Fibrosis Collaboration. Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age? Thorax. 2014 Apr;69(4):320-7.
38. 40. Ranganathan SC, Hall GL, Sly PD, Stick SM, Douglas TA; Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST-CF). Early Lung Disease in Infants and Preschool Children with Cystic Fibrosis. What Have We Learned and What Should We Do about It? Am J Respir Crit Care Med. 2017 Jun 15;195(12):1567-1575.
39. 41. Long FR, Williams RS, Castile RG. Structural airway abnormalities in infants and young children with cystic fibrosis. J Pediatr. 2004 Feb;144(2):154-61.
40. 42. Davis SD, Fordham LA, Brody AS, Noah TL, Retsch-Bogart GZ, Qaqish BF, et al. Computed tomography reflects lower airway inflammation and tracks changes in early cystic fibrosis. Am J Respir Crit Care Med. 2007 May 1;175(9):943-50.
41. 43 Amin R, Charron M, Grinblat L, Shammas A, Grasemann H, Graniel et al. Cystic fibrosis: detecting changes in airway inflammation with FDG PET/CT. Radiology. 2012 Sep;264(3):868-75
42. 44. Sly PD, Gangell CL, Chen L, Ware RS, Ranganathan S, Mott LS,et al. AREST CF Investigators. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med. 2013 May 23;368(21):1963-70.