Combined pulmonary fibrosis and empyhsema (CPFE) syndrome: imaging findings and follow-up.

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1 Combined pulmonary fibrosis and empyhsema (CPFE) syndrome: imaging findings and follow-up. Poster No.: C-0297 Congress: ECR 2014 Type: Scientific Exhibit Authors: F. Ciccarese, D. Attinà, M. Zompatori; Bologna/IT Keywords: Chronic obstructive airways disease, Education, CT, Lung DOI: /ecr2014/C-0297 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 10

2 Aims and objectives The advent of computed tomography-ct has led to the recognition of the coexistence of emphysema and pulmonary fibrosis in the same patient, which results in a clinical syndrome known as combined pulmonary fibrosis and emphysema-cpfe 1. Most patients with CPFE share the same features: male sex, history of smoking (current or former), relatively preserved spirometric values and severely impaired gas exchange (decreased diffusing capacity of lung for carbon monoxide-dlco). The imaging findings typically show an upper-lobe emphysema (bollous, paraseptal, centrilobular) and lower-lobe interstitial fibrotic changes (Fig. 1 on page 2, Fig. 2 on page 2); although a pattern of usual interstitial pneumonia-uip is most commonly detected, a variety of patterns have been reported including nonspecific interstitial pneumonia-nsip, airspace enlargement with fibrosis-aef, respiratory bronchiolitis-associated interstitial lung disease with alveolar septal fibrosis, desquamative interstitial pneumonia with extensive fibrosis and unclassifiable smoking-related interstitial fibrosis 2. Whether CPFE represents a unique entity or a combination of two pulmonary diseases is still unclear. Thus, our aim was to illustrate CPFE HRCT findings, focusing on their evolution, to evaluate whether emphysema and fibrosis show a different evolution over the time. Images for this section: Fig. 1: Typical findings of Combined Pulmonary Fibrosis and Emphysema syndrome (CPFE) consist in an upper-lobe emphysema (A) a and lower-lobe fibrosis (B). Page 2 of 10

3 Fig. 2: Chest X-Ray appearence of Combined Pulmonary Fibrosis and Emphysema syndrome (CPFE). Page 3 of 10

4 Methods and materials We reviewed HRCT images of 21 patients with CPFE. The inclusion criteria were emphysema visual score#10% (i.e. non trivial) and two HRCT scans for each patient, with the second one performed at least after one year. Gender 17 males, 4 females Age 64.6 years (range: 30-83) Smoke history 14 current smokers, 7 ex-smokers Distance between HRCT scans 22.3 months (range: 12-59) Demographic data of the studied population Two observers analyzed the baseline scan and the following one, recording: fibrosis extension 3 and pattern 4,5, type of emphysema and extension 6, other findings. Disagreements were solved by consensus. Diagnosis UIP NSIP AEF Fibrotic pattern Pattern Reticular opacities; honeycombing; traction bronchiectasis/bronchiolectasis; architectural distorsion; focal ground glass Ground glass attenuation; irregular lines; consolidations Multiple large thin-walled cystic lesions, thinner than that of UIP and thicker than that of emphysema Images for this section: Page 4 of 10

5 Fig. 3: Fibrotic patterns in Combined Pulmonary Fibrosis and Emphysema syndrome (CPFE) could be represented by usual interstitial pneumonia-uip (A), nonspecific interstitial pneumonia- NSIP (B) or airspace enlargment with fibrosis-aef (C). Page 5 of 10

6 Results At baseline, the average emphysema extension was of 16.5%, in the majority of patients both centrilobular and paraseptal (17/ % patients). Fibrosis extension was of 18.8%, with a UIP-like pattern in 10 patients, inconsistent UIP in 2 patients and non UIP in 9 patients (NSIP or AEF)- Fig. 3 on page 6. The second examination did not show change of pattern; on average, there was only a slight progression of emphysema (final extension:19.5%) and fibrosis (final extension:22.6%), that did not reach statistical significance. In contrast, UIP presented a major tendency of progression (in 6/ %patients versus 2/ % patients with other pattern-p=0.05)- Fig. 4 on page 6. Moreover, a high prevalence of complications were detected which were: dilatation of pulmonary arteries in 7/ % patients (Fig. 5 on page 7 ), development of lung cancer in 4/ % patients (Fig. 6 on page 7 ), acute lung injury in 1-4.8% patients (Fig. 7 on page 8 ). Images for this section: Fig. 3: Fibrotic patterns in Combined Pulmonary Fibrosis and Emphysema syndrome (CPFE) could be represented by usual interstitial pneumonia-uip (A), nonspecific interstitial pneumonia- NSIP (B) or airspace enlargment with fibrosis-aef (C). Page 6 of 10

7 Fig. 4: Progression of UIP from the baseline scan (A) to the second scan (B). Fig. 5: Pulmonary hypertension in Combined Pulmonary Fibrosis and Emphysema syndrome (CPFE), characterized by an enlarged diameter of main pulmonary arteries (A) and of right heart chambers (B). Page 7 of 10

8 Fig. 6: Small peripheral lung cancer (A) detected in Combined Pulmonary Fibrosis and Emphysema syndrome (CPFE) -B. Fig. 7: Acute Lung Injury in Combined Pulmonary Fibrosis and Emphysema syndrome (CPFE). Page 8 of 10

9 Conclusion The reasons for the coexistence of pulmonary fibrosis and emphysema remain unknown. Their relationship may be due to a pure coincidence or to a common environmental insult (tobacco smoke) upon an unknown genetic predisposition; moreover, one recent hypothesis suggests that fibrosis and emphysema share common pathogenetic mechanisms of accelerated senescence via telomere length abnormalities 7. However, the natural history of CPFE seems to be different from the one of patients with "pure" emphysema or fibrosis (in spite of some conflicting data, the majority of studies reveal a better prognosis than UIP and a worse prognosis than emphysema) 8,9. Predictors of survival may be linked to the development of complications which are pulmonary hypertension (which occurs up to 50% of patients with CPFE), lung cancer and acute lung injury 2. Our study revealed that in the short term follow-up (22 months) there is only a slight and non statistically significant progression of both emphysema and fibrosis except for patients with UIP; UIP pattern is associated with higher probabilities of evolution, as in patients with "pure" fibrotic changes of lung parenchyma. Thus, CT plays a key role in the management of patients with CPFE both in diagnosis (as it is based on imaging findings) and in follow-up (prognostic evaluation and detection of complications). Personal information Correspondance to: Federica Ciccarese, Cardio-Thoracic Radiology Unit, S.Orsola Malpighi University Hospital, Bologna, Italy; ciccarese.f@gmail.com References 1. Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005; 26: Jankowich MD, Rounds SI. Combined Pulmonary Fibrosis and Emphysema Syndrome. Chest 2012; 141: Akira M, Sakatani M, Ueda E. Idiopathic pulmonary fibrosis: progression of honeycombing at thinsection CT. Radiology 1993; 189: Page 9 of 10

10 4. American Thoracic Society/European Respiratory Society. International multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med : Yamada T, Nakanishi Y, Homma T, et al. Airspace enlargement with fibrosis shows characteristic histology and immunohistology different from usual interstitial pneumonia, nonspecific interstitial pneumonia and centrilobular emphysema. Pathol Int 2013;63: COPD Gene CT Workshop Group, Barr RG, BerkowitzEA, et al. A combined pulmonary -radiology workshop for visual evaluation of COPD: study design, chest CT findings and concordance with quantitative evaluation. COPD 2012; 9: Papiris SA, Triantafillidou C, Manali ED, et al. Combined pulmonary fibrosis and emphysema. Expert Rev Respir Med 2013; 7: Kurashima K, Takayanagi N, Tsuchiya N, et al. The effect of emphysema on lung function and survival in patients with idiopathic pulmonary fibrosis. Respirology 2010; 15: Lee CH, Kim HJ, Park CM et al. The impact of combined pulmonary fibrosis and emphysema on mortality. Int j Tuberc Lung Dis 2011; 15: Page 10 of 10

CT findings in Differential Diagnosis between Tuberculous Pleurisy and Malignant Effusion

CT findings in Differential Diagnosis between Tuberculous Pleurisy and Malignant Effusion Poster No.: E-0084 Congress: ESTI 2012 Type: Scientific Exhibit Authors: S. S. Shim, Y. Kim; Seoul/KR Keywords: