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A rare entity of interstitial lung disease, pleuropulmonary fibroelastosis: does it affect the chest wall geometry?

Egyptian Journal of Bronchology volume 13, pages 363–369 (2019)Cite this article

Abstract

Introduction

Pleuropulmonary fibroelastosis (PPFE) is a rare type of interstitial lung disease (ILD); however, it may not be as rare as it was described. PPFE has been recognized increasingly worldwide during the past years.

Patients and methods

The study was held in the Chest Department, Kasr Al-Ainy hospitals, during the period from January 2015 till June 2018. Seventy patients were included and divided into two main groups. Group 1 included 36 cases with PPFE, diagnosed either radiologically alone or combined with histopathological examination of lung biopsy. Group 2 included 34 cases of hypersensitivity pneumonitis (HP) as controls. Group 1 was further subdivided into two subgroups: group A included patients with 19 PPFE without any other pattern of ILD, and group B included 17 cases of PPFE associated with other forms of ILD. Clinical assessment, BMI, and high-resolution computed tomography chest were done. The inner anteroposterior diameter (APD) and transverse diameter (TD) of the chest wall were measured, and the ratio between them was calculated.

Results

Significant female predominance was observed. Both groups of PPFE presented at earlier age than the HP group. Patients with PPFE had a lower body weight and BMI than HP group. There was a significant reduction in the APD and TD in both groups of PPFE than HP group.

Conclusion

Thirty-six cases with PPFE presented either alone or in association with other forms of ILD. Significant reduction in their chest wall APD in comparison with TD was observed, giving a characteristic flat shape of the chest. Further evaluation of this phenomena and its explanation is required.

References

  1. An Official American Thoracic Society/European Respiratory Society Statement. Update of the International multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188:733–748.

    Article  Google Scholar 

  2. Amitani R, Niimi A, Kuze F. Idiopathic pulmonary upper lobe fibrosis. Kokyu 1992; 11:693–699.

    Google Scholar 

  3. Redondoa MT, Meloa N, Motaa PC, Jesus JM, Moura CS, Guimarães S, et al. Idiopathic pleuroparenchymal fibroelastosis:a rare but increasingly recognized entity. Rev Port Pneumol 2015; 21:41–44.

    Google Scholar 

  4. Reddy TL, Tominaga M, Hansell DM, von der Thusen J, Rassl D, Parfrey H, et al. Pleuroparenchymal fibroelastosis:a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012; 40:377–385.

    Article  Google Scholar 

  5. Watanabe K. Pleuroparenchymal fibroelastosis:its clinical characteristics. Curr Respir Med Rev 2013; 9:229–237.

    Article  CAS  Google Scholar 

  6. Vasakova M, Morell F, Walsh S, Leslie K, Raghu G. Hypersensitivity pneumonitis:perspectives in diagnosis and management. Am J Respir Crit Care Med 2017; 196:680–689.

    Article  Google Scholar 

  7. Esteves C, Costa FR, Redondo MT, Moura CS, Guimarães S, Morais A, et al. Pleuroparenchymal fibroelastosis:role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core lung biopsy. Insights Imaging 2016; 7:155–162.

    Article  Google Scholar 

  8. Chan YH. Biostatistics102:quantitative data – parametric & non-parametric tests. Singapore Med J 2003; 44:391–396.

    CAS  PubMed  Google Scholar 

  9. Chan YH. Biostatistics 103:qualitative data -tests of independence. Singapore Med J 2003; 44:498–503.

    CAS  PubMed  Google Scholar 

  10. Chan YH. Biostatistics 104:correlational analysis. Singapore Med J 2003c; 44:614–619.

  11. Harada T, Yoshida Y, Kitasato Y, Tsuruta N, Wakamatsu K, Hirota T. The thoracic cage becomes flattened in the progression of pleuroparenchymalfibroelastosis. EurRespir Rev 2014; 23:263–266.

    Google Scholar 

  12. Shioya M, Otsuka M, Yamada G, Umeda Y, Ikeda K, Nishikiori H, et al. Poorer prognosis of idiopathic pleuroparenchymal fibroelastosis compared with idiopathic pulmonary fibrosis in advanced stage. Can Respir J 2018; 2018:6043053.

    Article  Google Scholar 

  13. Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis:description of a novel clinicopathologic entity. Chest 2004; 126:2007–2013.

    Article  Google Scholar 

  14. Newton CA, Batra K, Torrealba J, Meyer K, Raghu G, Garcia CK. Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations. Eur Respir J 2017; 49:1700696.

    Article  Google Scholar 

  15. Pereira CAC, Gimenez A, Kuranishi L, Storrer K. Chronic hypersensitivity pneumonitis. J Asthma Allergy 2016; 9:171–181.

    Article  CAS  Google Scholar 

  16. Singh S, Collins BF, Sharma BB, Joshi JM, Talwar D, Katiyar S, et al. Interstitial lung disease in India. Results of a prospective registry. Am J Respir Crit Care Med 2017; 195:801–813.

    Article  Google Scholar 

  17. Kusagaya H, Nakamura Y, Kono M, Kaida Y, Kuroishi S, Enomoto N, et al. Idiopathic pleuroparenchymal fibroelastosis:consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med 2012; 12:72.

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Chest, Faculty of Medicine, Cairo University, 16 El Tayaran Street, 11759, Nasr City, Cairo, Egypt

    Yosri M. K. Akl, Mohamed S. Ismail & Yasmine H. El-Hinnawy MD

  2. Department of Diagnostic and Interventional Radiology, Faculty of Medicine, Cairo University, Cairo, Egypt

    Shady N. Mashhour

Authors
  1. Yosri M. K. Akl
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  2. Mohamed S. Ismail
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  3. Yasmine H. El-Hinnawy MD
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  4. Shady N. Mashhour
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Corresponding author

Correspondence to Yasmine H. El-Hinnawy MD.

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Akl, Y.M.K., Ismail, M.S., El-Hinnawy, Y.H. et al. A rare entity of interstitial lung disease, pleuropulmonary fibroelastosis: does it affect the chest wall geometry?. Egypt J Bronchol 13, 363–369 (2019). https://doi.org/10.4103/ejb.ejb_94_18

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  • DOI: https://doi.org/10.4103/ejb.ejb_94_18

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