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Pulmonary Fibrosis (CUI C0034069) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0034069

NCI Thesaurus Code: C26869  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

NCIt Definition: Chronic progressive interstitial lung disorder characterized by the replacement of the lung tissue by connective tissue, leading to progressive dyspnea, respiratory failure, or right heart failure. Causes include chronic inflammatory processes, exposure to environmental irritants, radiation therapy, autoimmune disorders, certain drugs, or it may be idiopathic (no identifiable cause).

HPO Definition: Replacement of normal lung tissues are progressively replaced by fibroblasts and collagen. [DDD:tkuijpers, HPO:probinson]

MEDLINEPLUS Definition: 

Pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. This tissue gets thick and stiff. That makes it hard for you to catch your breath, and your blood may not get enough oxygen.

Causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue diseases, and interstitial lung disease. Interstitial lung disease is the name for a large group of diseases that inflame or scar the lungs. In most cases, the cause cannot be found. This is called idiopathic pulmonary fibrosis.

Symptoms include

  • Shortness of breath
  • A dry, hacking cough that doesn't get better
  • Fatigue
  • Weight loss for no known reason
  • Aching muscles and joints
  • Clubbing, which is the widening and rounding of the tips of the fingers or toes

Your doctor may use your medical history, imaging tests, a biopsy, and lung function tests to diagnose pulmonary fibrosis. There is no cure. Treatments can help with symptoms and improve your quality of life. They include medicines, oxygen therapy, pulmonary rehabilitation, or a lung transplant.

NIH: National Heart, Lung, and Blood Institute

CTCAE Definition: A disorder characterized by the replacement of the lung tissue by connective tissue, leading to progressive dyspnea, respiratory failure or right heart failure.

NICHD Definition: A interstitial lung disease characterized by the replacement of lung tissue with connective tissue.

MSH Definition: A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.

Synonyms & Abbreviations: (see Synonym Details)
Cirrhosis of lung
FIBROSES PULM
Fibroses, Pulmonary
FIBROSIS LUNG
Fibrosis of lung (disorder)
Fibrosis of lung
FIBROSIS PULMONARY
FIBROSIS PULM
Fibrosis, Pulmonary
IPF
LUNG FIBROSIS
PULM FIBROSES
PULM FIBROSIS
Pulmonary Fibroses
Pulmonary Fibrosis [Disease/Finding]
pulmonary fibrosis
Pulmonary Interstitial Fibrosis

External Source Codes: 
NCI Thesaurus Code C26869 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AN usually chronic so do not coordinate with CHRONIC DISEASE; IDIOPATHIC PULMONARY FIBROSIS is also available; DF: PULM 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 900000000000448009 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
Contributing_Source MedDRA NCI
Contributing_Source NICHD NCI
CTV3ID X102u SNOMEDCT_US
DATE_CREATED 12/13/1999 MEDLINEPLUS
DC 1 MSH
DEFINITION_STATUS_ID 900000000000073002 SNOMEDCT_US
DID 2596-8265 CSP
DX 19660101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
HPO_COMMENT Pulmonary fibrosis leads to an irreversible impairment of alveolar oxygen transfer. HPO
MDA 19990101 MSH
MESH_DEFINITION A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death. NDFRT
MESH_DUI D011658 NDFRT
MESH_NAME Pulmonary Fibrosis NDFRT
MESH_UI M0018134 NDFRT
MMR 20090706 MSH
MN C08.381.765 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
MP_HEALTH_TOPIC_URL https://www.nlm.nih.gov/medlineplus/pulmonaryfibrosis.html MEDLINEPLUS
MP_OTHER_LANGUAGE_URL Spanish https://www.nlm.nih.gov/medlineplus/spanish/pulmonaryfibrosis.html MEDLINEPLUS
MP_PRIMARY_INSTITUTE_URL National Heart, Lung, and Blood Institute http://www.nhlbi.nih.gov/ MEDLINEPLUS
NDFRT_KIND DISEASE_KIND NDFRT
NICHD_Hierarchy_Term Pulmonary Fibrosis NCI
NUI N0000002544 NDFRT
PRIMARY_PATH 10037383$10033979$10024967$10038738$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 20.0 MDR
PT_IN_VERSION 20.1 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
RXAUI 3160625 RXNORM
RXAUI 3160639 RXNORM
RXAUI 3226042 RXNORM
RXAUI 3226043 RXNORM
RXAUI 3226044 RXNORM
RXCUI 1022670 RXNORM
SID HP:0006523 HPO
SMQ_TERM_ADDVERSION 9.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 9.0 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMED_CID 51615001 NDFRT
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 J84.10 | DESCENDANTS NOT EXHAUSTIVELY MAPPED 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~J84.10 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~X102u 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 T034437 MSH
TERMUI T034438 MSH
TERMUI T034439 MSH
TERMUI T034440 MSH
TH NLM (1966) MSH
TH NLM (1992) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

URL to Bookmark: https://ncim-stage.nci.nih.gov/ncimbrowser/ConceptReport.jsp?dictionary=NCI Metathesaurus&code=C0034069

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