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Bilateral apical pulmonary fibrosis

MedGen UID:
1778266
•Concept ID:
C5539667
•
Disease or Syndrome
HPO: HP:0033539

Definition

A reticular pattern of linear or lineonodular densities in apical portions of both the right and left lungs seen initially on high-resolution computed tomography and in case of progression also on standard chest x-ray. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBilateral apical pulmonary fibrosis

Recent clinical studies

Therapy

Unilateral Upper Lung Field Pulmonary Fibrosis Radiologically Consistent with Pleuroparenchymal Fibroelastosis after Thoracotomy: A New Disease Entity Related to Thoracotomy.
Sekine A, Satoh H, Iwasawa T, Matsui K, Ikeya E, Ikeda S, Yamakawa H, Okuda R, Kitamura H, Shinohara T, Baba T, Komatsu S, Kato T, Hagiwara E, Ogura T
Respiration 2017;94(5):431-441. Epub 2017 Sep 1 doi: 10.1159/000479331. PMID: 28858871
A Patient with Idiopathic Pleuroparenchymal Fibroelastosis Showing a Sustained Pulmonary Function due to Treatment with Pirfenidone.
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
Long-term follow-up of pulmonary function in chronic eosinophilic pneumonia. Groupe d'Etude en Pathologie Interstitielle de la Société de Pathologie Thoracique du Nord.
Durieu J, Wallaert B, Tonnel AB
Eur Respir J 1997 Feb;10(2):286-91. doi: 10.1183/09031936.97.10020286. PMID: 9042622
Tuberculosis in liver transplant patients.
Meyers BR, Halpern M, Sheiner P, Mendelson MH, Neibart E, Miller C
Transplantation 1994 Aug 15;58(3):301-6. PMID: 8053051
Spontaneous pneumothorax in patients irradiated for Hodgkin's disease and other malignant lymphomas.
Pezner RD, Horak DA, Sayegh HO, Lipsett JA
Int J Radiat Oncol Biol Phys 1990 Jan;18(1):193-8. doi: 10.1016/0360-3016(90)90284-q. PMID: 2298622

Prognosis

Hermansky-Pudlak syndrome-associated pneumothorax with rapid progression of respiratory failure: a case report.
Kato Y, Kato M, Ihara H, Hayakawa E, Shibayama K, Miura K, Yamada T, Mitsuishi Y, Shukuya T, Ito J, Matsunaga T, Sato T, Suzuki K, Takahashi K
BMC Pulm Med 2020 Oct 6;20(1):259. doi: 10.1186/s12890-020-01302-8. PMID: 33023548Free PMC Article
Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study.
Enomoto Y, Nakamura Y, Satake Y, Sumikawa H, Johkoh T, Colby TV, Yasui H, Hozumi H, Karayama M, Suzuki Y, Furuhashi K, Fujisawa T, Enomoto N, Inui N, Iwashita T, Kuroishi S, Yokomura K, Koshimizu N, Toyoshima M, Imokawa S, Yamada T, Shirai T, Hayakawa H, Suda T
Respir Med 2017 Dec;133:1-5. Epub 2017 Nov 6 doi: 10.1016/j.rmed.2017.11.003. PMID: 29173442
Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease.
Enomoto Y, Nakamura Y, Colby TV, Johkoh T, Sumikawa H, Nishimoto K, Yoshimura K, Matsushima S, Oyama Y, Hozumi H, Kono M, Fujisawa T, Enomoto N, Inui N, Iwashita T, Suda T
PLoS One 2017;12(6):e0180283. Epub 2017 Jun 30 doi: 10.1371/journal.pone.0180283. PMID: 28666014Free PMC Article
Pulmonary manifestations of ankylosing spondylitis.
Kanathur N, Lee-Chiong T
Clin Chest Med 2010 Sep;31(3):547-54. doi: 10.1016/j.ccm.2010.05.002. PMID: 20692546
Bronchiectasis-related amyloidosis as a cause of chronic renal failure.
Akçay S, Akman B, Ozdemir H, Eyüboğlu FO, Karacan O, Ozdemir N
Ren Fail 2002 Nov;24(6):815-23. doi: 10.1081/jdi-120015683. PMID: 12472203

Clinical prediction guides

Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis.
Tetikkurt C, Kubat B, Kulahci C, Tetikkurt S, Caliskaner Ozturk B
Monaldi Arch Chest Dis 2021 Apr 6;91(3) doi: 10.4081/monaldi.2021.1713. PMID: 33840184
Unilateral Upper Lung Field Pulmonary Fibrosis Radiologically Consistent with Pleuroparenchymal Fibroelastosis after Thoracotomy: A New Disease Entity Related to Thoracotomy.
Sekine A, Satoh H, Iwasawa T, Matsui K, Ikeya E, Ikeda S, Yamakawa H, Okuda R, Kitamura H, Shinohara T, Baba T, Komatsu S, Kato T, Hagiwara E, Ogura T
Respiration 2017;94(5):431-441. Epub 2017 Sep 1 doi: 10.1159/000479331. PMID: 28858871
Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease.
Enomoto Y, Nakamura Y, Colby TV, Johkoh T, Sumikawa H, Nishimoto K, Yoshimura K, Matsushima S, Oyama Y, Hozumi H, Kono M, Fujisawa T, Enomoto N, Inui N, Iwashita T, Suda T
PLoS One 2017;12(6):e0180283. Epub 2017 Jun 30 doi: 10.1371/journal.pone.0180283. PMID: 28666014Free PMC Article
Pleural and parenchymal fibrosis mainly affecting the upper lung lobes in persons exposed to asbestos.
Hillerdal G
Respir Med 1990 Mar;84(2):129-34. doi: 10.1016/s0954-6111(08)80015-7. PMID: 2371434
Spontaneous pneumothorax in patients irradiated for Hodgkin's disease and other malignant lymphomas.
Pezner RD, Horak DA, Sayegh HO, Lipsett JA
Int J Radiat Oncol Biol Phys 1990 Jan;18(1):193-8. doi: 10.1016/0360-3016(90)90284-q. PMID: 2298622

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