https://doi.org/10.4081/monaldi.2023.2632
Overcoming the challenges of a misdiagnosed rare lung disease – idiopathic pleuroparenchymal fibroelastosis
Submitted: May 8, 2023
Accepted: June 26, 2023
Published: August 2, 2023
Accepted: June 26, 2023
Abstract Views: 1207
PDF: 62
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.
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 condition characterized by pleural and subpleural lung fibroelastosis with an upper lobe predominance. We present the third case of idiopathic PPFE from India, as well as the second ante-mortem diagnosis. A 27-year-old man presented with a 1-year history of modified Medical Research Council class II shortness of breath and dry cough. He described a 15-kg weight loss. After a clinico-radiological diagnosis, he was given anti-tubercular treatment and referred because he showed no improvement. A high-resolution computed tomography of the chest revealed bilateral upper lobe bullae, parenchymal and subpleural fibrosis, and irregular pleural thickening. PPFE was found in surgical lung and pleural biopsies. He was given systemic glucocorticoids but did not respond clinically or radiologically. Pirfenidone and a lung transplant were out of reach for him. He died 9 months after being diagnosed with his condition. Finally, idiopathic PPFE is an extremely rare entity, with only three cases reported from our subcontinent. As a result, it is easily underdiagnosed or misdiagnosed; clinician awareness of this condition is critical for better diagnosis and management.
Thangakunam B, Isaac BTJ, Christopher DJ, Burad D. Idiopathic pleuroparenchymal fibroelastosis – a rare idiopathic interstitial pneumonia. Respir Med Case Rep 2016;17:8-11.
Maturu VN, Rajendran P, Narahari NK. Idiopathic pleuroparenchymal fibroelastosis presenting as bilateral spontaneous pneumothorax: a case report. Lung India 2019;36:75-7.
Bhaskar M, Tisekar O, Tandon S, et al. Rapidly progressive pleuro-parenchymal fibroelastosis secondary to cyclophosphamide chemotherapy. Indian J Med Paediatr Oncol 2020;41:427-9.
Fukada A, Suzuki Y, Mori K, et al. Idiopathic pleuroparenchymal fibroelastosis: three-dimensional computed tomography assessment of upper-lobe lung volume. Eur Respir J 2022;60:2200637.
Kinoshita Y, Kushima H, Ishii H. Idiopathic pleuroparenchymal fibroelastosis. BMJ Case Rep 2022;15:e251483.
Kinoshita Y, Miyamura T, Ikeda T, et al. Limited efficacy of nintedanib for idiopathic pleuroparenchymal fibroelastosis. Respir Investig 2022;60:562-9.
Boerner EB, Costabel U, Wessendorf TE, et al. Idiopathic pleuroparenchymal fibroelastosis (PPFE) – a case study of a rare entity. Rev Port Pneumol Engl Ed 2017;23:352-5.
Amitani R. Idiopathic pulmonary 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.
Konen E, Weisbrod GL, Pakhale S, et al. Fibrosis of the upper lobes: a newly identified late-onset complication after lung transplantation?. AJR Am J Roentgenol 2003;181:1539-43.
Jacob J, Odink A, Brun AL, et al. Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis. Respir Med 2018;138:95-101.
Enomoto Y, Nakamura Y, Colby TV, et al. Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease. PLoS One 2017;12:e0180283.
Xu L, Rassaei N, Caruso C. Pleuroparenchymal fibroelastosis with long history of asbestos and silicon exposure. Int J Surg Pathol 2018;26:190-3.
Reddy TL, Tominaga M, Hansell DM, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012;40:377-85.
Watanabe K, Ishii H, Kiyomi F, et al. Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: a proposal. Respir Investig 2019;57:312-20.
Cuppens K, Verbeken E, Coolen J, et al. Idiopathic pleuroparenchymatous fibroelastosis: a case report and brief review of the literature. Respir Med Case Rep 2014;12:7-9.
Becker CD, Gil J, Padilla ML. Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity?. Mod Pathol 2008;21:784-7.
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.
Sato S, Hanibuchi M, Takahashi M, et al. A patient with idiopathic pleuroparenchymal fibroelastosis showing a sustained pulmonary function due to treatment with pirfenidone. Intern Med 2016;55:497-501.
Nasser M, Chebib N, Philit F, et al. Treatment with nintedanib in patients with pleuroparenchymal fibroelastosis. Eur Respir J 2017;50:PA4876.
How to Cite
Garg, Shivam, Pratap Upadhya, Arul Kumar, Norton Stephen, and Bala Subramanian. 2023. “Overcoming the Challenges of a Misdiagnosed Rare Lung Disease – Idiopathic Pleuroparenchymal Fibroelastosis”. Monaldi Archives for Chest Disease 94 (2). https://doi.org/10.4081/monaldi.2023.2632.
Copyright (c) 2023 The Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.
Similar Articles
- Cristoforo Incorvaia, Fulvia Paterniti, Laura Pessina, Roberta Caligiuri, Franco Frati, Mona-Rita Yacoub, Gian Galeazzo Riario Sforza, Six-Minute Walk Test cut-off value identifying COPD patients with physical disability: a pilot study , Monaldi Archives for Chest Disease: Vol. 73 No. 4 (2010): Pulmonary series
- Neeta Singla, Amitesh Gupta, U.K. Khalid, Ravindra Kumar Dewan, Rupak Singla, Clinical profile, risk factors, disease severity, and outcome for COVID-19 disease in patients with tuberculosis on treatment under the National Tuberculosis Elimination Program: a cohort of 1400 patients , Monaldi Archives for Chest Disease: Early Access
- N. Barbarito, A. Vaghi, E. De Mattia, Prevalence of airflow obstruction according GOLD, ATS and ERS criteria in symptomatic ever-smokers referring to a Pulmonary Rehabilitation Department , Monaldi Archives for Chest Disease: Vol. 75 No. 3 (2011): Pulmonary series
- A.M. Moretti, S. Tafuri, D. Parisi, C. Germinario, Epidemiology of Pulmonary Embolism in Apulia from analysis of current data , Monaldi Archives for Chest Disease: Vol. 73 No. 1 (2010): Pulmonary series
- Raffaele Griffo, Antonio Spanevello, Pier Luigi Temporelli, Pompilio Faggiano, Mauro Carone, Giovanna Magni, Nicolino Ambrosino, Luigi Tavazzi, Italian survey on prevalence and disease management of chronic heart failure and chronic obstructive pulmonary disease comorbidity in ambulatory patients. SUSPIRIUM study rationale and design , Monaldi Archives for Chest Disease: Vol. 82 No. 1 (2014): Cardiac series
- D. Lykouras, F. Sampsonas, A. Kaparianos, K. Karkoulias, G. Tsoukalas, K. Spiropoulos, Human genes in TB infection: their role in immune response , Monaldi Archives for Chest Disease: Vol. 69 No. 1 (2008): Pulmonary series
- M. Vitacca, L. Bianchi, L. Barbano, N. Ambrosino, Lung and respiratory muscle function at discharge from a respiratory intensive care unit , Monaldi Archives for Chest Disease: Vol. 63 No. 3 (2005): Pulmonary series
- Tarun Krishna Boppana, Saurabh Mittal, Karan Madan, Anant Mohan, Vijay Hadda, Randeep Guleria, Tuberculosis endemicity and BCG vaccination: Protection against COVID-19 , Monaldi Archives for Chest Disease: Vol. 93 No. 1 (2023)
- Vera Auchynka, Ajay M.V. Kumar, Hennadz Hurevich, Yuliia Sereda, Varvara Solodovnikova, Dzmitry Katovich, Svetlana Setkina, Askar Yedilbayev, Aliaksandr Skrahin, Alena Skrahina, Effectiveness and cardiovascular safety of delamanid-containing regimens in adults with multidrug-resistant or extensively drug-resistant tuberculosis: A nationwide cohort study from Belarus, 2016-18 , Monaldi Archives for Chest Disease: Vol. 91 No. 1 (2021)
- Pankti Sheth, Shibdas Chakrabarti, Neeraj Kumar Gupta, Pranav Ish, Rohit Kumar, Nitesh Gupta, A middle-aged lady with near-fatal respiratory failure , Monaldi Archives for Chest Disease: Vol. 92 No. 3 (2022)
<< < 8 9 10 11 12 13 14 15 16 17 > >>
You may also start an advanced similarity search for this article.
