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Open Access 01-12-2018 | Research

Cryobiopsies are diagnostic in Pleuroparenchymal and Airway-centered Fibroelastosis

Authors: Sissel Kronborg-White, Claudia Ravaglia, Alessandra Dubini, Sara Piciucchi, Sara Tomassetti, Elisabeth Bendstrup, Venerino Poletti

Published in: Respiratory Research | Issue 1/2018

Abstract

Background

Idiopathic pulmonary fibroelastosis (iPPFE) is a rare lung lesion characterized by pleural and subpleural parenchymal thickening due to accumulation of fibroelastotic tissue. Only recently, a few cases with a peribronchiolar distribution of fibroelastotic tissue have been reported. These lesions are more prominent in the upper lobes. Even though high resolution computed tomography (HRCT) scan features are considered characteristic, a histological confirmation is suggested, mainly when the clinical setting is not clearly defined. However, due to non-negligible complications, a surgical lung biopsy is not often recommended. The prognosis is usually poor and currently, the only effective treatment is lung transplantation.

Method

Patients with a multidisciplinary diagnosis of iPPFE or airway-centered fibroelastosis (airway-centered FE), with histological confirmation by transbronchial cryobiopsy, were identified from an ongoing interstitial lung disease registry. Data on patient demographics, HRCT patterns, size and number of biopsies, histology patterns and complications were registered.

Results

Seven patients were diagnosed with iPPFE and one patient was diagnosed with airway-centered FE. Pneumothorax was documented in three cases, but none of them required a chest tube. No other complications during or after the procedure were observed.

Conclusion

This study suggests that using cryobiopsies in the diagnostics of PPFE and airway-centered FE is safe and effective.

Poletti V, Ravaglia C, Gurioli C, Piciucchi S, Dubini A, Cavazza A, et al. Invasive diagnostic techniques in idiopathic interstitial pneumonias. Respirology. 2016;21:44–50.

Kronborg-White S, Folkersen B, Rasmussen TR, Voldby N, Madsen LB, Rasmussen F, et al. Introduction of cryobiopsies in the diagnostics of interstitial lung diseases – experiences in a referral center. Eur Clin Respir J [Internet]. 2017;4(1):1274099. Available from: https://www.tandfonline.com/doi/full/10.1080/20018525.2016.1274099

Lentz RJ, Christine Argento A, Colby TV, Rickman OB, Maldonado F. Transbronchial cryobiopsy for diffuse parenchymal lung disease: a state-of-the-art review of procedural techniques, current evidence, and future challenges. J Thorac Dis. 2017;9:2186–203.

Johannson KA, Marcoux VS, Ronksley PE, Ryerson CJ. Diagnostic yield and complications of transbronchial lung cryobiopsy for interstitial lung disease: A systematic review and metaanalysis. Annals of the Am Thorac Soc. 2016;13:1828–38.

Travis WD, Costabel U, Hansell DM, King 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 pneumonias. Am J Respir Crit Care Med. 2013;188(6):733–48.CrossRefPubMedPubMedCentral

Ravaglia C, Bonifazi M, Wells AU, Tomassetti S, Gurioli C, Piciucchi S, et al. Safety and diagnostic yield of Transbronchial lung Cryobiopsy in diffuse parenchymal lung diseases: a comparative study versus video-assisted Thoracoscopic lung biopsy and a systematic review of the literature. Respiration. 2016;91(3):215–27.CrossRefPubMed

Jabbardarjani H, Kiani A, Karimi M, Fakhri M. Cryobiopsy shows better safety profile and diagnostic yield as compared to conventional forceps for interstitial lung disease. Eur Respir J [Internet]. 2013;42. http://erj.ersjournals.com/content/42/Suppl_57/P2301.full.pdf+html?sid=b2d79afe-49ab-4aaf-b4f3-c231dc3d7efe%5Cn.

Ganganah O, Guo SL, Chiniah M, Li YS. Efficacy and safety of cryobiopsy versus forceps biopsy for interstitial lung diseases and lung tumours: A systematic review and meta-analysis. Respirology [Internet]. 2016 Jul [cited 2017 Mar 16];21(5):834–841. Available from: http://doi.wiley.com/10.1111/resp.12770

Piciucchi S, Tomassetti S, Casoni G, Sverzellati N, Carloni A, Dubini A, et al. High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res [Internet]. 2011;12(1):111. Available from: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=3197499&tool=pmcentrez&rendertype=abstract.

10.

Rea G, Poletti V, Iadevaia C, Bocchino M, Mazzarella G. Idiopathic pleuroparenchymal fibroelastosis: incidental findings in a patient with suspected pneumonia. J Bras Pneumol [Internet]. 2015 Oct [cited 2017 Mar 16];41(5):478–479. Available from: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1806-37132015000500478&lng=en&nrm=iso&tlng=en.

11.

Bonifazi M, Montero MA, Renzoni EA. Idiopathic Pleuroparenchymal Fibroelastosis. Curr Pulmonol Reports [Internet]. 2017 Mar 27 [cited 2017 Apr 10];6(1):9–15. Available from: http://link.springer.com/10.1007/s13665-017-0160-5

12.

Khiroya R, Macaluso C, Montero MA, Wells AU, Chua F, Kokosi M, et al. Pleuroparenchymal Fibroelastosis. Am J Surg Pathol [Internet]. 2017 Dec [cited 2018 Feb 6];41(12):1683–1689. Available from: http://www.ncbi.nlm.nih.gov/pubmed/28877057

13.

Kinoshita Y, Watanabe K, Ishii H, Kushima H, Fujita M, Nabeshima K. Significant increase of lymphatic vessels in pleuroparenchymal fibroelastosis. Histopathology [Internet]. 2018 Apr 19 [cited 2018 May 30]; Available from: http://www.ncbi.nlm.nih.gov/pubmed/29675827

14.

Enomoto Y, Matsushima S, Meguro S, Kawasaki H, Kosugi I, Fujisawa T, et al. Podoplanin-positive myofibroblasts: a pathological hallmark of pleuroparenchymal fibroelastosis. Histopathology [Internet]. 2018 Jun [cited 2018 May 30];72(7):1209–1215. Available from: http://doi.wiley.com/10.1111/his.13494

15.

Cheng SKH, Chuah KL. Pleuroparenchymal fibroelastosis of the lung: a review. Arch Pathol Lab Med. 2016;140:849–53.

16.

Mariani F, Gatti B, Rocca A, Bonifazi F, Cavazza A, Fanti S, et al. Pleuroparenchymal fibroelastosis: the prevalence of secondary forms in hematopoietic stem cell and lung transplantation recipients. Diagnostic Interv Radiol. 2016;22(5):400–6.CrossRef

17.

Rosenbaum JN, Butt YM, Johnson KA, Meyer K, Batra K, Kanne JP, et al. Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury. Hum Pathol. 2015;46(1):137–46.CrossRefPubMed

18.

Kusagaya H, Fujisawa T, Enomoto N, Inui N, Nakamura Y, Suda T. Co-occurrence of pneumoperitoneum and pneumothorax in a patient with Pleuroparenchymal Fibroelastosis. Am J Respir Crit Care Med [Internet]. 2015 May 15 [cited 2017 Oct 23];191(10):1200–1201. Available from: http://www.ncbi.nlm.nih.gov/pubmed/25978573

19.

Watanabe K. Pleuroparenchymal Fibroelastosis: its clinical characteristics. Curr Respir Med Rev. 2013;9:229–37.CrossRefPubMedCentral

20.

Kokosi MA, Nicholson AG, Hansell DM, Wells AU. Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP. Respirology [Internet]. 2016 May [cited 2017 Oct 30];21(4):600–614. Available from: http://www.ncbi.nlm.nih.gov/pubmed/26627191

21.

Pradere P, Gauvain C, Danel C, Debray MP, Borie R, Plantier L, et al. Airway-centered fibroelastosis a distinct entity. Chest. 2016;149(3):767–74.CrossRefPubMed

22.

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;40:377–85.

23.

Esteves C, Costa FR, Redondo MT, Moura CS, Guimar??es S, Morais A, et al. Pleuroparenchymal fibroelastosis: role of high-resolution computed tomography (HRCT) and CT-guided transthoracic core lung biopsy. Insights into Imaging. 2016;7:155–62.

24.

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 [Internet]. 2012 Aug [cited 2017 Oct 31];40(2):377–385. Available from: http://erj.ersjournals.com/lookup/doi/10.1183/09031936.00165111

25.

Von Der Thüsen JH. Pleuroparenchymal Fibroelastosis: its pathological characteristics. Curr Respir Med Rev. 2013;9:238–47.CrossRefPubMedPubMedCentral

26.

Cuppens K, Verbeken E, Coolen J, Verschakelen J, Wuyts W. Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature. Respir Med Case Reports [Internet]. 2014 [cited 2017 Oct 31];12:7–9. Available from: http://www.ncbi.nlm.nih.gov/pubmed/26029526

27.

Piciucchi S, Tomassetti S, Casoni G, Sverzellati N, Carloni A, Dubini A, et al. High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res [Internet]. 2011 Dec 1 [cited 2017 Oct 23];12(1):111. Available from: http://www.ncbi.nlm.nih.gov/pubmed/21861891

28.

Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 2004;126(6):2007–13.CrossRefPubMed

29.

Hirota T, Fujita M, Matsumoto T, Higuchi T, Shiraishi T, Minami M, et al. Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection? Eur Respir J [Internet]. 2013 Jan 1 [cited 2017 Oct 31];41(1):243–245. Available from: http://www.ncbi.nlm.nih.gov/pubmed/23277524

30.

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 [Internet]. 2012 Dec 5 [cited 2017 Mar 16];12(1):72. Available from: http://www.ncbi.nlm.nih.gov/pubmed/23216996

31.

Kreider ME, Hansen-Flaschen J, Ahmad NN, Rossman MD, Kaiser LR, Kucharczuk JC, et al. Complications of Video-Assisted Thoracoscopic Lung Biopsy in Patients with Interstitial Lung Disease. Ann Thorac Surg [Internet]. 2007 Mar [cited 2017 Oct 31];83(3):1140–1144. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17307476

32.

Kondoh Y, Taniguchi H, Kitaichi M, Yokoi T, Johkoh T, Oishi T, et al. Acute exacerbation of interstitial pneumonia following surgical lung biopsy. Respir Med [Internet]. 2006 Oct [cited 2017 Oct 31];100(10):1753–1759. Available from: http://www.ncbi.nlm.nih.gov/pubmed/16584880

33.

Utz JP, Ryu JH, Douglas WW, Hartman TE, Tazelaar HD, Myers JL, et al. High short-term mortality following lung biopsy for usual interstitial pneumonia. Eur Respir J [Internet]. 2001 Feb [cited 2017 Oct 31];17(2):175–179. Available from: http://www.ncbi.nlm.nih.gov/pubmed/11334116

34.

Park JH, Kim DK, Kim DS, Koh Y, Lee S-D, Kim WS, et al. Mortality and risk factors for surgical lung biopsy in patients with idiopathic interstitial pneumonia. Eur J Cardiothorac Surg [Internet]. 2007 Jun [cited 2017 Oct 31];31(6):1115–1119. Available from: https://academic.oup.com/ejcts/article-lookup/doi/10.1016/j.ejcts.2007.02.035

35.

Brunelli A, Monteverde M, Borri A, Salati M, Marasco RD, Fianchini A. Predictors of prolonged air leak after pulmonary lobectomy. Ann Thorac Surg [Internet]. 2004 Apr [cited 2017 Oct 31];77(4):1205–1210. Available from: http://www.ncbi.nlm.nih.gov/pubmed/15063235

36.

Billé A, Borasio P, Gisabella M, Errico L, Lausi P, Lisi E, et al. Air leaks following pulmonary resection for malignancy: risk factors, qualitative and quantitative analysis. Interact Cardiovasc Thorac Surg [Internet]. 2011 Jul [cited 2017 Oct 31];13(1):11–15. Available from: https://academic.oup.com/icvts/article-lookup/doi/10.1510/icvts.2011.266247

37.

Becker CD, Gil J, Padilla ML. Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity? Mod Pathol [Internet]. 2008 Jun 11 [cited 2017 Oct 31];21(6):784–787. Available from: http://www.nature.com/doifinder/10.1038/modpathol.2008.56

38.

Watanabe S, Waseda Y, Takato H, Matsunuma R, Johkoh T, Egashira R, et al. Pleuroparenchymal fibroelastosis: Distinct pulmonary physiological features in nine patients. Respir Investig [Internet]. 2015 Jul [cited 2017 Oct 31];53(4):149–155. Available from: http://linkinghub.elsevier.com/retrieve/pii/S2212534515000301

39.

Iftikhar IH, Alghothani L, Sardi A, Berkowitz D, Musani AI. Transbronchial Lung Cryobiopsy and Video-Assisted Thoracoscopic Lung Biopsy in the Diagnosis of Diffuse Parenchymal Lung Disease: A Meta-analysis of Diagnostic Test Accuracy. Ann Am Thorac Soc [Internet]. 2017 Apr 11 [cited 2017 Oct 31];AnnalsATS.201701-086SR. Available from: http://www.atsjournals.org/doi/10.1513/AnnalsATS.201701-086SR

40.

Casoni GL, Tomassetti S, Cavazza A, Colby TV, Dubini A, Ryu JH, et al. Transbronchial lung cryobiopsy in the diagnosis of fibrotic interstitial lung diseases. PLoS One. 2014;9(2)

41.

Hagmeyer L, Theegarten D, Treml M, Priegnitz C, Randerath W. Validation of transbronchial cryobiopsy in interstitial lung disease - interim analysis of a prospective trial and critical review of the literature. Sarcoidosis, Vasc Diffus lung Dis Off J WASOG [Internet]. 2016 Mar 29 [cited 2017 Oct 31];33(1):2–9. Available from: http://www.ncbi.nlm.nih.gov/pubmed/27055830

42.

Oyama Y, Enomoto N, Suzuki Y, Kono M, Fujisawa T, Inui N, et al. Evaluation of urinary desmosines as a noninvasive diagnostic biomarker in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE). Respir Med [Internet]. 2017 Feb [cited 2017 Oct 31];123:63–70. Available from: http://linkinghub.elsevier.com/retrieve/pii/S0954611116303419

Title: Cryobiopsies are diagnostic in Pleuroparenchymal and Airway-centered Fibroelastosis
Authors: Sissel Kronborg-White
Claudia Ravaglia
Alessandra Dubini
Sara Piciucchi
Sara Tomassetti
Elisabeth Bendstrup
Venerino Poletti
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: Respiratory Research / Issue 1/2018
Electronic ISSN: 1465-993X
DOI: https://doi.org/10.1186/s12931-018-0839-3

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