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Fibrotic non-specific interstitial pneumonia

MedGen UID:
1786141
•Concept ID:
C5539702
•
Disease or Syndrome
Synonym: Fibrotic-nonspecific interstitial pneumonia
 
HPO: HP:0033585

Definition

A type of non-specific interstitial pneumonia in which interstitial thickening is due to uniform dense or loose fibrosis and mild chronic inflammation. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFibrotic non-specific interstitial pneumonia

Professional guidelines

PubMed

Panel of serum biomarkers for differential diagnosis of idiopathic interstitial lung disease and interstitial lung disease-secondary to systemic autoimmune rheumatic disease.
d'Alessandro M, Cameli P, Cotton CV, Lamb JA, Bergantini L, Gangi S, Sugden S, Spencer LG, Frediani B, New RP, Chinoy H, Bargagli E, Conticini E
PLoS One 2024;19(10):e0311357. Epub 2024 Oct 3 doi: 10.1371/journal.pone.0311357. PMID: 39361584Free PMC Article
Practical management of Idiopathic Pulmonary Fibrosis.
Kishaba T
Sarcoidosis Vasc Diffuse Lung Dis 2015 Jul 22;32(2):90-8. PMID: 26278687

Recent clinical studies

Etiology

Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial.
Behr J, Prasse A, Kreuter M, Johow J, Rabe KF, Bonella F, Bonnet R, Grohe C, Held M, Wilkens H, Hammerl P, Koschel D, Blaas S, Wirtz H, Ficker JH, Neumeister W, Schönfeld N, Claussen M, Kneidinger N, Frankenberger M, Hummler S, Kahn N, Tello S, Freise J, Welte T, Neuser P, Günther A; RELIEF investigators
Lancet Respir Med 2021 May;9(5):476-486. Epub 2021 Mar 30 doi: 10.1016/S2213-2600(20)30554-3. PMID: 33798455
Prognostic factors and disease behaviour of pathologically proven fibrotic non-specific interstitial pneumonia.
Yamakawa H, Kitamura H, Takemura T, Ikeda S, Sekine A, Baba T, Iwasawa T, Hagiwara E, Sato S, Ogura T
Respirology 2018 Nov;23(11):1032-1040. Epub 2018 Apr 24 doi: 10.1111/resp.13313. PMID: 29693298
Confluent fibrosis and fibroblast foci in fibrotic non-specific interstitial pneumonia.
Churg A, Bilawich A
Histopathology 2016 Jul;69(1):128-35. Epub 2016 Mar 31 doi: 10.1111/his.12950. PMID: 26864142
Practical management of Idiopathic Pulmonary Fibrosis.
Kishaba T
Sarcoidosis Vasc Diffuse Lung Dis 2015 Jul 22;32(2):90-8. PMID: 26278687
Desquamative interstitial pneumonia.
Tazelaar HD, Wright JL, Churg A
Histopathology 2011 Mar;58(4):509-16. Epub 2010 Sep 21 doi: 10.1111/j.1365-2559.2010.03649.x. PMID: 20854463

Diagnosis

Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial.
Behr J, Prasse A, Kreuter M, Johow J, Rabe KF, Bonella F, Bonnet R, Grohe C, Held M, Wilkens H, Hammerl P, Koschel D, Blaas S, Wirtz H, Ficker JH, Neumeister W, Schönfeld N, Claussen M, Kneidinger N, Frankenberger M, Hummler S, Kahn N, Tello S, Freise J, Welte T, Neuser P, Günther A; RELIEF investigators
Lancet Respir Med 2021 May;9(5):476-486. Epub 2021 Mar 30 doi: 10.1016/S2213-2600(20)30554-3. PMID: 33798455
Cicatricial organising pneumonia mimicking a fibrosing interstitial pneumonia.
Churg A, Wright JL, Bilawich A
Histopathology 2018 Apr;72(5):846-854. Epub 2018 Jan 29 doi: 10.1111/his.13443. PMID: 29193209
Confluent fibrosis and fibroblast foci in fibrotic non-specific interstitial pneumonia.
Churg A, Bilawich A
Histopathology 2016 Jul;69(1):128-35. Epub 2016 Mar 31 doi: 10.1111/his.12950. PMID: 26864142
Practical management of Idiopathic Pulmonary Fibrosis.
Kishaba T
Sarcoidosis Vasc Diffuse Lung Dis 2015 Jul 22;32(2):90-8. PMID: 26278687
Desquamative interstitial pneumonia.
Tazelaar HD, Wright JL, Churg A
Histopathology 2011 Mar;58(4):509-16. Epub 2010 Sep 21 doi: 10.1111/j.1365-2559.2010.03649.x. PMID: 20854463

Therapy

Ixekizumab-induced interstitial lung disease: a case report confirmed by transbronchial lung cryobiopsy.
Kaburaki S, Okada N, Tanaka T, Kamio K, Tanaka Y, Terasaki Y, Kasahara K, Seike M
J Dermatolog Treat 2024 Dec;35(1):2424338. Epub 2024 Nov 10 doi: 10.1080/09546634.2024.2424338. PMID: 39523594
Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial.
Behr J, Prasse A, Kreuter M, Johow J, Rabe KF, Bonella F, Bonnet R, Grohe C, Held M, Wilkens H, Hammerl P, Koschel D, Blaas S, Wirtz H, Ficker JH, Neumeister W, Schönfeld N, Claussen M, Kneidinger N, Frankenberger M, Hummler S, Kahn N, Tello S, Freise J, Welte T, Neuser P, Günther A; RELIEF investigators
Lancet Respir Med 2021 May;9(5):476-486. Epub 2021 Mar 30 doi: 10.1016/S2213-2600(20)30554-3. PMID: 33798455
Cicatricial organising pneumonia mimicking a fibrosing interstitial pneumonia.
Churg A, Wright JL, Bilawich A
Histopathology 2018 Apr;72(5):846-854. Epub 2018 Jan 29 doi: 10.1111/his.13443. PMID: 29193209
Practical management of Idiopathic Pulmonary Fibrosis.
Kishaba T
Sarcoidosis Vasc Diffuse Lung Dis 2015 Jul 22;32(2):90-8. PMID: 26278687
Desquamative interstitial pneumonia.
Tazelaar HD, Wright JL, Churg A
Histopathology 2011 Mar;58(4):509-16. Epub 2010 Sep 21 doi: 10.1111/j.1365-2559.2010.03649.x. PMID: 20854463

Prognosis

Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial.
Behr J, Prasse A, Kreuter M, Johow J, Rabe KF, Bonella F, Bonnet R, Grohe C, Held M, Wilkens H, Hammerl P, Koschel D, Blaas S, Wirtz H, Ficker JH, Neumeister W, Schönfeld N, Claussen M, Kneidinger N, Frankenberger M, Hummler S, Kahn N, Tello S, Freise J, Welte T, Neuser P, Günther A; RELIEF investigators
Lancet Respir Med 2021 May;9(5):476-486. Epub 2021 Mar 30 doi: 10.1016/S2213-2600(20)30554-3. PMID: 33798455
Prognostic factors and disease behaviour of pathologically proven fibrotic non-specific interstitial pneumonia.
Yamakawa H, Kitamura H, Takemura T, Ikeda S, Sekine A, Baba T, Iwasawa T, Hagiwara E, Sato S, Ogura T
Respirology 2018 Nov;23(11):1032-1040. Epub 2018 Apr 24 doi: 10.1111/resp.13313. PMID: 29693298
Confluent fibrosis and fibroblast foci in fibrotic non-specific interstitial pneumonia.
Churg A, Bilawich A
Histopathology 2016 Jul;69(1):128-35. Epub 2016 Mar 31 doi: 10.1111/his.12950. PMID: 26864142
Practical management of Idiopathic Pulmonary Fibrosis.
Kishaba T
Sarcoidosis Vasc Diffuse Lung Dis 2015 Jul 22;32(2):90-8. PMID: 26278687
Desquamative interstitial pneumonia.
Tazelaar HD, Wright JL, Churg A
Histopathology 2011 Mar;58(4):509-16. Epub 2010 Sep 21 doi: 10.1111/j.1365-2559.2010.03649.x. PMID: 20854463

Clinical prediction guides

Lung transplantation for interstitial lung disease: evolution over three decades.
Leong SW, Bos S, Lordan JL, Nair A, Fisher AJ, Meachery G
BMJ Open Respir Res 2023 Feb;10(1) doi: 10.1136/bmjresp-2022-001387. PMID: 36854571Free PMC Article
Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo-controlled, phase 2b trial.
Behr J, Prasse A, Kreuter M, Johow J, Rabe KF, Bonella F, Bonnet R, Grohe C, Held M, Wilkens H, Hammerl P, Koschel D, Blaas S, Wirtz H, Ficker JH, Neumeister W, Schönfeld N, Claussen M, Kneidinger N, Frankenberger M, Hummler S, Kahn N, Tello S, Freise J, Welte T, Neuser P, Günther A; RELIEF investigators
Lancet Respir Med 2021 May;9(5):476-486. Epub 2021 Mar 30 doi: 10.1016/S2213-2600(20)30554-3. PMID: 33798455
Prognostic factors and disease behaviour of pathologically proven fibrotic non-specific interstitial pneumonia.
Yamakawa H, Kitamura H, Takemura T, Ikeda S, Sekine A, Baba T, Iwasawa T, Hagiwara E, Sato S, Ogura T
Respirology 2018 Nov;23(11):1032-1040. Epub 2018 Apr 24 doi: 10.1111/resp.13313. PMID: 29693298
Practical management of Idiopathic Pulmonary Fibrosis.
Kishaba T
Sarcoidosis Vasc Diffuse Lung Dis 2015 Jul 22;32(2):90-8. PMID: 26278687
Decreased interstitial FOXP3(+) lymphocytes in usual interstitial pneumonia with discrepancy of CXCL12/CXCR4 axis.
Shimizu Y, Dobashi K, Endou K, Ono A, Yanagitani N, Utsugi M, Sano T, Ishizuka T, Shimizu K, Tanaka S, Mori M
Int J Immunopathol Pharmacol 2010 Apr-Jun;23(2):449-61. doi: 10.1177/039463201002300207. PMID: 20646340

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