Fibroelastosis pleuroparenquimatosa e hipertension pulmonar: un fenotipo diferente?

Autores/as

  • Lourdes González Ministerio de Salud Pública y Bienestar Social. Hospital General de Luque. Luque, Paraguay
  • Federico Sosa Ministerio de Salud Pública y Bienestar Social. Hospital General de Luque. Luque, Paraguay
  • Diana Florentín Ministerio de Salud Pública y Bienestar Social. Hospital General de Luque. Luque, Paraguay
  • Domingo Pérez Bejarano Ministerio de Salud Pública y Bienestar Social. Hospital General de Luque. Luque, Paraguay

DOI:

https://doi.org/10.53732/rccsalud/2023.e5208

Palabras clave:

enfermedades pulmonares intersticiales, fibrosis pulmonar, enfermedades pleurales, fibroelastosis pleuroparenquimatosa

Resumen

La fibroelastosis pleuroparenquimatosa es una enfermedad pulmonar inusual con características clínicas, radiológicas y patológicas únicas, que se ha incluido recientemente en el consenso actualizado sobre neumonías intersticiales idiopáticas. Su nombre hace referencia a una combinación de fibrosis que involucra la pleura visceral y cambios fibroelastóticos que predominan en el parénquima pulmonar subpleural. Aunque se han descrito varias asociaciones de enfermedades, no se ha identificado de manera inequívoca ninguna causa única. El diagnostico se sustenta en criterios clínicos-radiológicos y de hallazgos histopatológicos cuando se dispone de biopsias. Son escasos los reportes sobre la asociación entre esta entidad y la hipertensión pulmonar. No existe tratamiento hasta la fecha, aunque se relatan sucesos en algunos casos de transplante pulmonar. Describimos las características clínicas de un paciente atendido en un hospital del Paraguay.

Citas

Buschulte K, Cottin V, Wijsenbeek M, Kreuter M, Diesler R. The world of rare interstitial lung diseases. EurRespir Rev. 2023;32(167):220161. https://doi.org/10.1183/16000617.0161-2022

Nakatani T, Arai T, Kitaichi M, Akira M, Tachibana K, Sugimoto Ch. Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity? EurRespir J. 2015;45(4):1183-6. https://doi.org/10.1183/09031936.00214714

Davies D, Crowther JS, MacFarlane A. Idiopathic progressive pulmonary fibrosis. Thorax. 1975;30(3):316-25. https://doi.org/10.1136/thx.30.3.316

Watanabe K, Ishii H, Kiyomi F, Terasaki Y, Hebisawa A, Kawabata Y, et al. Study Group on Diffuse Pulmonary Disorders, Scientific Research/Research on Intractable Diseases in Japan. Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal. Respir Investig. 2019;57(4):312-320. https://doi.org/10.1016/j.resinv.2019.02.007

Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 2004;126(6):2007-13. https://doi.org/10.1378/chest.126.6.2007

Travis WD, Costabel U, Hansell DM, King Jr TE, Lynch DA, Nicholson AG. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J RespirCrit Care Med. 2013;188(6):733-48. https://pubmed.ncbi.nlm.nih.gov/24032382/

Gomes PS, Shiang C, Szarf G, Aparecida Martins Coletta EN, Castro Pereira CA. Pleuroparenchymal fibroelastosis: report of two cases in Brazil. J Bras Pneumol. 2017;43(1):72-75. https://doi.org/10.1590/S1806-37562016000000045

Atag E, Bas Ikizoglu N, Gokdemir Y, Erdem Eralp E, Kiyan G, Yilmazbayhan D, et al. Adolescente con fibroelastosis pleuroparenquimatosa idiopática. A propósito de un caso. Arch Argent Pediatr. 2018;116(1):e147-e150. http://www.scielo.org.ar/pdf/aap/v116n1/v116n1a40.pdf

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;191:106437. https://doi.org/10.1016/j.rmed.2021.106437

Fujisawa T, Horiike Y, Egashira R, Sumikawa H, Iwasawa T, Matsushita Sh, et al. Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias. Respir Res. 2021;22(1):290. https://doi.org/10.1186/s12931-021-01892-9

Newton CA, Batra K, Torrealba J y cols. Pleuroparenchymal fibroelastosis associated with telomerase reverse transcriptase mutations. Eur Respir J. 2017;49(5):1700696. https://doi.org/110.1183/13993003.00696-2017

Orlandi M, Landini N, Bruni C, Sambataro G, Cosimo Nardi C, Bargagli E. Pleuroparenchymal fibroelastosis in rheumatic autoimmune diseases: a systematic literature review. Rheumatology (Oxford). 2020;59(12):3645-3656. https://doi.org/10.1093/rheumatology/keaa451

Enomoto Y, Nakamura Y, Colby TV, Takeshi Johkoh, Hiromitsu Sumikawa H, Nishimoto K, et al. Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease. PLoS One. 2017;12(6):e0180283. https://doi.org/10.1371/journal.pone.0180283

Gudmundsson E, Zhao A, Mogulkoc N, Stewart I, Jones MG, Van Moorsel C. H. M, et al. Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment. EClinicalMedicine. 2021;38:101009. https://doi.org/10.1016/j.eclinm.2021.101009

Kurosaki F, Bando M, Nakayama M, Mato N, Nakaya T, Yamasawa H, et al. Clinical features of pulmonary aspergillosis associated with interstitial pneumonia. Intern Med. 2014;53(12):1299-306. https://pubmed.ncbi.nlm.nih.gov/24930648/

Kushima H, Ishii H, Kinoshita Y, Fujita M, Kentaro Watanabe K. Chronic Pulmonary Aspergillosis with Pleuroparenchymal Fibroelastosis-like Features. Intern Med. 2019;58(8):1137-1140. https://doi.org/10.2169/internalmedicine.1930-18

Kono M, Nakamura Y, Enomoto Y, Yasui H; Hozumi H; Karayama M, et al. Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors. Respiration. 2021;100(1):19-26. https://doi.org/10.1159/000511965

Chua F, Desai SR, Nicholson AG, Devaraj A, Renzoni E, Alexandra Rice A, et al. Pleuroparenchymal Fibroelastosis. A Review of Clinical, Radiological, and Pathological Characteristics. Ann Am Thorac Soc. 2019;16(11):1351-1359. https://pubmed.ncbi.nlm.nih.gov/31425665/

Watanabe K. Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics. Curr Respir Med Rev. 2013;9(4):299-237. https://doi.org/10.2174/1573398X0904140129125307

Yoshida Y, Nagata N, Tsuruta Net, 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

Kato M, Sasaki S, Kurokawa K, Nakamura T; Yamada T; Sasano. H, et al. Usual Interstitial Pneumonia Pattern in the Lower Lung Lobes as a Prognostic Factor in Idiopathic Pleuroparenchymal Fibroelastosis. Respiration. 2019;97(4):319-328. https://doi.org/10.1159/000494061

Dhont S, Zwaenepoel B, Vandecasteele E, Brusselle G, De Pauw M. Pulmonary hypertension in interstitial lung disease: an area of unmet clinical need. ERJ Open Res. 2022;8(4):00272-2022. https://doi.org/10.1183/23120541.00272-2022

Gupta R, Judson MA, Baughman RP. Management of Advanced Pulmonary Sarcoidosis. Am J RespirCrit Care Med. 2022;205(5):495-506. https://pubmed.ncbi.nlm.nih.gov/34813386/

Boerner EB, Costabel U, Wessendorf TE, Theegarte D, Bonella F. Idiopathic pleuroparenchymal fibroelastosis (PPFE) - A case study of a rare entity. Rev Port Pneumol (2006). 2017;23(6):352-355. https://doi.org/10.1016/j.rppnen.2017.06.006

Kinoshita Y, Watanabe K, Ishii H, Kushima H, Nabeshima K. Lower-lobe predominant pleuroparenchymal fibroelastosis. Pathol Int. 2019;69(9):536-540. https://pubmed.ncbi.nlm.nih.gov/31273873/

Muto Y, Sekine A, Hagiwara E, Komatsu Sh, Baba T, Oda T, et al. Clinical characteristics of pulmonary hypertension in patients with pleuroparenchymalfibroelastosis. RespirInvestig. 2022;60(4):543-550. https://doi.org/10.1016/j.resinv.2022.02.005

Khiroya R, Macaluso C, Montero MA, Montero M; Wells A; Chua F, et al. Pleuroparenchymal Fibroelastosis: A Review of Histopathologic Features and the Relationship Between Histologic Parameters and Survival. Am J SurgPathol.

;41(12):1683-1689. https://doi.org/10.1097/PAS.0000000000000928

Farkas L, Gauldie J, Voelkel NF, Kolb M. Pulmonary hypertension and idiopathic pulmonary fibrosis: a tale of angiogenesis, apoptosis, and growth factors. Am J Respir Cell Mol Biol. 2011;45(1):1-15. https://pubmed.ncbi.nlm.nih.gov/21057104/

Nikkho SM, Richter MJ, Shen E, Abman S, Antoniou K, Chung J, et al. Clinical significance of pulmonary hypertension in interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's innovative drug development initiative-Group 3 pulmonary hypertension. Pulm Circ. 2022;12(3):e12127. https://pubmed.ncbi.nlm.nih.gov/36016668/

Tandon R, Baughman RP, Stanley J, A Khan A. The link between chronic thromboembolic pulmonary hypertension and sarcoidosis: association or visual masquerade? Sarcoidosis Vasc Diffuse Lung Dis. 2017;34(4):352-355. https://doi.org/10.36141/svdld.v34i4.5852

Sobiecka M, Szturmowicz M, Lewandowska K, Kowalik A, Łyżwa E, Zimna K, et al. Chronic hypersensitivity pneumonitis is associated with an increased risk of venous thromboembolism: a retrospective cohort study. BMC Pulm Med. 2021;21(1):416. https://doi.org/10.1186/s12890-021-01794-y

Sugino K, Ono H, Shimizu H, Kurosawa T, Matsumoto K, Ando M, et al. Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia. ERJ Open Res. 2021;7(1):00196-2020. https://doi.org/10.1183/23120541.00196-2020

Descargas

Publicado

2023-12-23

Cómo citar

González, L., Sosa, F., Florentín, D., & Pérez Bejarano, D. (2023). Fibroelastosis pleuroparenquimatosa e hipertension pulmonar: un fenotipo diferente?. Revista científica Ciencias De La Salud, 5, 01–07. https://doi.org/10.53732/rccsalud/2023.e5208