https://doi.org/10.4081/monaldi.2022.2363
Diagnosis of pleuroparenchymal fibroelastosis: A review
Submitted: June 29, 2022
Accepted: October 10, 2022
Published: October 21, 2022
Accepted: October 10, 2022
Abstract Views: 2182
PDF: 540
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease with unprecedented features characterized by fibroelastotic changes in the subpleural lung parenchyma affecting the upper lobes. PPFE is usually idiopathic, but it can be caused by infection, autoimmunity, bone marrow or lung transplantation, or a genetic predisposition. Histopathologic examination of lung biopsy samples reveals homogenous subpleural fibrosis and abundant elastic fibers, allowing for a definitive diagnosis. As PPFE mimics many interstitial lung diseases, clinicians face significant difficulties in making a definitive final diagnosis. Since most disease-related comorbid conditions manifest at an advanced stage, invasive tissue sampling for histopathologic evaluation is consistently impossible. Such a patient presentation highlights the importance of an analysis based solely on clinical findings, which would provide a definitive diagnosis without the need for a biopsy. Because of its exceptional and inconceivable presentation, PPFE creates a diagnostic dilemma. In light of our two cases and the literature data, we present a diagnostic assessment score assay that relies solely on clinical manifestations without histopathological tissue verification to shed light on the diagnosis of PPFE. This review focuses on PPFE identification through the use of a diagnostic assessment analysis to improve early disease recognition without the use of invasive diagnostic interventions to obtain biopsy samples for histopathologic evaluation. This analytic approach, while not diagnostic in and of itself, may provide a useful pathway for differential diagnosis and may preclude redundant initiatives.
Amitani R, Nimi A, Kuse F. Idiopathic upper lobe fibrosis (IPUF). Kokyu 1992;11:693-9.
Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis. Description of a novel clinicopathologic entity. Chest 2004;126:2007-13.
DOI: https://doi.org/10.1378/chest.126.6.2007
Reddy TL, Tominaga M, Hansell DM, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012;40:377-85.
DOI: https://doi.org/10.1183/09031936.00165111
Travis WD, Costabel U, Hansell DM, 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;188:733–48.
DOI: https://doi.org/10.1164/rccm.201308-1483ST
Iesato K, Ogasawara T, Masuda A, et al. Idiopathic pulmonary upper lobe fibrosis; clinical and pathological features. Rinsho Hoshasen 2005;50:13-25.
Chua F, Desai SR, Nicholson AG, et al. Pleuroparenchymal fibroelastosis. A review of clinical, radiological, and pathological characteristics. Ann Am Thorac Soc 2019;16:1351-9.
DOI: https://doi.org/10.1513/AnnalsATS.201902-181CME
Watanabe K. Pleuroparenchymal fibroelastosis: its clinical characteristics. Curr Respir Med Rev 2013;9:229–37.
DOI: https://doi.org/10.2174/1573398X0904140129125307
Becker CD, Gil J, Padilla ML. Idiopathic pleuropulmonary fibroelastosis: an unrecognized or misdiagnosed entity ? Mod Pat 2008;21:784-87.
DOI: https://doi.org/10.1038/modpathol.2008.56
Cheng SK, Chuah KL. Pleuroparenchymal fibroelastosis of the lung: A review. Arch Pat Lab Med 2016;140:849-53.
DOI: https://doi.org/10.5858/arpa.2015-0166-RS
Nakatani T, Arai T, Kitaichi M, et al. Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? Eur Respir J 2015;45:1183-6.
DOI: https://doi.org/10.1183/09031936.00214714
Rosenbaum JN, Butt YM, Johnson KA, et al. Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury. Hum Pathol 2015;46:137-46.
DOI: https://doi.org/10.1016/j.humpath.2014.10.007
Hirota T, Yoshida Y, Kitasato Y, et al. Histological evolution of pleuroparenchymal fibroelastosis. Histopathology 2015;66:545-54.
Tanizawa K, Handa T, Kubo T, Chen F. Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: A retrospective cohort study. Respir Res 2018;19:162-72.
DOI: https://doi.org/10.1186/s12931-018-0860-6
Shioya M, Otsuka M, Yamada G, et al. Poorer prognosis of idiopathic pleuroparenchymal fibroelastosis compared with idiopathic pulmonary fibrosis in advanced stage. Can Respir J 2018;2018:6043053.
DOI: https://doi.org/10.1155/2018/6043053
Nakasone E, Bando M, Nakao T, Yamasawa HS. Pleuroparenchymal fibroelastosis in patients with pulmonary disease after bone marrow transplantation. Nikon Kokyuki Gakkai Zasshi 20121:562-6.
Von der Thusen JH, Hansell DM, Tominaga M, et al. Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation. Modern Pathol 2011;24:1163-9.
DOI: https://doi.org/10.1038/modpathol.2011.114
Hirota T, Fujita M, Matsumoto, et al. Pleuroparenchymal fibroelastosis as a manifestastion of chronic lung rejection. Eur Respir J 2013;41:243-5.
DOI: https://doi.org/10.1183/09031936.00103912
Inuzuka K, Yasui M, Waseda Y, et al. A case of repeated bilateral pneumothorax associated with upper lobe predominant fibrosis in an aluminum processing worker. Nikon Kokyuki Gakkai Zasshi 2010;8:92-6.
Shiota S, Shimizu K, Suzuki M, et al. Seven cases of marked pulmonary fibrosis in the upper lobe. Nihon Kokyuki Gakkai Zasshi 1999;37:87–96.
Hirota T, Yoshida Y, Kitasato Y, et al. Histological evolution of pleuroparenchymal fibroelastosis. Histopathology 2015;66:545–54.
DOI: https://doi.org/10.1111/his.12554
Yoshida Y, Nagata N, Tsurata N, et al. Heterogenous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis. Respir Investig 2016;54:162-9.
DOI: https://doi.org/10.1016/j.resinv.2015.11.002
Azoulay E, Paugam B, Heymann MF, et al. Familial extensive idiopathic bilateral pleural fibrosis. Eur Respir J 1999;14:971–3.
DOI: https://doi.org/10.1034/j.1399-3003.1999.14d41.x
Borie R, Tabèze L, Thabut G, et al. Prevalence and characteristics of TERT and TERC mutations in suspected genetic pulmonary fibrosis. Eur Respir J 2016; 48: 1721–31.
DOI: https://doi.org/10.1183/13993003.02115-2015
Newton CA, Batra K, Torrealba J, et al. Telomere-related lung fibrosis is diagnostically heterogeneous but uniformly progressive. Eur Respir J 2016;48:1710-20.
DOI: https://doi.org/10.1183/13993003.00308-2016
Stuart BD, Lee JS, Kozlitina J, et al. Effect of telomere length on survival in patients with idiopathic pulmonary fibrosis: an observational cohort study with independent validation. Lancet Respir Med 2014;2:557-65.
DOI: https://doi.org/10.1016/S2213-2600(14)70124-9
Oyama Y, Enomoto N, Suzuki Y, et al. Evaluation of urinary desmosines as a noninvasive diagnostic biomarker in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE) Respir Med 2017;123:63–70.
DOI: https://doi.org/10.1016/j.rmed.2016.12.013
Kinoshita Y, Ikeda T, Miyamura T, et al. A proposed prognostic prediction score for pleuroparenchymal fibroelastosis. Respir Res 2021;22:215.
DOI: https://doi.org/10.1186/s12931-021-01810-z
Ishii W, Watanabe K, Kushima H, et al. Pleuroparenchymal fibroelastosis diagnosis by multidisciplinary discussions in Japan. Respir Med 2018;141:190-7.
DOI: https://doi.org/10.1016/j.rmed.2018.06.022
Maher TM, Corte TJ, Fischer A, et al. Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med 2020;8:147-57.
DOI: https://doi.org/10.1016/S2213-2600(19)30341-8
George PM, Wells AU. Pirfenidone for the treatment of idiopathic pulmonary fibrosis. Expert Rev Clin Pharmacol 2017;10:483-91.
DOI: https://doi.org/10.1080/17512433.2017.1295846
Ruwanpura SM, Thomas BJ, Bardin PG. Pirfenidone: Molecular mechanisms and potential clinical applications in lung disease. Am J Respir Cell Mol Biol 2020;62:413-22.
DOI: https://doi.org/10.1165/rcmb.2019-0328TR
Vancheri C, Kreuter M, Richeldi L, et al. al. Nintedanib with add-on pirfenidone in idiopathic pulmonary fibrosis. Results of the INJOURNEY trial. Am J Respir Crit Care Med 2018;197:356-63.
DOI: https://doi.org/10.1164/rccm.201706-1301OC
Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med 2019;381:1718-27.
DOI: https://doi.org/10.1056/NEJMoa1908681
Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014;370:2071-82.
DOI: https://doi.org/10.1056/NEJMoa1402584
Distler O, Highland KB, Gahlemann M, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med 2019;380:2518-28.
DOI: https://doi.org/10.1056/NEJMoa1903076
Tetikkurt C, Kubat B, Kulahci C, et al. Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis. Monaldi Arch Chest Dis 2021;91:1713.
DOI: https://doi.org/10.4081/monaldi.2021.1713
Sekine A, Satoh H, Iwasawa T, et al. Unilateral upper lung field pulmonary fibrosis radiologically consistent with pleuroparenchymal fibroelastosis after thoracotomy: A new disease entity related to thoracotomy. Respiration 2017;94:431-41.
DOI: https://doi.org/10.1159/000479331
Watanabe K, Ishii H, Kiyomi F, et al. Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal. Respir Investig 2019;57:312-20.
DOI: https://doi.org/10.1016/j.resinv.2019.02.007
Piciucchi S, Tomassetti S, Casoni G, et al. High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res 2011;12:111-5.
DOI: https://doi.org/10.1186/1465-9921-12-111
Oda T, Ogura T, Kitamura H, et al. Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis. Chest 2014;146:1248-55.
DOI: https://doi.org/10.1378/chest.13-2866
How to Cite
Tetikkurt, Cuneyt, Buket Caliskaner Ozturk, and Nejdiye Gungordu. 2022. “Diagnosis of Pleuroparenchymal Fibroelastosis: A Review”. Monaldi Archives for Chest Disease 93 (3). https://doi.org/10.4081/monaldi.2022.2363.
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