Abstract
Objectives
To determine high-resolution computed tomography (HRCT) findings helpful in predicting mortality in patients with acute exacerbation of idiopathic pulmonary fibrosis (AEx-IPF).
Methods
Sixty patients with diagnosis of AEx-IPF were reviewed retrospectively. Two groups (two observers each) independently evaluated pattern, distribution, and extent of HRCT findings at presentation and calculated an HRCT score at AEx based on normal attenuation areas and extent of abnormalities, such as areas of ground-glass attenuation and/or consolidation with or without traction bronchiectasis or bronchiolectasis and areas of honeycombing. The correlation between the clinical data including the HRCT score and mortality (cause-specific survival) was evaluated using the univariate and multivariate Cox-regression analyses.
Results
Serum KL-6 level, PaCO2, and the HRCT score were statistically significant predictors on univariate analysis. Multivariate analysis revealed that the HRCT score was an independently significant predictor of outcome (hazard ratio, 1.13; 95% confidence interval, 1.06–1.19, P = 0.0002). The area under receiver operating characteristics curve for the HRCT score was statistically significant in the classification of survivors or nonsurvivors (0.944; P < 0.0001). Survival in patients with HRCT score ≥245 was worse than those with lower score (log-rank test, P < 0.0001).
Conclusion
The HRCT score at AEx is independently related to prognosis in patients with AEx-IPF.
Key Points
•High-Resolution Computed Tomography (HRCT) helps clinicians to assess patients with interstitial fibrosis.
•The main abnormalities of acute exacerbation are ground-glass opacification and consolidation.
•Extent of abnormalities on HRCT correlates with poor prognosis.
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References
American Thoracic Society/European Respiratory Society (2002) American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 165:277–304
Martinez FJ, Safrin S, Weycker D et al (2005) The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med 142:963–967
Kondoh Y, Taniguchi H, Kawabata Y, Yokoi T, Suzuki K, Takagi K (1993) Acute exacerbation in idiopathic pulmonary fibrosis: analysis of clinical and pathologic findings in three cases. Chest 103:1808–1812
Akira M, Hamada H, Sakatani M, Kobayashi C, Nishioka M, Yamamoto S (1997) CT findings during phase of accelerated deterioration in patients with idiopathic pulmonary fibrosis. AJR Am J Roentgenol 168:79–83
Ambrosini V, Cancellieri A, Chilosi M et al (2003) Acute exacerbation of idiopathic pulmonary fibrosis: report of a series. Eur Respir J 22:821–826
Taniguchi H, Kondoh Y (2004) Revised criteria for acute exacerbation of idiopathic pulmonary fibrosis. The Annual Report by Study Group of Ministry of Health and Welfare for Diffuse Lung Disease, pp 114–119
Parambil JG, Myers JL, Ryu JH (2005) Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest 128:3310–3315
Kim DS, Park JH, Park BK, Lee JS, Nicholson AG, Colby T (2006) Acute exacerbation of idiopathic pulmonary fibrosis: frequency and clinical features. Eur Respir J 27:143–150
Collard HR, Moore BB, Flaherty KR et al (2007) Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 176:636–643
Taniguchi H, Ebina M, Kondoh Y et al (2010) Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 35:821–829
Churg A, Müller NL, Silva CIS, Wright JL (2007) Acute exacerbation (acute lung injury of unknown cause) in UIP and other forms of fibrotic interstitial pneumonias. Am J Surg Pathol 31:277–284
Silva CIS, Müller NL, Fujimoto K et al (2007) Acute exacerbation of chronic interstitial pneumonia: high-resolution computed tomography and pathologic findings. J Thorac Imag 22:221–229
Song JW, Hong SB, Lim CM, Lim CM, Koh Y, Kim DS (2011) Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors, and outcome. Eur Respir J 37:356–363
Akira M, Kozuka T, Yamamoto S, Sakatani M (2008) Computed tomographic findings in acute exacerbation of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 178:372–378
Ichikado K, Johkoh T, Ikezoe J et al (1997) Acute interstitial pneumonia: high-resolution CT findings correlated with pathology. AJR Am J Roentgenol 168:333–338
Ichikado K, Suga M, Gushima Y et al (2000) Hyperoxia-induced diffuse alveolar damage in pigs: correlation between thin-section CT and histopathologic findings. Radiology 216:531–538
Ichikado K, Suga M, Müller NL et al (2002) Acute interstitial pneumonia: comparison of high-resolution computed tomography findings between survivors and non-survivors. Am J Respir Crit Care Med 165:1551–1556
Ichikado K, Suga M, Muranaka H et al (2006) Prediction of prognosis for acute respiratory distress syndrome with thin-section CT: validation in 44 cases. Radiology 238:321–329
World Medical Association (2008) Declaration of Helsinki: ethical principles for medical research involving human subjects, 2008 version (59th World Medical Association General Assembly, Seoul, October 2008). World Health Organization, Geneva, Available via http://www.wma.net/en/30publications/10policies/b3/index.html. Accessed 1 Jan, 2011
Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J (2008) Fleischner Society: glossary terms for thoracic imaging. Radiology 246:697–722
Sumikawa H, Johkoh T, Colby TV et al (2008) Computed tomography findings in pathological usual interstitial pneumonia: relationship to survival. Am J Respir Crit Care Med 177:433–439
Kundel HL, Polansky M (2003) Measurement of observer agreement. Radiology 228:303–308
Bland JM, Altman DG (1986) Statistical methods for assessing agreement between two methods of clinical measurement. Lancet 1(8476):307–310
Akobeng AK (2007) Understanding diagnostic tests 3: receiver operating characteristic curves. Acta Paediatr 96:644–647
Bernard GR, Artigas A, Brigham KL et al (1994) The American-European Consensus Conference on ARDS: definitions, mechanisms, relevant outcomes, and clinical trial coordination. Am J Respir Crit Care Med 149:818–824
Sakamoto K, Taniguchi H, Kondoh Y et al (2010) Serum KL-6 in fibrotic NSIP: correlations with physiologic and radiologic parameters. Respir Med 104:127–133
Lee KS, Kullnig P, Hartman TE, Müller NL (1994) Cryptogenic organizing pneumonia: CT findings in 43 patients. AJR Am J Roentgenol 162:543–546
Akira M, Yamamoto S, Sakatani M (1998) Bronchiolitis obliterans organizing pneumonia manifesting as multiple large nodules or masses. AJR Am J Roentgenol 170:291–295
Acknowledgements
This study was partially supported by a Grant to the Diffuse Lung Disease Group from the Ministry of Health, Labour and Welfare (Japan).
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Fujimoto, K., Taniguchi, H., Johkoh, T. et al. Acute exacerbation of idiopathic pulmonary fibrosis: high-resolution CT scores predict mortality. Eur Radiol 22, 83–92 (2012). https://doi.org/10.1007/s00330-011-2211-6
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DOI: https://doi.org/10.1007/s00330-011-2211-6