Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease
Yıl: 2022 Cilt: 52 Sayı: 1 Sayfa Aralığı: 83 - 88 Metin Dili: İngilizce DOI: 10.3906/sag-2107-13 İndeks Tarihi: 13-06-2022
Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease
Öz: Background/aim: To explore the frequency and clinical associations of radiologic pleuroparenchymal fibroelastosis (PPFE) in patients
with systemic sclerosis-associated interstitial lung disease (SSc-ILD).
Materials and methods: In this single-center retrospective study, high resolution computed tomography (HRCT) images of 105 patients
with SSc-ILD were examined for the presence of PPFE. Demographic, clinical, laboratory, and pulmonary function test (PFT) data of
patients with and without PPFE were compared.
Results: PPFE was detected in 19 (18.1%) patients (‘definite PPFE’ in 13 and ‘consistent with PPFE’ in 6 patients). Patients with PPFE
had higher age and longer disease duration than PPFE (-) patients (p < 0.05 for both). Radiologic usual interstitial pneumoniae (UIP)
pattern was more frequent (26.3% vs. 4.7%, p = 0.01) and median force vital capacity (FVC) was lower in patients with PPFE (64% vs.
82%, p = 0.005). Spontaneous pneumothorax developed in one patient with PPFE. More deaths occured in PPFE (+) group during
follow-up (31% vs. 11%, p = 0.04).
Conclusion: PPFE on HRCT is not uncommon in SSc-ILD and is associated with radiologic UIP pattern and worse lung functions. Further studies are needed to elucidate the prognostic value of PPFE in SSc-ILD.
Anahtar Kelime: Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
- 1. Denton CP, Wells AU, Coghlan JG. Major lung complications of systemic sclerosis. Nature Reviews Rheumatology 2018;14(9):511-527. doi: 10.1038/s41584-018-0062-0
- 2. Khanna D, Nagaraja V, Tseng CH, Abtin F, Suh R et al. Predictors of lung function decline in scleroderma-related interstitial lung disease based on high-resolution computed tomography: implications for cohort enrichment in systemic sclerosis-associated interstitial lung disease trials. Arthritis Research & Therapy 2015;23; 17: 372. doi: 10.1186/s13075- 015-0872-2
- 3. Goldin JG, Lynch DA, Strollo DC, Suh RD, Schraufnagel DE et al. High-resolution CT scan findings in patients with symptomatic scleroderma-related interstitial lung disease. Chest 2008; 134:358. doi: 10.1378/chest.07-2444
- 4. Bouros D, Wells AU, Nicholson AG, Colby TV, Polychronopoulos V et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. American Journal of Respiratory and Critical Care Medicine 2002;165(12):1581-6. doi: 10.1164/ rccm.2106012
- 5. Cheng SK, Chuah KL. Pleuroparenchymal fibroelastosis of the lung: a review. Archives of Pathology & Laboratory Medicine 2016;140(8):849-53. doi: 10.5858/arpa.2015-0166-RS
- 6. Travis WD, Costabel U, Hansell DM, King ET, Lynch DA et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. American Journal of Respiratory and Critical Care Medicine 2013;188(6):733–748.doi: 10.1164/rccm.201308-1483ST
- 7. Nakatani T, Arai T, Kitaichi M, Akira M, Tachibana K. Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? European Respiratory Journal 2015;45(4):1183– 1186. doi: 10.1183/09031936.00214714
- 8. Bonifaz iM, Sverzellati N, Negri E, Jacob J, Egashira R et al. Pleuroparenchymal fibroelastosis in systemic sclerosis: prevalence and prognostic impact. European Respiratory Journal 2020;9;56(1):1902135. doi: 10.1183/13993003.02135- 2019
- 9. Bargagli E, Mazzei MA, Orlandi M, Gentili F, Bellisai F et al. Pleuroparenchymal fibroelastosis in patients affected by systemic sclerosis: What should the rheumatologist do? Medicine (Baltimore) 2019: 98(26):e16086. doi: 10.1097/ MD.0000000000016086
- 10. Van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Annals of the Rheumatic Diseases 2013:72(11):1747-55. doi: 10.1136/ annrheumdis-2013-204424
- 11. Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). European Heart Journal 2016; 1;37(1):67-119. doi: 10.1093/eurheartj/ehv317
- 12. Reddy TL, Tominaga M, Hansell DM, von der Thusen J, Rassl D et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. European Respiratory Journal 2012; 40: 377-85. doi: 10.1183/09031936.00165111
- 13. Lynch DA, Sverzellati N, Travis WD, Brown KK, Colby TV et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. The Lancet Respiratory Medicine 2018; 6(2):138-153. doi: 10.1016/S2213- 2600(17)30433-2
- 14. Kligerman SJ, Groshong S, Brown KK, Lynch DA. Non specific interstitial pneumonia: radiologic, clinical, and pathologic considerations. Radiographics 2009;29(1):73-87. doi: 10.1148/ rg.291085096
- 15. Goh NS, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ et al. Interstitial lung disease in systemic sclerosis: a simple staging system. American Journal of Respiratory and Critical Care Medicine 2008 1;177(11):1248-54. doi: 10.1164/rccm.200706- 877OC
- 16. Takahashi M, Fukuoka J, Nitta N, Takazakura R, Nagatani Y et al. Imaging of pulmonaryemphysema: A pictorialreview. Int J ChronObstructPulmonDis. 2008; 3(2): 193–204. doi: 10.2147/ copd.s2639.
- 17. Schraufnagel DE, Michel JC, Sheppard TJ, Saffold PC, George T et al. CT of the normal esophagus to define the normal air column and its extent and distribution. American Journal of Roentgenology 2008;191(3):748-52. doi: 10.2214/AJR.07.345
- 18. Enomoto Y, Nakamura Y, Colby TV, Johkoh T, Sumikawa H et al. Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease. PLoS One 2017;30;12(6):e0180283. doi: 10.1371/journal. pone.0180283
- 19. Oda T, Ogura T, Kitamura H, Hagiwara E, Baba T et al. Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis. Chest 2014;146: 1248–1255. doi: 10.1378/ chest.13-2866
- 20. Sugino K, Ono H, Shimizu H, Kurosawa T, Matsumoto K et al. Treatment with anti fibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia. ERJ Open Research 2021;1;7(1):00196-2020. doi: 10.1183/23120541.00196-2020
| APA | SARI A, Önder Ö, ARMAĞAN B, Bolek E, farisoğulları b, Bilgin E, Yardimci G, ARIYUREK O, Akdoğan A (2022). Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. , 83 - 88. 10.3906/sag-2107-13 |
| Chicago | SARI ALPER,Önder Ömer,ARMAĞAN Berkan,Bolek Ertugrul Cagri,farisoğulları bayram,Bilgin Emre,Yardimci Gozde Kubra,ARIYUREK Orhan Macit,Akdoğan Ali Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. (2022): 83 - 88. 10.3906/sag-2107-13 |
| MLA | SARI ALPER,Önder Ömer,ARMAĞAN Berkan,Bolek Ertugrul Cagri,farisoğulları bayram,Bilgin Emre,Yardimci Gozde Kubra,ARIYUREK Orhan Macit,Akdoğan Ali Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. , 2022, ss.83 - 88. 10.3906/sag-2107-13 |
| AMA | SARI A,Önder Ö,ARMAĞAN B,Bolek E,farisoğulları b,Bilgin E,Yardimci G,ARIYUREK O,Akdoğan A Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. . 2022; 83 - 88. 10.3906/sag-2107-13 |
| Vancouver | SARI A,Önder Ö,ARMAĞAN B,Bolek E,farisoğulları b,Bilgin E,Yardimci G,ARIYUREK O,Akdoğan A Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. . 2022; 83 - 88. 10.3906/sag-2107-13 |
| IEEE | SARI A,Önder Ö,ARMAĞAN B,Bolek E,farisoğulları b,Bilgin E,Yardimci G,ARIYUREK O,Akdoğan A "Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease." , ss.83 - 88, 2022. 10.3906/sag-2107-13 |
| ISNAD | SARI, ALPER vd. "Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease". (2022), 83-88. https://doi.org/10.3906/sag-2107-13 |
| APA | SARI A, Önder Ö, ARMAĞAN B, Bolek E, farisoğulları b, Bilgin E, Yardimci G, ARIYUREK O, Akdoğan A (2022). Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. Turkish Journal of Medical Sciences, 52(1), 83 - 88. 10.3906/sag-2107-13 |
| Chicago | SARI ALPER,Önder Ömer,ARMAĞAN Berkan,Bolek Ertugrul Cagri,farisoğulları bayram,Bilgin Emre,Yardimci Gozde Kubra,ARIYUREK Orhan Macit,Akdoğan Ali Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. Turkish Journal of Medical Sciences 52, no.1 (2022): 83 - 88. 10.3906/sag-2107-13 |
| MLA | SARI ALPER,Önder Ömer,ARMAĞAN Berkan,Bolek Ertugrul Cagri,farisoğulları bayram,Bilgin Emre,Yardimci Gozde Kubra,ARIYUREK Orhan Macit,Akdoğan Ali Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. Turkish Journal of Medical Sciences, vol.52, no.1, 2022, ss.83 - 88. 10.3906/sag-2107-13 |
| AMA | SARI A,Önder Ö,ARMAĞAN B,Bolek E,farisoğulları b,Bilgin E,Yardimci G,ARIYUREK O,Akdoğan A Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. Turkish Journal of Medical Sciences. 2022; 52(1): 83 - 88. 10.3906/sag-2107-13 |
| Vancouver | SARI A,Önder Ö,ARMAĞAN B,Bolek E,farisoğulları b,Bilgin E,Yardimci G,ARIYUREK O,Akdoğan A Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. Turkish Journal of Medical Sciences. 2022; 52(1): 83 - 88. 10.3906/sag-2107-13 |
| IEEE | SARI A,Önder Ö,ARMAĞAN B,Bolek E,farisoğulları b,Bilgin E,Yardimci G,ARIYUREK O,Akdoğan A "Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease." Turkish Journal of Medical Sciences, 52, ss.83 - 88, 2022. 10.3906/sag-2107-13 |
| ISNAD | SARI, ALPER vd. "Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease". Turkish Journal of Medical Sciences 52/1 (2022), 83-88. https://doi.org/10.3906/sag-2107-13 |
