Enfermedades no tan huérfanas: fibroelastosis pleuropulmonar un desafío diagnóstico
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La fibroelastosis pleuropulmonar (FEPP) es una enfermedad poco frecuente dentro del espectro de las enfermedades pulmonares intersticiales difusas (EPI). Se clasifica como una neumonía intersticial idiopática rara, según la American Thoracic Society (ATS) y la European Respiratory Society (ERS). Histológicamente, se caracteriza por fibrosis de la pleura visceral asociada a cambios fibroelásticos en el parénquima pulmonar subpleural, con predominio en lóbulos superiores. Su incidencia real es desconocida, probablemente debido al subdiagnóstico y a la ausencia de criterios diagnósticos estandarizados. En este trabajo se reportan dos casos de FEPP en fase avanzada diagnosticados en un centro de Montevideo, Uruguay. Esta entidad representa un desafío diagnóstico y su reconocimiento oportuno es fundamental ya que podría ser más prevalente de lo que se percibe. Debe sospecharse ante pacientes con engrosamiento pleural asociado a compromiso intersticial de lóbulos superiores, lo cual justifica su pronta derivación a centros especializados. Dado su comportamiento progresivo, impacto negativo en la calidad de vida y elevada mortalidad, el tratamiento de la FEPP continúa siendo el principal desafío de esta entidad.
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Bonifazi M, Montero MA, Renzoni EA. Idiopathic Pleuroparenchymal Fibroelastosis. Curr Pulmonol Reports 2017;6(1):9–15. https://doi.org/10.1007/s13665-017-0160-5
Giménez A, Mazzini S, Franquet T. El informe radiológico en patología intersticial pulmonar. Radiologia 2022;64:142–9. https://doi.org/10.1016/j.rx.2022.01.007
Chua F, Desai SR, Nicholson AG, Devaraj A, Renzoni E, Rice A et al. Pleuroparenchymal fibroelastosis a review of clinical, radiological, and pathological characteristics. Ann Am Thorac Soc 2019;16(11):1351–9. https://doi.org/10.1513/AnnalsATS.201902-181CME
Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest 2004;126(6):2007–2013. https://doi.org/10.1378/chest.126.6.2007
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;2018:6043053. https://doi.org/10.1155/2018/6043053
Ishii H, Kinoshita Y, Kushima H, Nagata N, Watanabe K. The similarities and differences between pleuroparenchymal fibroelastosis and idiopathic pulmonary fibrosis. Chron Respir Dis 2019;16:1–9. https://doi.org/10.1177/1479973119867945
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;44:523–527. https://doi.org/10.1183/09031936.00214713
Enomoto Y, Nakamura Y, Colby TV, Johkoh T, Sumikawa H, Nishimoto K et al. Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease. PLoS One 2017;12:e0180283. https://doi.org/10.1371/journal.pone.0180283
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. https://doi.org/10.1016/j.rmed.2018.03.031
Watanabe K. Pleuroparenchymal fibroelastosis: its clinical characteristics. Curr Respir Med Rev 2013;9:299–237. https://doi.org/10.2174/1573398X0904140129125307
Xu L, Rassaei N, Caruso C. Pleuroparenchymal fibroelastosis with long history of asbestos and silicon exposure. Int J Surg Pathol 2018;26:190–193. https://doi.org/10.1177/1066896917739399
Piciucchi S, Tomassetti S, Casoni G, Sverzellati N, Carloni A, Dubini A et al. High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res 2011;12:111–115. https://doi.org/10.1186/1465-9921-12-111
Von der Thüsen JH. Pleuroparenchymal fibroelastosis: its pathological characteristics. Curr Respir Med Rev 2013;9:238–247. https://doi.org/10.2174/1573398X113096660025
Ricoy J, Suárez-Antelo J, Antúnez J, Martínez de Alegría A, Ferreiro L, Toubes ME et al. Pleuroparenchymal fibroelastosis: Clinical, radiological and histopathological features. Respir Med 2022;191:1–7. https://doi.org/10.1016/j.rmed.2021.106437
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. https://doi.org/10.1183/09031936.00165111
Morán Álvarez P, Bachiller-Corral J, Gorospe Sarasúa L, de la Puente Bujidos C. Fibroelastosis pleuroparenquimatosa: un nuevo tipo de neumonía intersticial asociada a conectivopatías. Reumatol Clínica 2020;16(6):513–4. https://doi.org/10.1016/j.reuma.2018.09.003
Bonifaci M, Sverzellati N, Negri E, Jacob J, Egashira R, Moser J et al. Pleurparenchymal fibroelastosis in systemic sclerosis: prevalence and prognostic impact. Eur Respir J 2020;56:1902135. https://doi.org/10.1183/13993003.02135-2019
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):1–10. https://doi.org/10.1186/s12931-022-02064-z
Kokosi MA, Nicholson AG, Hansell DM, Wells AU. Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP. Respirology 2016;21(4):600–14 https://doi.org/10.1111/resp.12693
Harada T, Yoshida Y, Kitasato Y, Tsuruta M, Wakamatsu K, Hirota T et al. The thoracic cage becomes flattened in the progression of pleuroparenchymal fibroelastosis. Eur Respir Rev 2014;23(132):263–6. https://doi.org/10.1183/09059180.00006713
Ishii H, Watanabea K, Kushimaa H, Babab T, Watanabec S, Yamadad Y. Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan. Resp Med 2018;141:190-197. https://doi.org/10.1016/j.rmed.2018.06.022
Rosenbaum JN, Butt YM, Johnson KA, Meyer K, Batra K, Kanne JP et al. Pleuroparenchymal fibroelastosis: a pattern of chronic lung injury. Hum Pathol 2015;46(1):137–46. https://doi.org/10.1016/j.humpath.2014.10.007
Yoshida Y, Nagata N, Tsuruta N, Kitasato Y, Wakamatsu K, Yoshimi M et al. Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis. Respir Investig 2016;54(3):162–9. https://doi.org/10.1016/j.resinv.2015.11.002
Becker CD, Gil J, Padilla ML. Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity? Mod Pathol 2008;21:784–787. https://doi.org/10.1038/modpathol.2008.56
Nasser M, Si-Mohamed S, Turquier S, Traclet J, Ahmad K, Philit F et al. Nintedanib in idiopathic and secondary pleuroparenchymal fibroelastosis. Orphanet J Rare Dis 2021;16(1):1–9. https://doi.org/10.1186/s13023-021-02043-5
Kinoshita Y, Ishii H, Nabeshima K, Watanabe K. The pathogenesis and pathology of idiopathic pleuroparenchymal fibroelastosis. Histol Histopathol 2021;36(3):291–303. https://doi.org/10.14670/HH-18-289
Kusagaya H, Nakamura Y, Kono M, Kaida Y, Kuroishi S. Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulmonary Medicine 2012;12:72. https://doi.org/10.1186/1471-2466-12-72
Richeldi L, Du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U et al. Efcacy and safety of nintedanib in idiopathic pulmonary fbrosis. N Eng J Med 2014;370:2071–82. https://doi.org/10.1056/NEJMoa1402584
Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Eng J Med 2019;380:2518–28. https://doi.org/10.1056/NEJMoa1903076
Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Eng J Med 2019;381:1718–27. https://doi.org/10.1056/NEJMoa1908681
Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M et al. Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med 2020;8:147–57. https://doi.org/10.1016/S2213-2600(19)30341-8
Sato S, Hanibuchi M, Takahashi M, Fukuda Y, Morizumi S, Toyoda Y et al. A patient with idiopathic pleuroparenchymal fibroelastosis showing a sustained pulmonary function due to treatment with pirfenidone. Intern Med 2016;55:497–501. https://doi.org/10.2169/internalmedicine.55.5047
Chen F, Matsubara K, Miyagawa-Hayashino A, Tada K, Handa T, Yamada T et al. Lung transplantation for pleuroparenchymal fibroelastosis after chemotherapy. Ann Thorac Surg 2014;98:e115–e117. https://doi.org/10.1016/j.athoracsur.2014.07.045
Righi I, Morlacchi L, Rossetti V, Mendogni P, Palleschi A, Tosi D et al. Lung transplantation as successful treatment of end-stage idiopathic pleuroparenchymal fibroelastosis: a case report. Transplant Proc 2019;51:235–238. https://doi.org/10.1016/j.transproceed.2018.04.071
Hata A, Nakajima T, Yoshida S, Kinoshita T, Terada J, Tatsumi K et al. Living donor lung transplantation for pleuroparenchymal fibroelastosis. Ann Thorac Surg 2016;101:1970–1972. https://doi.org/10.1016/j.athoracsur.2015.07.056
Ali MS, Ramalingam VS, Haasler G, Presberg K. Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature. BMJ Case Rep 2019;12(4). https://doi.org/10.1136/bcr-2019-229402
Cottin V, Si-Mohamed S, Diesler R, Bonniaud P, Valenzuela C. Pleuroparenchymal fibroelastosis. Curr Opin Pulm Med 2022;28(5):432–40. https://doi.org/10.1097/MCP.0000000000000907
Portillo K, Guasch Arriaga I, Ruiz-Manzano J. Fibroelastosis pleuropulmonar: ¿es también una entidad idiopática? Arch Bronconeumol 2015;51(10):509–14. https://doi.org/10.1016/j.arbres.2015.05.002