Radiographic progression and survival of the different HRCT patterns of idiopathic pulmonary fibrosis

Main Article Content

Marco Mura
Carlotta Rellini
Nada Taha
Francesco Paolo Sbordone
flavia rufi
francesca montesanto
Roberto Floris
Maurizio Zompatori
Gianluigi Sergiacomi

Keywords

usual interstitial pneumonia, idiopathic pulmonary fibrosis, high-resolution chest CT scan, fibrosis score, progression

Abstract

Introduction. Idiopathic pulmonary fibrosis (IPF) is a chronic disease with typical HRCT patterns, but biopsy can show IPF in patients with atypical patterns. It is unknown how progression pattern varies among different radiographic presentations of IPF. The aim of this study was to investigate the longitudinal radiographic evolution of typical and non-typical patterns and to compare progression of disease among different presentation HRCT patterns.


Materials and Methods. One-hundred-twenty-three consecutive patients diagnosed with IPF in 2 tertiary referral hospitals were included in the study. Longitudinal evolution of non-typical patterns was considered. The HRCT visual fibrosis score was used as a reliable evaluation tool of disease progression. All HRCTs were scored by 2 senior chest radiologists with ILD expertise. The primary endpoint was the evolution of the presentation pattern to probable or typical. The secondary endpoint was lung transplant (LTx)-free survival from the time of diagnosis.


Results. Average interval between HRCTs was 16±5 months; average follow-up after the 2nd HRCT was 17±11 months. Four out of 45 (8.9%) patients with probable pattern “evolved” to a typical pattern of IPF, while 5 out of 31 (16.1%) with indeterminate/alternative pattern “evolved” to probable pattern. An average HRCT fibrosis score increase of 9±11% was observed with typical (n=49), 6±5% with probable (n=43) and 7±8% (n=31) with indeterminate/alternative presentation pattern. LTx-free survival did not show differences related to presentation HRCT patterns.

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References

1. Raghu G, RemyJardin M, Myers JL, et al. American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society: diagnosis of idiopathic pulmonary fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2018, 1;198(5):e44e68.
2. Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med 2018 Feb;6(2):138-153.
3. Lynch DA, Godwin JD, Safrin S, Starko KM, Hormel P, Brown KK, Raghu G, King TE Jr,
Bradford WZ, Schwartz DA, Richard Webb W, Idiopathic Pulmonary Fibrosis Study Group:
High resolution computed tomography in idiopathic pulmonary fibrosis, diagnosis and prognosis. Am J Respir Crit Care Med. 2005, 15;172(4):48893.
4. Yoo DK, Zompatori M, Barrile A, Rossi G, D'Amato D, Sergiacomi G, Rogliani P, Mura M: Associated pulmonary hypertension is an independent contributor to exercise intolerance in chronic fibrosing interstitial pneumonias. Respiration. 2018;96(6):543551.
5. Salisbury ML, Lynch DA, van Beek EJ, Kazerooni EA, Guo J, Xia M, Murray S, Anstrom
KJ, Yow E, Martinez FJ, Hoffman EA, Flaherty KR, IPFnet Investigators: idiopathic pulmonary fibrosis: the association between the adaptive multiple features method and fibrosis outcomes. Am J Respir Crit. Care Med. 2017, 1;195(7):921929.
6. Park HJ, Lee SM, Song JW, Lee SM, Oh SY, Kim N, Seo JB: Texture based automated quantitative assessment of regional patterns on initial CT in patients with idiopathic pulmonary fibrosis, relationship to decline in forced vital capacity. AJR Am J Roentgenology. 2016, 207(5):976983.
7. Taha N, D’Amato D, Hosein K, Ranalli T, Sergiacomi G, Zompatori M, Mura M. Longitudinal functional changes with clinically significant radiographic progression in idiopathic pulmonary fibrosis: are we following the right parameters? Respir Res 2020; 21:119.
8. Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011 Feb 15;183(4):431-40. doi: 10.1164/rccm.201006-0894CI. Epub 2010 Oct 8. PMID: 20935110.
9. Flaherty KR, Mumford JA, Murray S, Kazerooni EA, Gross BH, Colby TV, Travis WD, Flint A, Toews GB, Lynch JP 3rd, Martinez FJ: Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2003,
1;168(5):5438.
10. Sverzellati N, Silva M, Seletti V, Galeone C, Palmucci S, Piciucchi S, Vancheri C, Poletti V, Tomassetti S, Karwoski R, Bartholmai BJ: Stratification of longterm outcome in stable idiopathic pulmonary fibrosis by combining longitudinal computed tomography and forced vital capacity. Eur Radiol. 2020, Jan 31.
11. Yoo DK, Zompatori M, Barrile A, Rossi G, D'Amato D, Sergiacomi G, Rogliani P, Mura M: Associated Pulmonary Hypertension Is an Independent Contributor to Exercise Intolerance in Chronic Fibrosing Interstitial Pneumonias. Respiration 2018; 96: 543-551.
12. Watadani T, Sakai F, Johkoh T, Noma S, Akira M, Fujimoto K, Bankier AA, Lee KS, Müller NL, Song JW, Park JS, Lynch DA, Hansell DM, RemyJardin M, Franquet T, Sugiyama Y: Interobserver variability in the CT assessment of honeycombing in the lungs. Radiology. 2013,
266(3):93644.
13. Frailty and aging-associated syndromes in lung transplant candidates and recipients Am J Transplant 2021 Jun;21(6):2018-2024.
14. Mura M, Porretta MA, Bargagli E, Sergiacomi G, Zompatori M, Sverzellati N, Taglieri A,
Mezzasalma F, Rottoli P, Saltini C, Rogliani P: Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3 year prospective study. Eur Respir J. 2012, 40(1):1019.
15. Sgalla G, Biffi A, Richeldi L: Idiopathic pulmonary fibrosis: diagnosis, epidemiology and natural history. Respirology 2016 Apr;21(3):427-37.
16. Jonathan H Chung , David A Lynch:The Value of a Multidisciplinary Approach to the Diagnosis of Usual Interstitial Pneumonitis and Idiopathic Pulmonary Fibrosis: Radiology, Pathology, and Clinical Correlation. AJR Am J Roentgenol 2016 Mar;206(3):463-71

17. Gian Marco Manzetti, Karishma Hosein, Matthew J Cecchini, Keith Kwan, Mohamed Abdelrazek, Maurizio Zompatori , Paola Rogliani , Marco Mura : Validation of the risk stratification score in idiopathic pulmonary fibrosis: study protocol of a prospective, multi-centre, observational, 3-year clinical trial. BMC Pulm Med 2021 Dec 4;21(1):396.
18. Walsh SL, Calandriello L, Sverzellati N, Wells AU, Hansell DM, UIP Observer Consort: Interobserver agreement for the ATS/ERS/JRS/ALAT criteria for a UIP pattern on CT. Thorax.
2016, 71(1):4551.
19. Jonhatan H. C, Ashis C, David A. L. CT scan findings of probable usual interstitial pneumonitis have a high predictive value for histologic usual interstitial pneumonitis. Chest 2015 Feb;147(2):450-459.
20. Yaghiashi K, Hucklebarry J, A Lynch D. Radiologic-pathologic discordance in biopsy proven usual interstitial pneumonia. Eur Respir J. 2016 Apr;47(4):1189-97.
21. Flaherty KR, King TE Jr, Raghu G, Lynch JP 3rd, Colby TV, Travis WD, Gross BH, Kazerooni EA, Toews GB, Long Q, Murray S, Lama VN, Gay SE, Martinez FJ: Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med. 2004, 15;170(8):904-10.
22. Sverzellati N, Lynch DA, Hansell DM, et al. American Thoracic Society-European Respiratory Society Classification of the Idiopathic Interstitial Pneumonias: Advances in Knowledge since 2002. Radiographics, Nov-Dec 2015;35(7):1849-71.
23. Baddini-Martinez J, Baldi BG, Costa CH, Jezler S, Lima MS, Rufino R. Update on diagnosis and treatment of idiopathic pulmonary fibrosis. J Bras Pneumol. 2015;41(5):454-66.
24. Mura M, Belmonte G, Fanti S, Pacilli AMF, Fasano L, Zompatori M, Schiavina M, Fabbri M. Does technetium-99m diethylenetriaminepentaacetate clearance predict the clinical course of idiopathic pulmonary fibrosis? Can Respir J. 2004 Oct;11(7):477-9.
25. Travis WD, Costabel U, Hansell DM, King Jr TE, Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonia. Am J Respir Crit Care Med. 2013;188(6),733-48.

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