KEGG   DISEASE: Obliterative bronchiolitis
Entry
H01873                      Disease                                
Name
Obliterative bronchiolitis;
Bronchiolitis obliterans
Description
Obliterative bronchiolitis (OB), also known as bronchiolitis obliterans, is a rare but serious condition resulting in progressive and irreversible airway obstruction. This disease is the result of injury to the respiratory and terminal bronchioles from a wide variety of potential causes. Although the pathogenesis of OB remains poorly understood, it seems clear that a multifaceted process of inflammation and resultant fibrotic transition occurs, resulting in airflow limitation and development of disease. Although OB has been described in all age groups, the frequency of underlying causes and potential prognoses are different for children and adults. For example, OB in children is most often seen following a severe lower-respiratory-tract infection, usually of adenovirus, whereas OB in adults is more commonly associated with occupational inhalation injuries, hypersensitivity pneumonitis, and autoimmune disorders. In lung transplant recipients, bronchiolitis obliterans syndrome is the major cause of death after the first year of transplantation and is a form of allograft rejection. There is no uniformly accepted treatment protocol for patients with OB. Many patients are tried on corticosteroids or other immunosuppressants based on anecdotal evidence of improvement. However, these medications have significant toxicity.
Category
Respiratory system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 12 Diseases of the respiratory system
  Certain lower respiratory tract diseases
   CA22  Chronic obstructive pulmonary disease
    H01873  Obliterative bronchiolitis
   CA26  Chronic bronchiolitis
    H01873  Obliterative bronchiolitis
Drug
Prednisolone sodium phosphate [DR:D00981]
Comment
In the present manuscript, the following terminology is used: "bronchiolitis obliterans" (historically used for the histopathological lesions) to name the pathological feature of reduced lumen of the bronchioles, and "obliterative bronchiolitis" (OB) to name its functional counterpart with airflow obstruction.
Other DBs
ICD-11: CA26.0 CA22.1
MeSH: D001989
Reference
  Authors
Aguilar PR, Michelson AP, Isakow W
  Title
Obliterative Bronchiolitis.
  Journal
Transplantation 100:272-83 (2016)
DOI:10.1097/TP.0000000000000892
Reference
  Authors
Devouassoux G, Cottin V, Liote H, Marchand E, Frachon I, Schuller A, Bejui-Thivolet F, Cordier JF
  Title
Characterisation of severe obliterative bronchiolitis in rheumatoid arthritis.
  Journal
Eur Respir J 33:1053-61 (2009)
DOI:10.1183/09031936.00091608
Reference
  Authors
Moonnumakal SP, Fan LL
  Title
Bronchiolitis obliterans in children.
  Journal
Curr Opin Pediatr 20:272-8 (2008)
DOI:10.1097/MOP.0b013e3282ff62e9
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