Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis

Submitted: December 3, 2020
Accepted: February 28, 2021
Published: April 6, 2021
Abstract Views: 1249
PDF: 573
Publisher's note
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

Idiopathic pleuropulmonary fibroelastosis is an extremely rare lung disease characterized by the combination of fibrosis of the visceral pleura and the fibroelastotic changes transcending in the subpleural lung parenchyma that predominantly affects the upper lobes with accompanying volume loss. It is mostly idiopathic while infection, autoimmunity, bone marrow or lung transplantation and genetic predisposition may be associated with the development of PPFE. The disease is exceptionally rare as approximately ninety cases have been reported in the literature currently. A 35-year-old female presented with exertional dyspnea, dry cough and weight loss. Physical examination demonstrated platythorax, suprasternal notch deepening and fine rales over the upper lobes. Blood count, serum biochemistry, autoimmunity and serologic markers for collagen vascular diseases were within normal limits. Arterial blood gases demonstrated a low pO2 (48 mm Hg) and a high pCO2 (54 mm Hg) values. Chest x-ray showed bilateral parenchymal fibrotic lesions, left pneumothorax, bronchiectasis in the middle and pleural thickening in the upper lung zones while HRCT revealed bilateral apical pleural thickening, traction bronchiectasis, subpleural reticulations, ground-glass opacities and honeycombing in the upper lobes. Bronchoscopy, BAL cytology, smear and culture did not reveal any pathologic findings. Relevant with the clinical, laboratory, radiologic manifestations and the differential diagnosis with other interstitial lung diseases, PPFE was the final diagnosis. The aim of this case report was to present the clinical manifestations of our case. The second crucial objective was to establish a diagnostic scoring system relevant with the literature and the clinical manifestations of the patient.

Dimensions

Altmetric

PlumX Metrics

Downloads

Download data is not yet available.

Citations

Amitani R, Nimi A, Kuse F. Idiopathic upper lobe fibrosis (IPUF). Kokyu 1992;11:693-9.
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
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
Watanabe K. Pleuroparenchymal fibroelastosis: Its clinical characteristics. Curr Respir Med Rev 2013;9:229-37. DOI: https://doi.org/10.2174/1573398X0904140129125307
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
Becker CD, Gil J, Padilla ML. Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity? Mod Pathol 2008;21:784-7. DOI: https://doi.org/10.1038/modpathol.2008.56
Takeuchi Y, Miyagawa-Hayashino A, Chen F, et al. Pleuroparenchymal fibroelastosis and non-specific interstitial pneumonia: frequent pulmonary sequelae of haematopoietic stem cell transplantation. Histopathology 2015;66:536-44. DOI: https://doi.org/10.1111/his.12553
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
Ofek E, Sato M, Saito T, et al. Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis. Mod Pathol 2013;26:350-6. DOI: https://doi.org/10.1038/modpathol.2012.171
Hirota T, Fujita M, Matsumoto, et al. Pleuroparenchymal fibroelastosis as a manifestation 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.
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
Hirota T, Yoshida Y, Kitasato Y, et al. Histological evolution of pleuroparenchymal fibroelastosis. Histopathology 2015;66:545-54.
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
An Official American Thoracic Society/European Respiratory Society Statement: Update of the International multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Amer J Respir Crit Care Med 2013;188:733-48.
Nakamura J, Halliday NA, Fukuba E, et al. The microanatomic basis of finger clubbing: A high-resolution magnetic resonance imaging study. J Rheumatol 2014;41:523-7. DOI: https://doi.org/10.3899/jrheum.130823
Grathwohl KW, Thompson JW, Riordan KK, et al. Digital clubbing associated with polymyositis and interstitial lung disease. Chest 1995;108:1751-2. DOI: https://doi.org/10.1378/chest.108.6.1751
Kanematsu T, Kitaichi M, Nishimura K, et al. Clubbing of the fingers and smooth-muscle proliferation in fibrotic changes in the lung in patients with idiopathic pulmonary fibrosis. Chest 1994;105:339-42. DOI: https://doi.org/10.1378/chest.105.2.339
Sridhar KS, Lobo CF, Altman RD. Digital clubbing and lung cancer. Chest 1998;114:1535-7. DOI: https://doi.org/10.1378/chest.114.6.1535
Ward RW, Chin R Jr, Keyes JW Jr, Haponik EF. Digital clubbing. Demonstration with positron emission tomography. Chest 1995;107:1172-3. DOI: https://doi.org/10.1378/chest.107.4.1172
Castori M, Sinibaldi L, Mingarelli R, et al. Pachydermoperiostosis: an update. Clin Genet 2005;68:477-86. DOI: https://doi.org/10.1111/j.1399-0004.2005.00533.x
Tariq M, Azeem Z, Ali G, et al. Mutation in the HPGD gene encoding NAD+ dependent 15-hydroxyprostaglandin dehydrogenase underlies isolated congenital nail clubbing (ICNC). J Med Genet 2009;46:14-20. DOI: https://doi.org/10.1136/jmg.2008.061234
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
Bonifazi M, Montero MA, Renzoni EA. Idiopathic Pleuroparenchymal Fibroelastosis. Curr Pulmonol Rep. 2017;6:9-15. DOI: https://doi.org/10.1007/s13665-017-0160-5
Xu L, Rassaei N, Caruso C. Pleuroparenchymal fibroelastosis with long history of asbestos and silicon exposure. Int J Surg Pathol 2018;26:190–3. DOI: https://doi.org/10.1177/1066896917739399
Muzzammil A, Anne K, Palak S, Bajinder D. Pleuroparenchymal fibroelastosis with a positive p-ANCA. Arch Clin Med Case Rep 2019;3:51-4. DOI: https://doi.org/10.26502/acmcr.96550058
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
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
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
Cheng SKH, Chuah KL. Pleuroparenchymal fibroelastosis of the lung: A review. Arch Pathol Lab Med 2016;140:849–53. DOI: https://doi.org/10.5858/arpa.2015-0166-RS
Cuneyt Tetikkurt, Pulmonary Diseases Department, Cerrahpasa Medical Faculty, Istanbul University

Pulmonary Diseases

 

Professor

How to Cite

Tetikkurt, Cuneyt, Bahar Kubat, Cigdem Kulahci, Seza Tetikkurt, and Buket Caliskaner Ozturk. 2021. “Assessment Score for the Diagnosis of a Case With Pleuroparenchymal Fibroelastosis”. Monaldi Archives for Chest Disease 91 (3). https://doi.org/10.4081/monaldi.2021.1713.

Similar Articles

You may also start an advanced similarity search for this article.