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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. See: This record Not genetically inherited (Orphanet)

Related genes: Gene(s) associated with related conditions. For conditions in a hierarchy, the parent condition will list the genes associated with the children conditions.	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]

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

Abnormality of the respiratory system
- Abnormal respiratory system physiology
  - Abnormal respiratory system morphology
    - Abnormal tracheobronchial morphology
      - Abnormal bronchus morphology
        Bronchiectasis
        Idiopathic bronchiectasis
        Bronchiectasis with or without elevated sweat chloride 1
        Bronchiectasis with or without elevated sweat chloride 2
        Bronchiectasis with or without elevated sweat chloride 3

Follow this link to review classifications for Idiopathic bronchiectasis in Orphanet.

Professional guidelines

PubMed

Outcomes of bronchial artery embolization for the management of hemoptysis.

Abid N, Loukil M, Mokni A, Badri I, Bouzaidi K, Ghrairi H
Tunis Med 2021 Feb;99(2):264-268. PMID: 33899197 Free PMC Article

Primary immunodeficiency-related bronchiectasis in adults: comparison with bronchiectasis of other etiologies in a French reference center.

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: 31801528 Free PMC Article

Chronic cough due to bronchiectasis: ACCP evidence-based clinical practice guidelines.

Rosen MJ
Chest 2006 Jan;129(1 Suppl):122S-131S. doi: 10.1378/chest.129.1_suppl.122S. PMID: 16428701

See all (5)

Recent clinical studies

Etiology

Asymptomatic Dysphagia and Aspiration in Patients with Idiopathic Bronchiectasis.

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: 38499811 Free PMC Article

Pathophysiology and Genetics of Bronchiectasis Unrelated to Cystic Fibrosis.

Nikolic A
Lung 2018 Aug;196(4):383-392. Epub 2018 May 12 doi: 10.1007/s00408-018-0121-y. PMID: 29754320

Etiology of Bronchiectasis in a Cohort of 2047 Patients. An Analysis of the Spanish Historical Bronchiectasis Registry.

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

Etiology of Non-Cystic Fibrosis Bronchiectasis in Adults and Its Correlation to Disease Severity.

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: 26431397 Free PMC Article

Lack of association between KIR and HLA-C type and susceptibility to idiopathic bronchiectasis.

McDonnell MJ, Anwar GA, Rutherford RM, De Soyza A, Worthy S, Corris PA, Lordan JL, Bourke S, Afolabi G, Ward C, Middleton P, Middleton D
Respir Med 2014 Aug;108(8):1127-33. Epub 2014 Jun 18 doi: 10.1016/j.rmed.2014.05.017. PMID: 24986480

See all (52)

Diagnosis

Asymptomatic Dysphagia and Aspiration in Patients with Idiopathic Bronchiectasis.

Characterization of SLC26A9 in patients with CF-like lung disease.

Bakouh N, Bienvenu T, Thomas A, Ehrenfeld J, Liote H, Roussel D, Duquesnoy P, Farman N, Viel M, Cherif-Zahar B, Amselem S, Taam RA, Edelman A, Planelles G, Sermet-Gaudelus I
Hum Mutat 2013 Oct;34(10):1404-14. Epub 2013 Aug 13 doi: 10.1002/humu.22382. PMID: 24272871

A fatal case of cough.

Bossley CJ, Hogg C, Stoneham S, Jacques TS, Bush A
Pediatr Pulmonol 2010 Feb;45(2):205-7. doi: 10.1002/ppul.21152. PMID: 20082340

Regulation of immunity in bronchiectasis.

Boyton RJ
Med Mycol 2009;47 Suppl 1:S175-82. Epub 2008 Jun 4 doi: 10.1080/13693780802163370. PMID: 18608925

Mucosal inflammation in idiopathic bronchiectasis: cellular and molecular mechanisms.

Fuschillo S, De Felice A, Balzano G
Eur Respir J 2008 Feb;31(2):396-406. doi: 10.1183/09031936.00069007. PMID: 18238949

See all (43)

Therapy

Bronchiectasis in renal transplant patients: a cross-sectional study.

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: 38350996 Free PMC Article

Bronchiectasis in yellow nail syndrome.

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

Aetiology of bronchiectasis in Guangzhou, southern China.

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

First report of lung transplantation in a patient with active pulmonary Mycobacterium simiae infection.

Qvist T, Katzenstein TL, Lillebaek T, Iversen M, Mared L, Andersen AB
Transplant Proc 2013 Mar;45(2):803-5. Epub 2012 Sep 6 doi: 10.1016/j.transproceed.2012.02.034. PMID: 23267789

So called idiopathic bronchiectasis. (Special review about therapy and results).

Swierenga J
Bronches 1973 Mar-Apr;23(2):84-92. PMID: 4784219

See all (26)

Prognosis

Asymptomatic Dysphagia and Aspiration in Patients with Idiopathic Bronchiectasis.

Outcomes of bronchial artery embolization for the management of hemoptysis.

Abid N, Loukil M, Mokni A, Badri I, Bouzaidi K, Ghrairi H
Tunis Med 2021 Feb;99(2):264-268. PMID: 33899197 Free PMC Article

Bronchial artery embolization: A gratifying life-saving procedure.

Mahla H, Kunal S, Sharma SK, Mahla R, Jain A, Sharma SM
Indian J Tuberc 2021 Jan;68(1):40-50. Epub 2020 Sep 28 doi: 10.1016/j.ijtb.2020.09.019. PMID: 33641850

Pathophysiology and Genetics of Bronchiectasis Unrelated to Cystic Fibrosis.

Nikolic A
Lung 2018 Aug;196(4):383-392. Epub 2018 May 12 doi: 10.1007/s00408-018-0121-y. PMID: 29754320

A fatal case of cough.

Bossley CJ, Hogg C, Stoneham S, Jacques TS, Bush A
Pediatr Pulmonol 2010 Feb;45(2):205-7. doi: 10.1002/ppul.21152. PMID: 20082340

See all (24)

Clinical prediction guides

Asymptomatic Dysphagia and Aspiration in Patients with Idiopathic Bronchiectasis.

Bronchial artery embolization: A gratifying life-saving procedure.

Mahla H, Kunal S, Sharma SK, Mahla R, Jain A, Sharma SM
Indian J Tuberc 2021 Jan;68(1):40-50. Epub 2020 Sep 28 doi: 10.1016/j.ijtb.2020.09.019. PMID: 33641850

Bronchiectasis in yellow nail syndrome.

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

Lack of association between KIR and HLA-C type and susceptibility to idiopathic bronchiectasis.

Mucosal inflammation in idiopathic bronchiectasis: cellular and molecular mechanisms.

Fuschillo S, De Felice A, Balzano G
Eur Respir J 2008 Feb;31(2):396-406. doi: 10.1183/09031936.00069007. PMID: 18238949

See all (28)