{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T12:09:29Z","timestamp":1747224569930,"version":"3.40.5"},"reference-count":20,"publisher":"PAGEPress Publications","license":[{"start":{"date-parts":[[2023,8,2]],"date-time":"2023-08-02T00:00:00Z","timestamp":1690934400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Monaldi Arch Chest Dis"],"abstract":"<jats:p>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 pleuroparenchymal fibroelastosis (IPPFE) from India, as well as the second antemortem diagnosis. A 27-year-old man presented with a one-year history of mMRC class II dry cough and shortness of breath. He described a fifteen-kilogram 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 nine months after being diagnosed with his condition. Finally, IPPFE 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.<\/jats:p>","DOI":"10.4081\/monaldi.2023.2632","type":"journal-article","created":{"date-parts":[[2023,8,2]],"date-time":"2023-08-02T07:07:58Z","timestamp":1690960078000},"source":"Crossref","is-referenced-by-count":0,"title":["Overcoming the challenges of a misdiagnosed rare lung disease - Idiopathic pleuroparenchymal fibroelastosis"],"prefix":"10.4081","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2331-9703","authenticated-orcid":false,"given":"Shivam","family":"Garg","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9303-9008","authenticated-orcid":false,"given":"Pratap","family":"Upadhya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arul","family":"Kumar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Norton","family":"Stephen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bala","family":"Subramanian","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2549","published-online":{"date-parts":[[2023,8,2]]},"reference":[{"key":"29453","doi-asserted-by":"crossref","unstructured":"Thangakunam B, Isaac BTJ, Christopher DJ, Burad D. Idiopathic pleuroparenchymal fibroelastosis \u2013 A rare idiopathic interstitial pneumonia. Respir Med Case Rep 2016;17:8-11.","DOI":"10.1016\/j.rmcr.2015.11.004"},{"key":"29454","doi-asserted-by":"crossref","unstructured":"Maturu VN, Rajendran P, Narahari NK. Idiopathic pleuroparenchymal fibroelastosis presenting as bilateral spontaneous pneumothorax: A case report. Lung India 2019;36:75.","DOI":"10.4103\/lungindia.lungindia_248_18"},{"key":"29455","doi-asserted-by":"crossref","unstructured":"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\u20139.","DOI":"10.4103\/ijmpo.ijmpo_85_19"},{"key":"29456","doi-asserted-by":"crossref","unstructured":"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.","DOI":"10.1183\/13993003.00637-2022"},{"key":"29457","doi-asserted-by":"crossref","unstructured":"Kinoshita Y, Kushima H, Ishii H. Idiopathic pleuroparenchymal fibroelastosis. BMJ Case Rep 2022;15:e251483.","DOI":"10.1136\/bcr-2022-251483"},{"key":"29458","doi-asserted-by":"crossref","unstructured":"Kinoshita Y, Miyamura T, Ikeda T, et al. Limited efficacy of nintedanib for idiopathic pleuroparenchymal fibroelastosis. Respir Investig 2022;60:562\u20139.","DOI":"10.1016\/j.resinv.2022.03.001"},{"key":"29459","doi-asserted-by":"crossref","unstructured":"Boerner EB, Costabel U, Wessendorf TE, et al. Idiopathic pleuroparenchymal fibroelastosis (PPFE) \u2013 A case study of a rare entity. Rev Port Pneumol Engl Ed 2017;23:352\u20135.","DOI":"10.1016\/j.rppnen.2017.06.006"},{"key":"29460","unstructured":"Amitani R. Idiopathic pulmonary upper lobe fibrosis (IPUF). Kokyu 1992;11:693."},{"key":"29461","doi-asserted-by":"crossref","unstructured":"Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest 2004;126:2007\u201313.","DOI":"10.1378\/chest.126.6.2007"},{"key":"29462","doi-asserted-by":"crossref","unstructured":"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\u201343.","DOI":"10.2214\/ajr.181.6.1811539"},{"key":"29463","doi-asserted-by":"crossref","unstructured":"Jacob J, Odink A, Brun AL, et al. Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis. Respir Med 2018;138:95\u2013101.","DOI":"10.1016\/j.rmed.2018.03.031"},{"key":"29464","doi-asserted-by":"crossref","unstructured":"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.","DOI":"10.1371\/journal.pone.0180283"},{"key":"29465","doi-asserted-by":"crossref","unstructured":"Xu L, Rassaei N, Caruso C. Pleuroparenchymal fibroelastosis with long history of asbestos and silicon exposure. Int J Surg Pathol 2018;26:190\u20133.","DOI":"10.1177\/1066896917739399"},{"key":"29466","doi-asserted-by":"crossref","unstructured":"Reddy TL, Tominaga M, Hansell DM, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J 2012;40:377\u201385.","DOI":"10.1183\/09031936.00165111"},{"key":"29467","doi-asserted-by":"crossref","unstructured":"Watanabe K, Ishii H, Kiyomi F, et al. Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal. Respir Investig 2019;57:312\u201320.","DOI":"10.1016\/j.resinv.2019.02.007"},{"key":"29468","doi-asserted-by":"crossref","unstructured":"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\u20139.","DOI":"10.1016\/j.rmcr.2013.12.005"},{"key":"29469","doi-asserted-by":"crossref","unstructured":"Becker CD, Gil J, Padilla ML. Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity? Mod Pathol 2008;21:784\u20137.","DOI":"10.1038\/modpathol.2008.56"},{"key":"29470","doi-asserted-by":"crossref","unstructured":"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":"10.1155\/2018\/6043053"},{"key":"29471","doi-asserted-by":"crossref","unstructured":"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\u2013501.","DOI":"10.2169\/internalmedicine.55.5047"},{"key":"29472","doi-asserted-by":"crossref","unstructured":"Nasser M, Chebib N, Philit F, et al. Treatment with nintedanib in patients with pleuroparenchymal fibroelastosis. 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