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Idiopathic Pulmonary Fibrosis (CUI C1800706) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C1800706

NCI Thesaurus Code: C35716  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

NCIt Definition: Chronic and progressive fibrosis of the lung parenchyma of unknown cause.

RADLEX Definition: Pathology. - Idiopathic pulmonary fibrosis is a specific form of chronic fibrosing interstitial pneumonia of unknown cause and is characterized by a histologic pattern of usual interstitial pneumonia (5,64). Radiographs and CT scans. - The typical imaging findings are reticular opacities and honeycombing, with a predominantly peripheral and basal distribution (Fig 26). Ground-glass opacity, if present, is less extensive than reticular and honeycombing patterns. The typical radiologic findings (65,66) are also encountered in usual interstitial pneumonia secondary to specific causes, such as asbestos-induced pulmonary fibrosis (asbestosis), and the diagnosis is usually one of exclusion. (See also usual interstitial pneumonia.) [Fleischner Society]

GARD Definition: Idiopathic pulmonary fibrosis (IPF) is a condition in which tissues in the lungs become thick and stiff, or scarred, over time. The lungs then lose their ability to move oxygen to the brain and other parts of the body. Common symptoms include shortness of breath and a dry, hacking cough. In some cases fibrosis happens quickly, while in others, the process is much slower. Sometimes the disease stays the same for years. The condition is 'idiopathic' because the cause is unknown. When multiple family members are affected, it is called familial IPF. Many people with this condition live for about 3-5 years after the diagnosis. The most common cause of death is respiratory failure. - this information is from GARD/ORDR/NCATS.

NCI-GLOSS Definition: A disease in which the alveoli (tiny air sacs at the end of the bronchioles in the lungs) are overgrown with fibrous tissue. The cause of the disease is unknown and it gets worse over time. Symptoms include difficult, painful breathing and shortness of breath.

CSP Definition: chronic inflammation and progressive fibrosis of the pulmonary alveolar walls with steady progressive dyspnea; finally, resulting in death from lack of oxygen or right heart failure.

MSH Definition: A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.

Synonyms & Abbreviations: (see Synonym Details)
Alveolitides, Fibrosing
ALVEOLITIS FIBROSING
ALVEOLITIS, CHRONIC DIFFUSE FIBROSING
ALVEOLITIS, CHRONIC DIFFUSE SCLEROSING
ALVEOLITIS, FIBROSING
CFA - Cryptogenic fibrosing alveolitis
CFA
Cryptogenic Fibrosing Alveolitides
Cryptogenic fibrosing alveolitis
diffuse interstitial pulmonary fibrosis
diopathic pulmonary fibrosis
Dysplasia, Fibrocystic Pulmonary
Dysplasias, Fibrocystic Pulmonary
Familial idiopathic pulmonary fibrosis (disorder)
Familial idiopathic pulmonary fibrosis
Fibrocystic Pulmonary Dysplasias
Fibrocystic pulmonary dysplasia
Fibrosing Alveolitides, Cryptogenic
Fibrosing Alveolitides
Fibrosing alveolitis (idiopathic)
Fibrosing Alveolitis, Cryptogenic
Fibrosing alveolitis
FIBROSIS, IDIOPATHIC PULMONARY
FIBROSIS, PULMONARY, INTERSTITIAL DIFFUSE
Hamman-Rich Disease
Idiopath pulmon fibrosis
Idiopathic Fibrosing Alveolitis, Chronic Form
IDIOPATHIC FIBROSING ALVEOLITIS
Idiopathic Pulmonary Fibroses
Idiopathic pulmonary fibrosis (disorder)
Idiopathic Pulmonary Fibrosis [Disease/Finding]
Idiopathic Pulmonary Fibrosis, Familial
idiopathic pulmonary fibrosis
Interstitial Pneumonitis, Usual
IPF
Pulmonary Dysplasia, Fibrocystic
Pulmonary Dysplasias, Fibrocystic
Pulmonary Fibroses, Idiopathic
Pulmonary Fibrosis, Idiopathic
Usual Interstitial Pneumonia

External Source Codes: 
NCI Thesaurus Code C35716 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AN do not confuse with PULMONARY FIBROSIS MSH
AQL BL CF CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000020002 SNOMEDCT_US
CITE Fleischner Society RADLEX
CTV3ID XUfia SNOMEDCT_US
CTV3ID XUmtl SNOMEDCT_US
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_CREATED 2015-01-22T00:04:00 GARD
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 4008-0052 CSP
DISEASE_IDENTIFIER_ID 19625 GARD
DISEASE_IDENTIFIER_ID 19626 GARD
DISEASE_IDENTIFIER_ID 31232 GARD
DISEASE_IDENTIFIER_ID 31233 GARD
DX 20090101 MSH
EFFECTIVE_TIME 20070731 SNOMEDCT_US
EFFECTIVE_TIME 20140731 SNOMEDCT_US
GENELOCUS ,1,1,p,1,5, OMIM
GENESYMBOL MUC5B OMIM
HAS_GARD_PAGE true GARD
HN 2009 MSH
ICE Cryptogenic fibrosing alveolitis ICD9CM
IDENTIFIER_SOURCE Orp GARD
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
MDA 20080708 MSH
MESH_DEFINITION A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change. NDFRT
MESH_DUI D054990 NDFRT
MESH_NAME Idiopathic Pulmonary Fibrosis NDFRT
MESH_UI M0511887 NDFRT
MIMTYPE 3 OMIM
MIMTYPEMEANING Phenotype description, molecular basis known. OMIM
MIMTYPEVALUE pound OMIM
MMR 20130708 MSH
MN C08.381.483.487.500 MSH
MN C08.381.765.500 MSH
MOVED_FROM 135000 OMIM
NDFRT_KIND DISEASE_KIND NDFRT
NUI N0000181246 NDFRT
ORDER_NO 12679 ICD10CM
PM 2009 MSH
PRIMARY_PATH 10021240$10033979$10024967$10038738$Idiopathic pulmonary fibrosis$Parenchymal lung disorders NEC$Lower respiratory tract disorders (excl obstruction and infection)$Respiratory, thoracic and mediastinal disorders MDR
PRIMARY_SOC 10038738 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/8609/idiopathic-pulmonary-fibrosis/resources/1 GARD
RXAUI 3287024 RXNORM
RXAUI 3287600 RXNORM
RXAUI 3287601 RXNORM
RXAUI 5039364 RXNORM
RXAUI 5039504 RXNORM
RXAUI 5039870 RXNORM
RXAUI 5930913 RXNORM
RXAUI 5930953 RXNORM
RXAUI 5931240 RXNORM
RXCUI 1023381 RXNORM
SMQ_TERM_ADDVERSION 13.0 MDR
SMQ_TERM_ADDVERSION 9.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 13.0 MDR
SMQ_TERM_LMVERSION 16.0 MDR
SMQ_TERM_LMVERSION 9.0 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMEDID DF-007E1 SNOMEDCT_US
SNOMEDID R-FB6EE SNOMEDCT_US
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS J84.1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~J84.1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS J84.112 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~J84.112 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XUfia SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~XUmtl SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~DF-007E1 SNOMEDCT_US
SUBSET_MEMBER 900000000000498005~MAPTARGET~R-FB6EE SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T034441 MSH
TERMUI T703056 MSH
TERMUI T703057 MSH
TERMUI T703058 MSH
TERMUI T812632 MSH
TERMUI T812633 MSH
TERMUI T812634 MSH
TERMUI T812635 MSH
TERMUI T833798 MSH
TERMUI T833799 MSH
TERMUI T833800 MSH
TERMUI T843517 MSH
TH GHR (2014) MSH
TH NLM (2009) MSH
TH OMIM (2013) MSH
TH ORD (2010) MSH
TH UNK (19XX) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

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