Amitani R, Niimi A, Kuse F. Idiopathic pulmonary upper lobe fibrosis. Kokyu. 1992;11:693–639.

Google Scholar 

Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 2004;126(6):2007–13.

Article  PubMed  Google Scholar 

Travis WD, Costabel U, Hansell DM, King TE, Lynch DA, Nicholson AG, 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(6):733–48.

Article  PubMed  PubMed Central  Google Scholar 

Shioya M, Otsuka M, Yamada G, Umeda Y, Ikeda K, Nishikiori H, et al. Poorer prognosis of idiopathic pleuroparenchymal fibroelastosis compared with idiopathic pulmonary fibrosis in advanced stage. Can Respir J. 2018;13(2018):1–7.

Article  Google Scholar 

Nakatani T, Arai T, Kitaichi M, Akira M, Tachibana K, Sugimoto C, et al. Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? Eur Respir J. 2015;45(4):1183–6.

Article  PubMed  Google Scholar 

Tanizawa K, Handa T, Kubo T, Chen-Yoshikawa TF, Aoyama A, Motoyama H, et al. Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: a retrospective cohort study. Respir Res. 2018;19(1):162.

Article  PubMed  PubMed Central  Google Scholar 

Lee SI, Chae EJ, Song JS, Lee JH, Song JW. Pleuroparenchymal fibroelastosis in patients with idiopathic pulmonary fibrosis. Respirology. 2020;25(10):1046–52.

Article  PubMed  Google Scholar 

Mariani F, Gatti B, Rocca A, Bonifazi F, Cavazza A, Fanti S, et al. Pleuroparenchymal fibroelastosis: the prevalence of secondary forms in hematopoietic stem cell and lung transplantation recipients. Diagn Interv Radiol. 2016;22(5):400–6.

Article  PubMed  PubMed Central  Google Scholar 

Pakhale S, Hadjiliadis D, Howell D, Palmer S, Gutierrez. Upper lobe fibrosis: a novel manifestation of chronic allograft dysfunction in lung transplantation. J Heart Lung Transplant. 2005;24(9):1260–8.

• Kang J, Seo WJ, Lee EY, Chang SH, Choe J, Hong S, et al. Pleuroparenchymal fibroelastosis in rheumatoid arthritis-associated interstitial lung disease. Respir Res. 2022;23(1):143. Details the incidence of PPFE in RA-ILD which is higher than previously appreciated.

• Sarı A, Önder Ö, Armağan B, Bölek EÇ, Farisoğulları B, Bilgin E, et al. Pleuroparenchymal fibroelastosis in systemic sclerosis-associated interstitial lung disease. Turk J Med Sci. 2022;52(1):83–8. Reports that PPFE is found in SSc with relatively high frequency considering the overall rarity of PPFE as a condition.

Thusen J. Pleuroparenchymal fibroelastosis: its pathological characteristics. Curr Respir Med Rev. 2014;9(4):238–47.

Article  Google Scholar 

Kinoshita Y, Ishii H, Watanabe K. The pathogenesis and pathology of idiopathic pleuroparenchymal fibroelastosis. Histol Histopathol. 2021;36(3):291–303.

PubMed  Google Scholar 

Kinoshita Y, Ishii H, Kushima H, Fujita M, Nabeshima K, Watanabe K. Role of alveolar collapse in idiopathic pleuroparenchymal fibroelastosis. Sarcoidosis Vasc Diffuse Lung Dis. 2020;37(2):212–7.

PubMed  PubMed Central  Google Scholar 

Zaizen Y, Tachibana Y, Kashima Y, Bychkov A, Tabata K, Otani K, et al. Alveolar epithelial denudation is a major factor in the pathogenesis of pleuroparenchymal fibroelastosis. J Clin Med. 2021;10(5):895.

Article  PubMed  PubMed Central  Google Scholar 

Fukuda Y, Ferrans VJ, Schoenberger CI. Patterns of pulmonary structural remodeling after experimental paraquat toxicitiy. The morphogenesis of intraalveolar fibrosis. Am J Pathol. 1985;118(3):452–75.

Nunes H, Jeny F, Bouvry D. Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations. Eur Respir J. 2017;49(5):1602022.

Article  PubMed  Google Scholar 

Newton CA, Batra K, Torrealba J, Meyer K, Raghu G, Garcia CK. Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations. Eur Respir J. 2017;49(5):1700696.

Article  PubMed  PubMed Central  Google Scholar 

Oo ZP, Bychkov A, Zaizen Y, Yamasue M, Kadota JI, Fukuoka J. Combination of pleuroparenchymal fibroelastosis with non-specific interstitial pneumonia and bronchiolitis obliterans as a complication of hematopoietic stem cell transplantation — clues to a potential mechanism. Respir Med Case Rep. 2019;26:244–7.

Higo H, Miyahara N, Taniguchi A, Maeda Y, Kiura K. Cause of pleuroparenchymal fibroelastosis following allogeneic hematopoietic stem cell transplantation. Respir Investig. 2019;57(4):321–4.

Article  PubMed  Google Scholar 

Kinoshita Y, Ishii H, Kushima H, Johkoh T, Yabuuchi H, Fujita M, et al. Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis. Sci Rep. 2020;10(1):306.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Von Der Thüsen JH, Hansell DM, Tominaga M, Veys PA, Ashworth MT, Owens CM, et al. Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation. Mod Pathol. 2011;24(12):1633–9.

Article  PubMed  Google Scholar 

Oh S lin, Lee JW, Yoo SY, Kim JH, Kim YJ, Han J, et al. Pleuroparenchymal fibroelastosis after hematopoietic stem cell transplantation in children: a propensity score–matched analysis. Eur Radiol. 2022;33(3):2266–76.

Bondeelle L, Gras J, Michonneau D, Houdouin V, Hermet E, Blin N, et al. Pleuroparenchymal fibroelastosis after allogeneic hematopoietic stem cell transplantation. Bone Marrow Transplant. 2020;55(5):982–6.

Article  PubMed  Google Scholar 

Aono Y, Hozumi H, Kono M, Hashimoto D, Nakamura H, Yokomura K, et al. Prognostic significance of radiological pleuroparenchymal fibroelastosis in Mycobacterium avium complex lung disease: a multicentre retrospective cohort study. Thorax. 2022;thoraxjnl-2022–219116.

Yamamoto Y, Tsujino K, Kuge T, Okabe F, Kawasaki T, Matsuki T, et al. Pleuroparenchymal fibroelastosis in Mycobacterium avium complex pulmonary disease: clinical characteristics and prognostic impact. ERJ Open Res. 2021;7(1):00765–2020.

Article  PubMed  PubMed Central  Google Scholar 

Reddy TL, Tominaga M, Hansell DM, Von Der Thusen J, Rassl D, Parfrey H, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 2012;40(2):377–85.

Article  PubMed  Google Scholar 

Khiroya R, Macaluso C, Montero MA, Wells AU, Chua F, Kokosi M, et al. Pleuroparenchymal fibroelastosis: a review of histopathologic features and the relationship between histologic parameters and survival. Am J Surg Pathol. 2017;41(12).

Xu L, Rassaei N, Caruso C. Pleuroparenchymal fibroelastosis with long history of asbestos and silicon exposure. Int J Surg Pathol. 2018;26(2):190–3.

Article  PubMed  Google Scholar 

Kaneko Y, Kikuchi N, Ishii Y, Kawabata Y, Moriyama H, Terada M, et al. Upper lobe-dominant pulmonary fibrosis showing deposits of hard metal component in the fibrotic lesions. Intern Med. 2010;49(19):2143–5.

Article  PubMed  Google Scholar 

Yabuuchi Y, Goto H, Nonaka M, Tachi H, Akiyama T, Arai N, et al. A case of airway aluminosis with likely secondary pleuroparenchymal fibroelastosis. Multidiscip Respir Med. 2019;14(1):15.

Article  PubMed  PubMed Central  Google Scholar 

Sekine A, Hagiwara E, Iwasawa T, Otoshi R, Erina T, Shintani R, et al. Asbestos exposure and tuberculous pleurisy as developmental causes of progressive unilateral upper-lung field pulmonary fibrosis radiologically consistent with pleuroparenchymal fibroelastosis. Respir Investig. 2021;59(6):837–44.

Article  CAS  PubMed  Google Scholar 

Silva JP, Melo N, Guimarães S, Morais A. Pleuroparenchymal fibroelastosis and silicosis: an unexpected association. Arch Bronconeumol. 2018;54(10):529–31.

Article  PubMed  Google Scholar 

Beynat-Mouterde C, Beltramo G, Lezmi G, Pernet D, Camus C, Fanton A, et al. Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents. Eur Respir J. 2014;44(2):523–7.

Article  PubMed  Google Scholar 

Rodrigues Vieira AC, Monteiro Ferra JR, Vicente De Carvalho JS, Melo N, Mota P, E Bastos H, et al. Pleuroparenchymal fibroelastosis as a complication of chemotherapy: a case report. Mol Clin Oncol. 2022;16(3):71.

Hamada K, Nagai S, KiTAICHI M, Jin G, Shigematsu M, Nagao T, et al. Cyclophosphamide-induced late-onset lung disease. Intern Med. 2003;42(1):82–7.

Article  PubMed  Google Scholar 

Oliveira M, Melo N, Mota PC, E Bastos HN, Pereira JM, Carvalho A, et al. Pleuroparenchymal fibroelastosis as another potential lung toxicity pattern induced by amiodarone. Arch Bronconeumol Engl Ed. 2020;56(1):54–6.

Kushima H, Ishii H, Kinoshita Y, Fujita M, Watanabe K. Chronic pulmonary aspergillosis with pleuroparenchymal fibroelastosis-like features. Intern Med. 2019;58(8):1137–40.

Article  PubMed  Google Scholar 

Enomoto Y, Nakamura Y, Satake Y, Sumikawa H, Johkoh T, Colby TV, et al. Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: a retrospective multicenter study. Respir Med. 2017;133:1–5.

Article  PubMed  Google Scholar 

Jacob J, Odink A, Brun AL, Macaluso C, De Lauretis A, Kokosi M, et al. Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis. Respir Med. 2018;138:95–101.

Article  PubMed  PubMed Central  Google Scholar 

Oda T, Ogura T, Kitamura H, Hagiwara E, Baba T, Enomoto Y, et al. Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis. Chest. 2014;146(5):1248–55.

Article  PubMed  Google Scholar 

Enomoto N, Kusagaya H, Oyama Y, Kono M, Kaida Y, Kuroishi S, et al. Quantitative analysis of lung elastic fibers in idiopathic pleuroparenchymal fibroelastosis (IPPFE): comparison of clinical, radiological, and pathological findings with those of idiopathic pulmonary fibrosis (IPF). BMC Pulm Med. 2014;14(1):91.

Article  PubMed  PubMed Central  Google Scholar 

Bonifazi M, Sverzellati N, Negri E, Jacob J, Egashira R, Moser J, et al. Pleuroparenchymal fibroelastosis in systemic sclerosis: prevalence and prognostic impact. Eur Respir J. 2020;56(1):1902135.

Article  PubMed  Google Scholar 

Orlandi M, Landini N, Bruni C, Sambataro G, Nardi C, Bargagli E, et al. Pleuroparenchymal fibroelastosis in rheumatic autoimmune diseases: a systematic literature review. Rheumatology. 2020;59(12):3645–56.

Article  PubMed  Google Scholar 

Amado C, Ferreira PG. Pleuroparenchymal fibroelastosis associated with Crohn’s disease: a new aetiology? Eur J Case Rep Intern Med. 2020;7(12):002017.

PubMed  PubMed Central  Google Scholar 

Sugino K, Ono H, Saito M, Igarashi S, Kurosaki A, Tsuboi E. Immunoglobulin G4-positive interstitial pneumonia associated with pleuroparenchymal fibroelastosis. Respirol Case Rep. 2022;10(4):e0925.

Article  PubMed  PubMed Central  Google Scholar 

Awano N, Izumo T, Fukuda K, Tone M, Yamada D, Takemura T, et al. Is hypothyroidism in idiopathic pleuroparenchymal fibroelastosis a novel lung-thyroid syndrome? Respir Investig. 2018;56(1):48–56.

Article