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Idiopathic bronchiectasis

MedGen UID:
573462
Concept ID:
C0339985
Disease or Syndrome
Synonym: idiopathic bronchiectasis
SNOMED CT: Idiopathic bronchiectasis (233629001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Related genes: SCNN1G, SCNN1B, SCNN1A, CFTR
 
Monarch Initiative: MONDO:0018956
Orphanet: ORPHA60033

Definition

Idiopathic bronchiectasis (IB) is a progressive lung disease characterized by chronic dilation of the bronchi and destruction of the bronchial walls in the absence of any underlying cause (such as post infectious disease, aspiration, immunodeficiency, congenital abnormalities and ciliary anomalies). [from ORDO]

Professional guidelines

PubMed

Abid N, Loukil M, Mokni A, Badri I, Bouzaidi K, Ghrairi H
Tunis Med 2021 Feb;99(2):264-268. PMID: 33899197Free PMC Article
Goussault H, Salvator H, Catherinot E, Chabi ML, Tcherakian C, Chabrol A, Didier M, Rivaud E, Fischer A, Suarez F, Hermine O, Lanternier F, Lortholary O, Mahlaoui N, Devillier P, Couderc LJ
Respir Res 2019 Dec 4;20(1):275. doi: 10.1186/s12931-019-1242-4. PMID: 31801528Free PMC Article
Rosen MJ
Chest 2006 Jan;129(1 Suppl):122S-131S. doi: 10.1378/chest.129.1_suppl.122S. PMID: 16428701

Recent clinical studies

Etiology

Perluk T, Abu Bandora E, Freund O, Jacob T, Friedman Regev I, Kleinhendler E, Shteinberg M, Bar-Shai A, Oestriecher-Kedem Y
Lung 2024 Apr;202(2):189-195. Epub 2024 Mar 18 doi: 10.1007/s00408-024-00683-5. PMID: 38499811Free PMC Article
Yu I, Yong SJ, Lee WY, Kim SH, Lee H, Na JO, Kim DK, Oh YM, Lee JH; KMBARC
Korean J Intern Med 2022 Sep;37(5):1002-1010. Epub 2022 Aug 18 doi: 10.3904/kjim.2022.070. PMID: 35977811Free PMC Article
Nikolic A
Lung 2018 Aug;196(4):383-392. Epub 2018 May 12 doi: 10.1007/s00408-018-0121-y. PMID: 29754320
Olveira C, Padilla A, Martínez-García MÁ, de la Rosa D, Girón RM, Vendrell M, Máiz L, Borderías L, Polverino E, Martínez-Moragón E, Rajas O, Casas F, Cordovilla R, de Gracia J
Arch Bronconeumol 2017 Jul;53(7):366-374. Epub 2017 Jan 21 doi: 10.1016/j.arbres.2016.12.003. PMID: 28118936
Lonni S, Chalmers JD, Goeminne PC, McDonnell MJ, Dimakou K, De Soyza A, Polverino E, Van de Kerkhove C, Rutherford R, Davison J, Rosales E, Pesci A, Restrepo MI, Torres A, Aliberti S
Ann Am Thorac Soc 2015 Dec;12(12):1764-70. doi: 10.1513/AnnalsATS.201507-472OC. PMID: 26431397Free PMC Article

Diagnosis

Perluk T, Abu Bandora E, Freund O, Jacob T, Friedman Regev I, Kleinhendler E, Shteinberg M, Bar-Shai A, Oestriecher-Kedem Y
Lung 2024 Apr;202(2):189-195. Epub 2024 Mar 18 doi: 10.1007/s00408-024-00683-5. PMID: 38499811Free PMC Article
Yu I, Yong SJ, Lee WY, Kim SH, Lee H, Na JO, Kim DK, Oh YM, Lee JH; KMBARC
Korean J Intern Med 2022 Sep;37(5):1002-1010. Epub 2022 Aug 18 doi: 10.3904/kjim.2022.070. PMID: 35977811Free PMC Article
Woodfield G, Nisbet M, Jacob J, Mok W, Loebinger MR, Hansell DM, Wells AU, Wilson R
Respirology 2017 Jan;22(1):101-107. Epub 2016 Aug 23 doi: 10.1111/resp.12866. PMID: 27551950
Boyton RJ
Med Mycol 2009;47 Suppl 1:S175-82. Epub 2008 Jun 4 doi: 10.1080/13693780802163370. PMID: 18608925
Fuschillo S, De Felice A, Balzano G
Eur Respir J 2008 Feb;31(2):396-406. doi: 10.1183/09031936.00069007. PMID: 18238949

Therapy

Mulette P, Perotin JM, Muggeo A, Guillard T, Brisebarre A, Meyer H, Hagenburg J, Ancel J, Dormoy V, Vuiblet V, Launois C, Lebargy F, Deslee G, Dury S
Eur J Med Res 2024 Feb 13;29(1):120. doi: 10.1186/s40001-024-01701-1. PMID: 38350996Free PMC Article
Bates AS, Natarajan M, Reddy RV
BMJ Case Rep 2018 Nov 28;11(1) doi: 10.1136/bcr-2015-211610. PMID: 30567079Free PMC Article
Woodfield G, Nisbet M, Jacob J, Mok W, Loebinger MR, Hansell DM, Wells AU, Wilson R
Respirology 2017 Jan;22(1):101-107. Epub 2016 Aug 23 doi: 10.1111/resp.12866. PMID: 27551950
Sehgal IS, Dhooria S, Bal A, Agarwal R
BMJ Case Rep 2015 Aug 6;2015 doi: 10.1136/bcr-2015-211493. PMID: 26250371Free PMC Article
Guan WJ, Gao YH, Xu G, Lin ZY, Tang Y, Li HM, Lin ZM, Zheng JP, Chen RC, Zhong NS
Respirology 2015 Jul;20(5):739-48. Epub 2015 Mar 26 doi: 10.1111/resp.12528. PMID: 25819403

Prognosis

Perluk T, Abu Bandora E, Freund O, Jacob T, Friedman Regev I, Kleinhendler E, Shteinberg M, Bar-Shai A, Oestriecher-Kedem Y
Lung 2024 Apr;202(2):189-195. Epub 2024 Mar 18 doi: 10.1007/s00408-024-00683-5. PMID: 38499811Free PMC Article
Mulette P, Perotin JM, Muggeo A, Guillard T, Brisebarre A, Meyer H, Hagenburg J, Ancel J, Dormoy V, Vuiblet V, Launois C, Lebargy F, Deslee G, Dury S
Eur J Med Res 2024 Feb 13;29(1):120. doi: 10.1186/s40001-024-01701-1. PMID: 38350996Free PMC Article
Abid N, Loukil M, Mokni A, Badri I, Bouzaidi K, Ghrairi H
Tunis Med 2021 Feb;99(2):264-268. PMID: 33899197Free PMC Article
Nikolic A
Lung 2018 Aug;196(4):383-392. Epub 2018 May 12 doi: 10.1007/s00408-018-0121-y. PMID: 29754320
Guan WJ, Jiang M, Gao YH, Li HM, Xu G, Zheng JP, Chen RC, Zhong NS
Int J Tuberc Lung Dis 2016 Mar;20(3):402-10. doi: 10.5588/ijtld.15.0500. PMID: 27046724

Clinical prediction guides

Perluk T, Abu Bandora E, Freund O, Jacob T, Friedman Regev I, Kleinhendler E, Shteinberg M, Bar-Shai A, Oestriecher-Kedem Y
Lung 2024 Apr;202(2):189-195. Epub 2024 Mar 18 doi: 10.1007/s00408-024-00683-5. PMID: 38499811Free PMC Article
Yu I, Yong SJ, Lee WY, Kim SH, Lee H, Na JO, Kim DK, Oh YM, Lee JH; KMBARC
Korean J Intern Med 2022 Sep;37(5):1002-1010. Epub 2022 Aug 18 doi: 10.3904/kjim.2022.070. PMID: 35977811Free PMC Article
Nucci MCNM, Fernandes FLA, Salge JM, Stelmach R, Cukier A, Athanazio R
J Bras Pneumol 2020;46(5):e20190162. Epub 2020 Jun 15 doi: 10.36416/1806-3756/e20190162. PMID: 32556031Free PMC Article
Woodfield G, Nisbet M, Jacob J, Mok W, Loebinger MR, Hansell DM, Wells AU, Wilson R
Respirology 2017 Jan;22(1):101-107. Epub 2016 Aug 23 doi: 10.1111/resp.12866. PMID: 27551950
Fuschillo S, De Felice A, Balzano G
Eur Respir J 2008 Feb;31(2):396-406. doi: 10.1183/09031936.00069007. PMID: 18238949

Recent systematic reviews

Sehgal IS, Dhooria S, Bal A, Agarwal R
BMJ Case Rep 2015 Aug 6;2015 doi: 10.1136/bcr-2015-211493. PMID: 26250371Free PMC Article

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