"evidence of restriction (reduced vital capacity, often with an increased FEV1/FVC ratio); and impaired gas exchange (increased alveolar–arterial oxygen pressure difference (P[A–a]O2) decreased alveolar oxygen pressure (PaO2) at rest or exercise, or decreased diffusing capacity (DLCO)) 5"
"evidence of restriction (reduced vital capacity, often with an increased FEV1/FVC ratio); and impaired gas exchange (increased alveolar–arterial oxygen pressure difference (P[A–a]O2) decreased alveolar oxygen pressure (PaO2) at rest or exercise, or decreased diffusing capacity (DLCO)) 5"
"evidence of restriction (reduced vital capacity, often with an increased FEV1/FVC ratio); and impaired gas exchange (increased alveolar–arterial oxygen pressure difference (P[A–a]O2) decreased alveolar oxygen pressure (PaO2) at rest or exercise, or decreased diffusing capacity (DLCO)) 5"
"evidence of restriction (reduced vital capacity, often with an increased FEV1/FVC ratio); and impaired gas exchange (increased alveolar–arterial oxygen pressure difference (P[A–a]O2) decreased alveolar oxygen pressure (PaO2) at rest or exercise, or decreased diffusing capacity (DLCO)) 5"
"In 2018, these criteria were revised in a collaborative statement by the ATS, ERS, Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT) 14. The major and minor criteria were eliminated, and only the following diagnostic criteria remain:"
"exclusion of other known causes of interstitial lung disease (e.g. domestic and occupational environmental exposures, connective tissue disease, and drug toxicity)"
"Classification tends to ignore the extent and variety of lung injury in idiopathic pulmonary fibrosis; e.g. damage to alveolar lining cells, pulmonary vessels, and pleura may also be present. There is a marked decrease in the volume of alveolar gas and damage and loss of small airways, associated with mosaic attenuation, which may mislead radiologists into suggesting an alternative diagnosis 17."
"MUC5B, e.g. the rs35705950 single-nucleotide polymorphism is considered the strongest genetic risk factor for IPF"
"TERT, associated with dyskeratosis congenita"
"others: SFTPC, SFTPA2, RTEL1, hTR, ABCA3, DKC1, TINF2"
Expected headings
"Major criteria"
"Minor criteria"
"absence of alternative causes such as drug toxicity, environmental exposure (e.g. asbestos) or collagen vascular disease (e.g. systemic sclerosis, rheumatoid arthritis)"
"exclusion of other known causes of interstitial lung disease (e.g. domestic and occupational environmental exposures, connective tissue disease, and drug toxicity)"
"evidence of restriction (reduced vital capacity, often with an increased FEV1/FVC ratio); and impaired gas exchange (increased alveolar–arterial oxygen pressure difference (P[A–a]O2) decreased alveolar oxygen pressure (PaO2) at rest or exercise, or decreased diffusing capacity (DLCO)) 5"
"evidence of restriction (reduced vital capacity, often with an increased FEV1/FVC ratio); and impaired gas exchange (increased alveolar–arterial oxygen pressure difference (P[A–a]O2) decreased alveolar oxygen pressure (PaO2) at rest or exercise, or decreased diffusing capacity (DLCO)) 5"
"Idiopathic pulmonary fibrosis, as the name states, is idiopathic; however, there is an association with concurrent or previous history of smoking in ~40-80% of patients, and genetic factors 20-22. Up to 5-20% of patients with idiopathic pulmonary fibrosis have a family history of interstitial lung disease (ILD) or pulmonary fibrosis. It has been shown that fibroblasts in this process demonstrate neoplastic or neoproliferative characteristics 11."
"Classification tends to ignore the extent and variety of lung injury in idiopathic pulmonary fibrosis; e.g. damage to alveolar lining cells, pulmonary vessels, and pleura may also be present. There is a marked decrease in the volume of alveolar gas and damage and loss of small airways, associated with mosaic attenuation, which may mislead radiologists into suggesting an alternative diagnosis 17."
"others: SFTPC, SFTPA2, RTEL1, hTR, ABCA3, DKC1, TINF2"
"Classification tends to ignore the extent and variety of lung injury in idiopathic pulmonary fibrosis; e.g. damage to alveolar lining cells, pulmonary vessels, and pleura may also be present. There is a marked decrease in the volume of alveolar gas and damage and loss of small airways, associated with mosaic attenuation, which may mislead radiologists into suggesting an alternative diagnosis 17."