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
- Rahul Tyagi, Saurabh Mittal, Karan Madan, Anant Mohan, Vijay Hadda, Pawan Tiwari, Randeep Guleria, Assessment of the impact and reorganization of interventional pulmonology services at a tertiary care centre during nationwide lockdown for COVID-19 pandemic , Monaldi Archives for Chest Disease: Vol. 91 No. 1 (2021)
- Tarun Krishna Boppana, Saurabh Mittal, Karan Madan, Anant Mohan, Vijay Hadda, Pawan Tiwari, Randeep Guleria, Steroid therapy for COVID-19: A systematic review and meta-analysis of randomized controlled trials , Monaldi Archives for Chest Disease: Vol. 91 No. 4 (2021)
- Taymmia Ejaz, Mahmood Iqbal Malik, Jamal Ahmed, Rizwan Azam, Yousaf Jamal, Sheema Saadia, Clinico-radiological and bronchoscopic predictors of microbiological yield in sputum negative tuberculosis in Pakistan , Monaldi Archives for Chest Disease: Vol. 92 No. 3 (2022)
- Dario Amore, Emanuele Muto, Umberto Caterino, Dino Casazza, Alessandro Saglia, Pasquale Imitazione, Carlo Curcio, Anomalous segmental pulmonary vein: additional V6 behind the bronchus intermedius draining into the superior pulmonary vein , Monaldi Archives for Chest Disease: Vol. 92 No. 4 (2022)
- Sryma PB, Saurabh Mittal, Karan Madan, Anant Mohan, Pawan Tiwari, Vijay Hadda, Ravindra Mohan Pandey, Randeep Guleria, Awake prone positioning in non-intubated patients for the management of hypoxemia in COVID-19: A systematic review and meta-analysis , Monaldi Archives for Chest Disease: Vol. 91 No. 2 (2021)
- Parul Mrigpuri, Sonal Sonal, Sonam Spalgais, Nitin Goel, Balakrishnan Menon, Raj Kumar, Uncontrolled diabetes mellitus: A risk factor for post COVID fibrosis , Monaldi Archives for Chest Disease: Vol. 91 No. 1 (2021)
- Vivek Sharma, Pruthu Thekkur, Poonam R. Naik, Brojo Kishore Saha, Niraj Agrawal, Milan K. Dinda, S. Parthiban, Abhijit Dey, Basavaraj Poojar, Amrita N. Shamanewadi, Srinath Satyanarayana, Treatment success rates among tuberculosis patients notified from the private sector in West Bengal, India , Monaldi Archives for Chest Disease: Vol. 91 No. 1 (2021)
- Vijayarangam Narenchandra, Govindaraj Vishnukanth, Dharm Prakash Dwivedi, Munuswamy Hemachandren, Subathra Adithian, Saka Vinod Kumar, Manju Rajangam, Madhusmita Mohanty Mohapatra, Pratap Upadhya, Comparison of efficacy of autologous blood patch pleurodesis versus doxycycline pleurodesis in the management of persistent air leak in patients with secondary spontaneous pneumothorax. A randomized controlled trial , Monaldi Archives for Chest Disease: Vol. 92 No. 4 (2022)
- Ricardo Alves Pinto, Tânia Proença, Miguel Martins Carvalho, Sílvia Oliveira, Luis Adão, Filipe Macedo, Long-term prognosis of out-of-hospital cardiac arrest due to idiopathic ventricular arrhythmias , Monaldi Archives for Chest Disease: Vol. 93 No. 4 (2023)
- Dario Amore, Umberto Caterino, Dino Casazza, Riccardo Ievoli, Pasquale Imitazione, Alessandro Saglia, Alessandro Izzo, Marco Rispoli, Carlo Curcio, Persistent alveolar air leak following pulmonary lobectomy: an old problem in a modern era , Monaldi Archives for Chest Disease: Vol. 93 No. 4 (2023)
<< < 15 16 17 18 19 20 21 22 23 24 > >>
You may also start an advanced similarity search for this article.
