Pleuroparenchymal Fibroelastosis and Hypersensitivity Pneumonitis: A Clinical, Radiological and Pathological Overview

Main Article Content

Ana Luisa Fernandes Natália Melo Inês Neves Hélder Novais Bastos Patrícia Caetano Mota André Carvalho José Miguel Pereira Susana Guimarães Conceição Souto Moura António Morais


Non-idiopathic pleuroparenchymal fibroelastosis (PPFE) has been increasingly reported in the literature. Little is known about the clinical relevance of PFFE and hypersensitivity pneumonitis (HP) overlap; therefore, we sought to investigate the clinical, radiological, and pathological features of patients with these two diseases. Five patients were identified, and the detailed characterization of these cases revealed a heterogeneous group in terms of clinical and treatment options. No mortality, acute exacerbations, or a significant decline in lung function were verified. Our cases seem to have a more “benign” disease behavior, contrary to previous idiopathic PPFE studies. More studies are needed to corroborate these findings and to better elucidate the clinical significance of PPFE and HP overlap.

Article Details

How to Cite
FERNANDES, Ana Luisa et al. Pleuroparenchymal Fibroelastosis and Hypersensitivity Pneumonitis: A Clinical, Radiological and Pathological Overview. Medical Research Archives, [S.l.], v. 10, n. 7, july 2022. ISSN 2375-1924. Available at: <>. Date accessed: 08 aug. 2022. doi:
Case Reports


1. Travis WD, Costabel U, Hansell DM, 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. doi: 10.1164/rccm.201308-1483ST.
2. Reddy TL, Tominaga M, Hansell DM, et al. Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 2012;40(2):377–85. doi: 10.1183/09031936.00165111.
3. Oda T, Ogura T, Kitamura H, et al. Distinct characteristics of pleuroparenchymal fibroelastosis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis. Chest. 2014;146(5):1248–55. doi: 10.1378/chest.13-2866.
4. Nakatani T, Arai T, Kitaichi M, et al. Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? Eur Respir J. 2015;45:1183–1186. doi: 10.1183/09031936.00214714
5. Jacob J, Odink A, Brun AL et al. Functional associations of pleuroparenchymal fibroelastosis and emphysema with hypersensitivity pneumonitis. Respir Med. 2018;138:95-101. doi: 10.1016/j.rmed.2018.03.031.
6. Khiroya R, Macaluso C, Montero MA, et al. Pleuroparenchymal fibroelastosis: a review of histopathologic features and the relationship between histologic parameters and survival. Am. J. Surg. Pathol. 2017;41(12):1683-1689 doi: 10.1097/PAS.0000000000000928.
7. Azoulay E, Paugam B, Heymann M, et al. Familial extensive idiopathic bilateral pleural fibrosis. Eur Respir J. 1999;14(4):971-3. doi: 10.1034/j.1399-3003.1999.14d41.x
8. Cha YJ, Han J, Chung MP, Kim TJ, Shin S. Pleuroparenchymal fibroelastosis in heterogeneous clinical conditions: Clinicopathologic analysis of 7 cases. Clin Respir J. 2018 Apr;12(4):1495-1502. doi: 10.1111/crj.12696.
9. Carvalho J, Vieira AC, Ferra J, et al. Pleuroparenchymal Fibroelastosis in association with Connective Tissue Disease: a new interstitial pneumonia to be aware of. Acta Reumatol Port. 2019 Aug.
10. 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(6):e0180283. doi:10.1371/journal.pone.0180283
11. Mariani F, Gatti B, Rocca A, et al. Pleuroparenchymal fibroelastosis: the prevalence of secondary forms in hematopoietic stem cell and lung transplantation recipients. Diagn Interv Radiol. 2016;22(5): 400–406. doi: 10.5152/dir.2016.15516
12. Beynat-Mouterde C, Beltramo G, Lezmi G, et al. Pleuroparenchymal fibroelastosis as a late complication of chemotherapy agents. Eur Respir J. 2014;44(2):523–7. doi: 10.1183/09031936.00214713
13. Baroke E, Heussel CP, Warth A, et al. Pleuroparenchymal fibroelastosis in association with carcinomas. Respirology. 2016;21(1):191–4. doi: 10.1111/resp.12654
14. Silva JP, Melo N, Guimarães S, Morais A. Pleuroparenchymal fibroelastosis and Silicosis: na Unexpected Association. Arch Bronconeumol. 2018;54(10):529-531. doi: 10.1016/j.arbr.2018.02.026
15. Bonifazi M, Montero MA, Renzoni EA. Idiopathic Pleuroparenchymal Fibroelastosis. Curr Pulmonol Rep. 2017;6(1):9-15. doi: 10.1007/s13665-017-0160-5.
16. Enomoto Y, Nakamura Y, Satake Y, et al. Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study. Respir Med. 2017;133:1-5. doi: 10.1016/j.rmed.2017.11.003.
17. Watanabe K, Ishii H, Kiyomi F, et al. Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal. Respir Investig. 2019;57(4):312-320. doi: 10.1016/j.resinv.2019.02.007.
18. Nogueira R, Melo N, Bastos HN, et al. Hypersensitivity pneumonitis: Antigen diversity and disease implications. Pulmonology. 2019;25(2):97-108. doi: 10.1016/j.pulmoe.2018.07.003.
19. Vasakova M, Morell F, Walsh S, Leslie K, Raghu G. Hypersensitivity pneumonitis: perspectives in diagnosis and management. Am J Respir Crit Care Med. 2017;196(6):680---9. doi: 10.1164/rccm.201611-2201PP
20. Raghu G, Remy-Jardin M, Ryerson CJ, et al. Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline, Am J Respir Crit Care Med. 2020;202(3):e36-e69. doi: 10.1164/rccm.202005-2032ST.
21. Wuyts W, Sterclova M, Vasakova M. Pitfalls in diagnosis and management of hypersensitivity pneumonitis. Curr Opin Pulm Med. 2015;21:490---8. doi: 10.1097/MCP.0000000000000199.
22. Santos V, Martins N, Sousa C, et al. Hypersensitivity pneumonitis: Main features characterization in a Portuguese cohort, Pulmonology. 2020;26(3):130-137. doi: 10.1016/j.pulmoe.2019.09.004.
23. Morisset J, Johannson K, Jones K, et al. Identification of diagnostic criteria for chronic hypersensitivity pneumonitis: an international modified Delphi survey. Am J Respir Crit Care Med. 2017;197:1036---44. doi: 10.1164/rccm.201710-1986OC
24. Vasakova M, Selman M, Morell F, Sterclova M, Molina-Molina M, Raghu G. Hypersensitivity pneumonitis: Current concepts of pathogenesis and potential targets for treatment. Am J Respir Crit Care Med. 2019;200(3):301-308. doi: 10.1164/rccm.201903-0541PP.
25. Lima MS, Coletta EN, Ferreira RG, et al. Subacute and chronic hypersensitivity pneumonitis: histopathological patterns and survival. Respir Med. 2009;103(4):508–515. doi: 10.1016/j.rmed.2008.12.016.
26. Pereira C, Gimenez A, Kuranishi L, Storrer K. Chronic hypersensitivity pneumonitis. J Asthma Allergy. 2016;9:171---81. doi: 10.2147/JAA.S81540.
27. Cheng SK, Chuah KL. Pleuroparenchymal fibroelastosis of the lung: a review. Arch Pathol Lab Med. 2016;140(8):849–53. doi: 10.5858/arpa.2015-0166-RS.
28. Watanabe K. Pleuroparenchymal fibroelastosis: its clinical characteristics. Curr Resp Med Rev 2013; 9: 229-237. doi: 10.2174/1573398X0904140129125307.
29. Chua F, Desai SR, Nicholson AG, et al., Pleuroparenchymal Fibroelastosis: A Review of Clinical, Radiological and Pathological Characteristics Ann Am Thorac Soc. 2019;16(11):1351-1359. doi: 10.1513/AnnalsATS.201902-181CME
30. Margaritopoulos GA, Vasarmidi E, Jacob J, Wells AU, Antoniou KM. Smoking and interstitial lung diseases. Eur Respir Rev. 2015;24(137):428-35. doi: 10.1183/16000617.0050-2015.
31. Von der Thusen JH. Pleuroparenchymal fibroelastosis: its pathological characteristics. Curr Respir Med Rev. 2013;9(4):238–47. doi: 10.2174/1573398X113096660025.
32. Hirota T, Yoshida Y, Kitasato Y, et al. Histological evolution of pleuroparenchymal fibroelastosis. Histopathol 2015; 66: 545-554.
33. Kokkarinen JI, Tukiainen HO, Terho EO. Effect of corticosteroid treatment on the recovery of pulmonary function in farmer’s lung. Am Rev Respir Dis 1992;145:3–5. doi: 10.1164/ajrccm/145.1.3
34. Adegunsoye A, Oldham JM, Fernández Pérez ER et al. Outcomes of immunosuppressive therapy in chronic hypersensitivity pneumonitis. ERJ Open Res. 2017;3(3):0016. doi: 10.1183/23120541.00016-2017.
35. Morisset J, Johannson KA, Vittinghoff E, et al. Use of Mycophenolate Mofetil or Azathioprine for the Management of Chronic Hypersensitivity Pneumonitis. Chest. 2017;151(3):619-625. doi: 10.1016/j.chest.2016.10.029.
36. Alexandre AT, Martins N, Raimundo S et al., Impact of Azathioprine use in chronic hypersensitivity pneumonitis patients. Pulm Pharmacol Ther. 2020; 60:101878. doi: 10.1016/j.pupt.2019.101878
37. 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(5): 497–501. doi: 10.2169/internalmedicine.55.5047.
38. Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med. 2019;381(18):1718-1727. doi: 10.1056/NEJMoa1908681.