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Idiopathic pleuroparenchymal fibroelastosis

MedGen UID:
1798890
Concept ID:
C5567467
Disease or Syndrome
Synonyms: idiopathic pleuroparenchymal fibroelastosis; Idiopathic pleuropulmonary fibroelastosis; idiopathic pleuropulmonary fibroelastosis; IPPFE; IPPFE - idiopathic pleuroparenchymal fibroelastosis
SNOMED CT: Idiopathic pleuroparenchymal fibroelastosis (1172606002); Idiopathic pleuropulmonary fibroelastosis (1172606002); IPPFE - idiopathic pleuroparenchymal fibroelastosis (1172606002)
 
Monarch Initiative: MONDO:0044633
Orphanet: ORPHA494428

Definition

A rare idiopathic interstitial pneumonia with characteristics of prominent subpleural and parenchymal fibroelastosis and pleural fibrosis, predominantly involving the upper lobes. Signs and symptoms include non-productive cough, dyspnoea, and recurrent respiratory infections. Pneumothorax is a frequently reported complication. Pulmonary function test reveals a restrictive pattern and reduced diffusing capacity. Computed tomography shows pleural thickening with signs of fibrosis (traction bronchiectasis, architectural distortion and loss of volume) and reticulation. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic pleuroparenchymal fibroelastosis

Professional guidelines

PubMed

Kinoshita Y, Utsunomiya T, Wada K, Nakashima S, Kushima H, Ishii H
Respir Med Res 2023 Nov;84:101046. Epub 2023 Aug 18 doi: 10.1016/j.resmer.2023.101046. PMID: 37729671
Watanabe K, Ishii H, Kiyomi F, Terasaki Y, Hebisawa A, Kawabata Y, Johkoh T, Sakai F, Kondoh Y, Inoue Y, Azuma A, Suda T, Ogura T, Inase N, Homma S; Study Group on Diffuse Pulmonary Disorders, Scientific Research/Research on Intractable Diseases in Japan
Respir Investig 2019 Jul;57(4):312-320. Epub 2019 Apr 10 doi: 10.1016/j.resinv.2019.02.007. PMID: 30981683

Recent clinical studies

Etiology

Suzuki Y
Respir Investig 2025 Jan;63(1):127-137. Epub 2024 Dec 17 doi: 10.1016/j.resinv.2024.12.009. PMID: 39693846
Suzuki Y, Kono M, Hasegawa H, Hashimoto D, Yokomura K, Imokawa S, Inoue Y, Hozumi H, Karayama M, Furuhashi K, Enomoto N, Fujisawa T, Inui N, Suda T
BMJ Open Respir Res 2023 Dec 11;10(1) doi: 10.1136/bmjresp-2023-001763. PMID: 38081767Free PMC Article
Kinoshita Y, Miyamura T, Ikeda T, Ueda Y, Yoshida Y, Kushima H, Ishii H
Respir Investig 2022 Jul;60(4):562-569. Epub 2022 Apr 6 doi: 10.1016/j.resinv.2022.03.001. PMID: 35398003
Watanabe K, Ishii H, Kiyomi F, Terasaki Y, Hebisawa A, Kawabata Y, Johkoh T, Sakai F, Kondoh Y, Inoue Y, Azuma A, Suda T, Ogura T, Inase N, Homma S; Study Group on Diffuse Pulmonary Disorders, Scientific Research/Research on Intractable Diseases in Japan
Respir Investig 2019 Jul;57(4):312-320. Epub 2019 Apr 10 doi: 10.1016/j.resinv.2019.02.007. PMID: 30981683
Ryerson CJ, Collard HR
Curr Opin Pulm Med 2013 Sep;19(5):453-9. doi: 10.1097/MCP.0b013e328363f48d. PMID: 23872860

Diagnosis

Suzuki Y, Kono M, Hasegawa H, Hashimoto D, Yokomura K, Imokawa S, Inoue Y, Hozumi H, Karayama M, Furuhashi K, Enomoto N, Fujisawa T, Inui N, Suda T
BMJ Open Respir Res 2023 Dec 11;10(1) doi: 10.1136/bmjresp-2023-001763. PMID: 38081767Free PMC Article
Franquet T, Giménez Palleiro A
Radiologia (Engl Ed) 2022 Dec;64 Suppl 3:301-307. doi: 10.1016/j.rxeng.2022.12.002. PMID: 36737168
Kinoshita Y, Ishii H, Nabeshima K, Watanabe K
Histol Histopathol 2021 Mar;36(3):291-303. Epub 2020 Dec 14 doi: 10.14670/HH-18-289. PMID: 33315234
Zhang Y, Yang L, Li Y, Ma H, Zhang Y
Pulmonology 2021 Mar-Apr;27(2):186-187. Epub 2020 Dec 1 doi: 10.1016/j.pulmoe.2020.11.001. PMID: 33277197
Watanabe K, Ishii H, Kiyomi F, Terasaki Y, Hebisawa A, Kawabata Y, Johkoh T, Sakai F, Kondoh Y, Inoue Y, Azuma A, Suda T, Ogura T, Inase N, Homma S; Study Group on Diffuse Pulmonary Disorders, Scientific Research/Research on Intractable Diseases in Japan
Respir Investig 2019 Jul;57(4):312-320. Epub 2019 Apr 10 doi: 10.1016/j.resinv.2019.02.007. PMID: 30981683

Therapy

Garg S, Upadhya P, Kumar A, Stephen N, Subramanian B
Monaldi Arch Chest Dis 2023 Aug 2;94(2) doi: 10.4081/monaldi.2023.2632. PMID: 37545339
Fujisaki M, Higuchi T, Konda N, Hirahara S, Watanabe K, Yamada R, Motoyama R, Yamaguchi R, Katsumata Y, Kawaguchi Y, Harigai M
Mod Rheumatol Case Rep 2023 Dec 29;8(1):141-144. doi: 10.1093/mrcr/rxad035. PMID: 37307430
Yamagata A, Arita M, Tachibana H, Tokioka F, Sugimoto C, Sumikawa H, Tanaka T, Yasui H, Fujisawa T, Nakamura Y, Suda T, Ishida T
Respir Res 2021 Apr 20;22(1):115. doi: 10.1186/s12931-021-01726-8. PMID: 33879137Free PMC Article
Sato S, Hanibuchi M, Takahashi M, Fukuda Y, Morizumi S, Toyoda Y, Goto H, Nishioka Y
Intern Med 2016;55(5):497-501. Epub 2016 Mar 1 doi: 10.2169/internalmedicine.55.5047. PMID: 26935370
Huang Z, Li S, Zhu Y, Zhu H, Yi X
Int J Clin Exp Pathol 2015;8(7):8676-9. Epub 2015 Jul 1 PMID: 26339456Free PMC Article

Prognosis

Suzuki Y, Kono M, Hasegawa H, Hashimoto D, Yokomura K, Imokawa S, Inoue Y, Hozumi H, Karayama M, Furuhashi K, Enomoto N, Fujisawa T, Inui N, Suda T
BMJ Open Respir Res 2023 Dec 11;10(1) doi: 10.1136/bmjresp-2023-001763. PMID: 38081767Free PMC Article
Cueto-Robledo G, Guerrero-Velazquez JF, Roldan-Valadez E, Graniel-Palafox LE, Cervantes-Naranjo FD, Cueto-Romero HD, Rivera-Sotelo N
Curr Probl Cardiol 2022 Dec;47(12):101368. Epub 2022 Aug 24 doi: 10.1016/j.cpcardiol.2022.101368. PMID: 36028054
Kinoshita Y, Ishii H, Nabeshima K, Watanabe K
Histol Histopathol 2021 Mar;36(3):291-303. Epub 2020 Dec 14 doi: 10.14670/HH-18-289. PMID: 33315234
Suzuki Y, Fujisawa T, Sumikawa H, Tanaka T, Sugimoto C, Kono M, Hozumi H, Karayama M, Furuhashi K, Enomoto N, Nakamura Y, Inui N, Suda T
Respir Med 2020 Sep;171:106078. Epub 2020 Jul 11 doi: 10.1016/j.rmed.2020.106078. PMID: 32917352
Watanabe K, Ishii H, Kiyomi F, Terasaki Y, Hebisawa A, Kawabata Y, Johkoh T, Sakai F, Kondoh Y, Inoue Y, Azuma A, Suda T, Ogura T, Inase N, Homma S; Study Group on Diffuse Pulmonary Disorders, Scientific Research/Research on Intractable Diseases in Japan
Respir Investig 2019 Jul;57(4):312-320. Epub 2019 Apr 10 doi: 10.1016/j.resinv.2019.02.007. PMID: 30981683

Clinical prediction guides

Kinoshita Y, Miyamura T, Ikeda T, Ueda Y, Yoshida Y, Kushima H, Ishii H
Respir Investig 2022 Jul;60(4):562-569. Epub 2022 Apr 6 doi: 10.1016/j.resinv.2022.03.001. PMID: 35398003
Kono M, Nakamura Y, Enomoto Y, Yasui H, Hozumi H, Karayama M, Suzuki Y, Furuhashi K, Miki Y, Hashimoto D, Fujisawa T, Enomoto N, Inui N, Kaida Y, Yokomura K, Koshimizu N, Toyoshima M, Imokawa S, Yamada T, Shirai T, Hayakawa H, Nakamura H, Suda T
Respiration 2021;100(1):19-26. Epub 2021 Jan 5 doi: 10.1159/000511965. PMID: 33401281
Kinoshita Y, Ishii H, Nabeshima K, Watanabe K
Histol Histopathol 2021 Mar;36(3):291-303. Epub 2020 Dec 14 doi: 10.14670/HH-18-289. PMID: 33315234
Suzuki Y, Fujisawa T, Sumikawa H, Tanaka T, Sugimoto C, Kono M, Hozumi H, Karayama M, Furuhashi K, Enomoto N, Nakamura Y, Inui N, Suda T
Respir Med 2020 Sep;171:106078. Epub 2020 Jul 11 doi: 10.1016/j.rmed.2020.106078. PMID: 32917352
Watanabe K, Ishii H, Kiyomi F, Terasaki Y, Hebisawa A, Kawabata Y, Johkoh T, Sakai F, Kondoh Y, Inoue Y, Azuma A, Suda T, Ogura T, Inase N, Homma S; Study Group on Diffuse Pulmonary Disorders, Scientific Research/Research on Intractable Diseases in Japan
Respir Investig 2019 Jul;57(4):312-320. Epub 2019 Apr 10 doi: 10.1016/j.resinv.2019.02.007. PMID: 30981683

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