Details of Disease

General Information of Disease (ID: DISCK9IV)

• Disease Name: Bronchiolitis obliterans syndrome
• Synonyms: bronchiolitis obliterans; organizing pneumonia; obliterative bronchiolitis; bronchiolitis exudativa; organising pneumonia; BO; bronchiolitis obliterans with obstructive pulmonary disease; BOOP; constrictive bronchiolitis; organized pneumonia; bronchiolitis fibrosa obliterans; organised pneumonia
• Definition: A lung disorder that is mainly associated with chronic allograft dysfunction after lung transplantation and that is characterized by inflammation and fibrosis of bronchiolar walls that reduce the diameter of the bronchioles and result in progressive and irreversible airflow obstruction.|This is restricted to lung transplant patients.
• Disease Hierarchy:
  DISWSHC6: Interstitial lung disease
  DISEE9BG: Bronchiolitis
  DISCK9IV: Bronchiolitis obliterans syndrome
• Disease Identifiers:
  MONDO ID: MONDO_0015265
  MESH ID: D001989
  UMLS CUI: C0006272
  MedGen ID: 665
  HPO ID: HP:0011946
  Orphanet ID: 1303
  SNOMED CT ID: 40100001

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Drugs in Phase 2 Trial

Drug Name	Drug ID	Highest Status	Drug Type	REF
Cyclosporin A	DMUYKBZ	Phase 2 Trial	Small molecular drug	[1]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 5 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CCL5	TT9DWLC	Strong	Biomarker	[2]
CD86	TT53XHB	Strong	Biomarker	[3]
CDCA8	TT04YCM	Strong	Genetic Variation	[4]
IL4	TTLGTKB	Strong	Biomarker	[5]
TNFRSF8	TT2GM5R	Strong	Altered Expression	[6]

This Disease Is Related to 10 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
IL26	OT2WYCW4	moderate	Biomarker	[7]
ANLN	OTXJY54C	Strong	Genetic Variation	[8]
CCL7	OTDIS99H	Strong	Biomarker	[9]
EYA1	OTHU807A	Strong	Genetic Variation	[10]
GOSR2	OTYHIYN2	Strong	Genetic Variation	[11]
KIR2DS5	OTXLEN11	Strong	Biomarker	[12]
LCN1	OT8BWXTV	Strong	Genetic Variation	[13]
NUDT6	OTCS3NYZ	Strong	Altered Expression	[14]
SIX1	OT70YYWM	Strong	Biomarker	[10]
SIX5	OTT1I9WY	Strong	Biomarker	[10]

References

• [1] Bronchiolitis obliterans syndrome is associated with increased senescent lymphocytes in the small airways. J Heart Lung Transplant. 2021 Feb;40(2):108-119.
• [2] A model of chronic lung allograft rejection in the rat.Eur Respir J. 2010 Jun;35(6):1354-63. doi: 10.1183/09031936.00110609. Epub 2009 Oct 19.
• [3] CD28-B7-2 but not CD28-B7-1 interaction is required for T cell costimulation in experimental obliterative bronchiolitis in the rat.Transplant Proc. 2001 Feb-Mar;33(1-2):535. doi: 10.1016/s0041-1345(00)02128-x.
• [4] Autosomal-dominant branchio-otic (BO) syndrome is not allelic to the branchio-oto-renal (BOR) gene at 8q13.Am J Med Genet. 1998 Apr 13;76(5):395-401.
• [5] Chronic aspiration of gastric fluid induces the development of obliterative bronchiolitis in rat lung transplants.Am J Transplant. 2008 Aug;8(8):1614-21. doi: 10.1111/j.1600-6143.2008.02298.x. Epub 2008 Jun 28.
• [6] Soluble CD30 levels as a diagnostic marker for bronchiolitis obliterans syndrome following human lung transplantation.Transpl Immunol. 2008 Jan;18(3):260-3. doi: 10.1016/j.trim.2007.07.007. Epub 2007 Sep 11.
• [7] Regulation of pulmonary graft-versus-host disease by IL-26+CD26+CD4 T lymphocytes.J Immunol. 2015 Apr 15;194(8):3697-712. doi: 10.4049/jimmunol.1402785. Epub 2015 Mar 18.
• [8] Identification of ANLN as a new likely pathogenic gene of branchio-otic syndrome in a three-generation Chinese family.Mol Genet Genomic Med. 2019 Feb;7(2):e00525. doi: 10.1002/mgg3.525. Epub 2018 Dec 11.
• [9] Bronchial and bronchiolar fibrosis in rats exposed to 2,3-pentanedione vapors: implications for bronchiolitis obliterans in humans.Toxicol Pathol. 2012 Apr;40(3):448-65. doi: 10.1177/0192623311431946. Epub 2012 Jan 3.
• [10] Whole exome sequencing identifies a mutation in EYA1 and GLI3 in a patient with branchiootic syndrome and esophageal atresia: Coincidence or a digenic mode of inheritance?.Mol Med Rep. 2018 Feb;17(2):3200-3205. doi: 10.3892/mmr.2017.8196. Epub 2017 Dec 6.
• [11] (1)H NMR To Evaluate the Metabolome of Bronchoalveolar Lavage Fluid (BALf) in Bronchiolitis Obliterans Syndrome (BOS): Toward the Development of a New Approach for Biomarker Identification.J Proteome Res. 2017 Apr 7;16(4):1669-1682. doi: 10.1021/acs.jproteome.6b01038. Epub 2017 Mar 14.
• [12] The killer immunoglobulin-like receptor (KIR) group A haplotype is associated with bronchiolitis obliterans syndrome after lung transplantation.J Heart Lung Transplant. 2008 Sep;27(9):995-1001. doi: 10.1016/j.healun.2008.06.006.
• [13] Airway inflammation in children and adolescents with bronchiolitis obliterans.Cytokine. 2015 May;73(1):156-62. doi: 10.1016/j.cyto.2014.10.026. Epub 2015 Mar 6.
• [14] Obliterative airway remodelling in transplanted and non-transplanted lungs.Virchows Arch. 2010 Sep;457(3):369-80. doi: 10.1007/s00428-010-0949-x. Epub 2010 Jul 15.