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Pulmonary artery adventitial fibrosis

MedGen UID:
1787531
•Concept ID:
C5539582
•
Pathologic Function
HPO: HP:0033422

Definition

Increased amount of collagen, proteoglycans, and other extracellular matrix proteins resulting in scarring and thickening in the adventia of the pulmonary arterial tree. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary artery adventitial fibrosis

Recent clinical studies

Etiology

Pirfenidone Inhibits Hypoxic Pulmonary Hypertension through the NADPH/ROS/p38 Pathway in Adventitial Fibroblasts in the Pulmonary Artery.
Zhang S, Yin Z, Qin W, Ma X, Zhang Y, Liu E, Chu Y
Mediators Inflamm 2020;2020:2604967. Epub 2020 Jun 11 doi: 10.1155/2020/2604967. PMID: 32587469Free PMC Article
Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis.
Kinoshita Y, Ishii H, Kushima H, Johkoh T, Yabuuchi H, Fujita M, Nabeshima K, Watanabe K
Sci Rep 2020 Jan 15;10(1):306. doi: 10.1038/s41598-019-57248-3. PMID: 31941942Free PMC Article
Pulmonary veno-occlusive disease: An important consideration in patients with pulmonary hypertension.
Balko R, Edriss H, Nugent K, Test V
Respir Med 2017 Nov;132:203-209. Epub 2017 Oct 20 doi: 10.1016/j.rmed.2017.10.015. PMID: 29229098
Takayasu's arteritis: a cell-mediated large-vessel vasculitis.
Rizzi R, Bruno S, Stellacci C, Dammacco R
Int J Clin Lab Res 1999;29(1):8-13. doi: 10.1007/s005990050055. PMID: 10356657
Pathology of coronary arteries, myocardium, and great arteries in supravalvular aortic stenosis. Report of five cases with implications for surgical treatment.
van Son JA, Edwards WD, Danielson GK
J Thorac Cardiovasc Surg 1994 Jul;108(1):21-8. PMID: 8028375

Diagnosis

Clinical-imaging-pathological correlation in pulmonary hypertension associated with left heart disease.
van de Veerdonk MC, Roosma L, Trip P, Gopalan D, Vonk Noordegraaf A, Dorfmüller P, Nossent EJ
Eur Respir Rev 2024 Jan 31;33(171) Epub 2024 Feb 28 doi: 10.1183/16000617.0144-2023. PMID: 38417969Free PMC Article
Pulmonary veno-occlusive disease: An important consideration in patients with pulmonary hypertension.
Balko R, Edriss H, Nugent K, Test V
Respir Med 2017 Nov;132:203-209. Epub 2017 Oct 20 doi: 10.1016/j.rmed.2017.10.015. PMID: 29229098
Venous and arterial changes in pulmonary veno-occlusive disease, mitral stenosis and fibrosing mediastinitis.
Chazova I, Robbins I, Loyd J, Newman J, Tapson V, Zhdaov V, Meyrick B
Eur Respir J 2000 Jan;15(1):116-22. doi: 10.1183/09031936.00.15111600. PMID: 10678631
Takayasu's arteritis: a cell-mediated large-vessel vasculitis.
Rizzi R, Bruno S, Stellacci C, Dammacco R
Int J Clin Lab Res 1999;29(1):8-13. doi: 10.1007/s005990050055. PMID: 10356657
Takayasu's truncoarteritis. Pulseless disease or aortitis syndrome.
Nasu T
Acta Pathol Jpn 1982;32 Suppl 1:117-31. PMID: 6139930

Therapy

Pirfenidone Inhibits Hypoxic Pulmonary Hypertension through the NADPH/ROS/p38 Pathway in Adventitial Fibroblasts in the Pulmonary Artery.
Zhang S, Yin Z, Qin W, Ma X, Zhang Y, Liu E, Chu Y
Mediators Inflamm 2020;2020:2604967. Epub 2020 Jun 11 doi: 10.1155/2020/2604967. PMID: 32587469Free PMC Article
Smoking-Related "Interstitial" Lung Disease.
Franks TJ, Galvin JR
Arch Pathol Lab Med 2015 Aug;139(8):974-7. Epub 2014 Nov 21 doi: 10.5858/arpa.2013-0384-RA. PMID: 25415179
2-methoxyestradiol attenuates bleomycin-induced pulmonary hypertension and fibrosis in estrogen-deficient rats.
Tofovic SP, Zhang X, Jackson EK, Zhu H, Petrusevska G
Vascul Pharmacol 2009 Aug-Sep;51(2-3):190-7. Epub 2009 Jun 21 doi: 10.1016/j.vph.2009.06.002. PMID: 19540933Free PMC Article
Inhaled prostanoids in the therapy of pulmonary hypertension.
Gessler T, Seeger W, Schmehl T
J Aerosol Med Pulm Drug Deliv 2008 Mar;21(1):1-12. doi: 10.1089/jamp.2007.0657. PMID: 18518827
Coronary artery operation in radiation-associated atherosclerosis: long-term follow-up.
Hicks GL Jr
Ann Thorac Surg 1992 Apr;53(4):670-4. doi: 10.1016/0003-4975(92)90331-w. PMID: 1554280

Prognosis

Clinical-imaging-pathological correlation in pulmonary hypertension associated with left heart disease.
van de Veerdonk MC, Roosma L, Trip P, Gopalan D, Vonk Noordegraaf A, Dorfmüller P, Nossent EJ
Eur Respir Rev 2024 Jan 31;33(171) Epub 2024 Feb 28 doi: 10.1183/16000617.0144-2023. PMID: 38417969Free PMC Article
Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis.
Kinoshita Y, Ishii H, Kushima H, Johkoh T, Yabuuchi H, Fujita M, Nabeshima K, Watanabe K
Sci Rep 2020 Jan 15;10(1):306. doi: 10.1038/s41598-019-57248-3. PMID: 31941942Free PMC Article
Pulmonary veno-occlusive disease: An important consideration in patients with pulmonary hypertension.
Balko R, Edriss H, Nugent K, Test V
Respir Med 2017 Nov;132:203-209. Epub 2017 Oct 20 doi: 10.1016/j.rmed.2017.10.015. PMID: 29229098
Takayasu's arteritis: a cell-mediated large-vessel vasculitis.
Rizzi R, Bruno S, Stellacci C, Dammacco R
Int J Clin Lab Res 1999;29(1):8-13. doi: 10.1007/s005990050055. PMID: 10356657
Coronary artery operation in radiation-associated atherosclerosis: long-term follow-up.
Hicks GL Jr
Ann Thorac Surg 1992 Apr;53(4):670-4. doi: 10.1016/0003-4975(92)90331-w. PMID: 1554280

Clinical prediction guides

Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis.
Kinoshita Y, Ishii H, Kushima H, Johkoh T, Yabuuchi H, Fujita M, Nabeshima K, Watanabe K
Sci Rep 2020 Jan 15;10(1):306. doi: 10.1038/s41598-019-57248-3. PMID: 31941942Free PMC Article
Adventitial fibroblasts induce a distinct proinflammatory/profibrotic macrophage phenotype in pulmonary hypertension.
El Kasmi KC, Pugliese SC, Riddle SR, Poth JM, Anderson AL, Frid MG, Li M, Pullamsetti SS, Savai R, Nagel MA, Fini MA, Graham BB, Tuder RM, Friedman JE, Eltzschig HK, Sokol RJ, Stenmark KR
J Immunol 2014 Jul 15;193(2):597-609. Epub 2014 Jun 13 doi: 10.4049/jimmunol.1303048. PMID: 24928992Free PMC Article
Human RELMbeta is a mitogenic factor in lung cells and induced in hypoxia.
Renigunta A, Hild C, Rose F, Klepetko W, Grimminger F, Seeger W, Hänze J
FEBS Lett 2006 Feb 6;580(3):900-3. Epub 2006 Jan 18 doi: 10.1016/j.febslet.2006.01.012. PMID: 16427636
Takayasu's arteritis: a cell-mediated large-vessel vasculitis.
Rizzi R, Bruno S, Stellacci C, Dammacco R
Int J Clin Lab Res 1999;29(1):8-13. doi: 10.1007/s005990050055. PMID: 10356657
Pathology of coronary arteries, myocardium, and great arteries in supravalvular aortic stenosis. Report of five cases with implications for surgical treatment.
van Son JA, Edwards WD, Danielson GK
J Thorac Cardiovasc Surg 1994 Jul;108(1):21-8. PMID: 8028375

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