DiseaseID 1286
肺纤维化
disease
NCI2016_02D:A rare, rapidly progressing interstitial lung disorder characterized by diffuse and bilateral alveolar damage. Patients present with sudden onset of dyspnea and rapid development of respiratory failure. Manag
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Disease: 1Experiment: 12Symptom: 12Target: 12Links: 36
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Record Fields
Scalar fields from the final disease record.
- Disease Id
- 1286
- Core Entity Id
- 1452
- Source Entity Count
- 1
- Preferred Name
- Pulmonary Fibrosis
- Name Cn
- 肺纤维化
- Name Pinyin
- Fei Xian Wei Hua
- Name En
- Pulmonary Fibrosis
- Name Latin
- Bilingual Status
- complete
- Disease Type
- disease
- Umls Disease Type
- Disease or Syndrome
- Disgenet Type
- disease
- Mesh Class
- Infections; Respiratory Tract DiseasesInfections; Respiratory Tract Diseases; Pathological Conditions, Signs and SymptomsRespiratory Tract Diseases
- Do Class
- disease of anatomical entity
- Hpo Class
- Abnormality of the respiratory system
- Mesh Class Name
- Infections; Respiratory Tract DiseasesPathological Conditions, Signs and Symptoms; Infections; Respiratory Tract DiseasesRespiratory Tract Diseases
- Hpo Class Name
- Abnormality of the respiratory system
- Do Class Name
- disease of anatomical entity
- Disease Definition
- NCI2016_02D:A rare, rapidly progressing interstitial lung disorder characterized by diffuse and bilateral alveolar damage. Patients present with sudden onset of dyspnea and rapid development of respiratory failure. Management is supportive and hospitalization and mechanical ventilation are usually required.
- Version
- v1,v2
- Suppressed
- No
Names
Preferred names, aliases, and source labels retained in the final schema.
Name
Pulmonary Fibrosis
Role
preferred
Name
Acute Interstitial Pneumonia
Role
preferred
Name
Chronic Idiopathic Pulmonary Fibrosis
Role
preferred
Name
Hamman-Rich Syndrome
Role
preferred
Name
Idiopathic Interstitial Pneumonias
Role
preferred
Name
Pulmonary Fibrosis, Idiopathic
Role
preferred
Name
Alveolitis, Fibrosing
Role
preferred
Name
Atypical Pneumonia
Role
preferred
Name
Familial Idiopathic Pulmonary Fibrosis
Role
preferred
Name
Hamman-Rich Disease
Role
preferred
Name
Idiopathic Pulmonary Fibrosis
Role
preferred
Name
Primary Atypical Pneumonia
Role
preferred
Name
Usual Interstitial Pneumonia
Role
preferred
Name
FIBROCYSTIC PULMONARY DYSPLASIA
Role
alias
Name
FIBROSING ALVEOLITIS, CRYPTOGENIC
Role
alias
Name
IDIOPATHIC PULMONARY FIBROSIS, FAMILIAL
Role
alias
Name
INTERSTITIAL PNEUMONITIS, USUAL
Role
alias
Name
IPF
Role
alias
Name
Idiopathic Interstitial Pneumonia
Role
alias
Name
UIP
Role
alias
Cross References
Trusted external identifiers retained for this final record.
Hpo
HP:0002206HP:0031950
Herb
HBDIS002540HBDIS003373HBDIS010708HBDIS013163HBDIS014208HBDIS014980HBDIS019115HBDIS022455HBDIS027769HBDIS027770HBDIS027771HBDIS027813
Me Sh
D011658D054988D054990
Omim
178500
Umls
C0034069C0085786C1279945C1800706C2350236C3812396
Icd10
J84.11J84.112J84.114
Sym Map
SMDE00855SMDE05437SMDE07189SMDE09209SMDE09764SMDE12591
Do Class
DOID:7
Dis Ge Net
C0034069C0085786C0702135C1279945C1412002C1800706C2350236C3812396C4721507C4721508C4721509C4721952
Umls Sty
T047
Hpo Class
HP:0002086
Me Sh Class
C01C08C23
Tcmbank Disease
125891688921342378724336248762744628327371741864443582188492
Itcmdb Generated
ITX-DISEASE-31BA02162121ITX-DISEASE-5786983B72B5ITX-DISEASE-612F24EC2F4EITX-DISEASE-6151AF840225ITX-DISEASE-73C9EA12B30AITX-DISEASE-E5FD3F2A79B9
Attributes
Merged source attributes and domain-specific metadata.
Version
v1,v2v2
Suppress
0
Do Class Name
disease of anatomical entity
Disease Type
disease
Hpo Class Name
Abnormality of the respiratory system
Do Disease Class
disease of anatomical entity
Hpo Disease Class
Abnormality of the respiratory system
Umls Disease Type
Disease or Syndrome
Disease Definition
NCI2016_02D:A rare, rapidly progressing interstitial lung disorder characterized by diffuse and bilateral alveolar damage. Patients present with sudden onset of dyspnea and rapid development of respiratory failure. Management is supportive and hospitalization and mechanical ventilation are usually required.NCI2016_02D:Non-infectious inflammation of the interstitial lung tissue. This category includes the desquamative interstitial pneumonia, usual interstitial pneumonia, lymphocytic interstitial pneumonia, acute interstitial pneumonia, and nonspecific interstitial pneumonia.|MSH2017_2016_08_12:A group of interstitial lung diseases with no known etiology. There are several entities with varying patterns of inflammation and fibrosis. They are classified by their distinct clinical-radiological-pathological features and prognosis. They include IDIOPATHIC PULMONARY FIBROSIS; CRYPTOGENIC ORGANIZING PNEUMONIA; and others.NCI2016_NCI-GLOSS_1602D: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.|NCI2016_02D:Interstitial pneumonia characterized by the presence of fibrosis in the interstitial lung tissue. The pathologic diagnosis is based on the identification of fibrotic lesions at different stages of development within a lung biopsy specimen. Typically there are foci of normal lung parenchyma alternating with interstitial inflammation and honeycombing. The term usual interstitial pneumonia sometimes is used interchangeably with idiopathic interstitial fibrosis. The two terms are not entirely synonymous. Usual interstitial pneumonia may be associated with other conditions such as connective tissue disorders and asbestosis. The diagnosis of idiopathic interstitial fibrosis requires the exclusion of such conditions. Patients with usual interstitial pneumonia present with progressive dyspnea, cough, and restrictive pulmonary function abnormalities. The prognosis is usually poor.|NCI2016_02D:Chronic and progressive fibrosis of the lung parenchyma of unknown cause.|MSH2017_2016_08_12: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.|CSP2006: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.NCI2016_NICHD_1602D:A interstitial lung disease characterized by the replacement of lung tissue with connective tissue.|NCI2016_CTCAE_1602D:A disorder characterized by the replacement of the lung tissue by connective tissue, leading to progressive dyspnea, respiratory failure or right heart failure.|NCI2016_02D: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).|MSH2017_2016_08_12: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.|MEDLINEPLUS_20151021:<p>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.</p> <p>Causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue diseases, and interstitial lung disease. <a href='https://www.nlm.nih.gov/medlineplus/interstitiallungdiseases.html'>Interstitial lung disease</a> 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.</p> <p>Symptoms include</p> <ul> <li>Shortness of breath</li> <li>A dry, hacking cough that doesn't get better</li> <li>Fatigue</li> <li>Weight loss for no known reason</li> <li>Aching muscles and joints</li> <li>Clubbing, which is the widening and rounding of the tips of the fingers or toes</li> </ul> <p>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.</p> <p >NIH: National Heart, Lung, and Blood Institute</p>|HPO2016_07_04:Replacement of normal lung tissues are progressively replaced by fibroblasts and collagen. [DDD:tkuijpers, HPO:probinson]
Me Sh Disease Class
Infections; Respiratory Tract DiseasesInfections; Respiratory Tract Diseases; Pathological Conditions, Signs and SymptomsRespiratory Tract Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Infections; Respiratory Tract DiseasesPathological Conditions, Signs and Symptoms; Infections; Respiratory Tract DiseasesRespiratory Tract Diseases
Umls Semantic Type Name
Disease or Syndrome