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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

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Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
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Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
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Chronic obstructive pulmonary disease is a common, preventable, and treatable respiratory disorder characterized by persistent symptoms and progressive airflow limitation. This limitation results from a combination of small-airway disease (obstructive bronchiolitis) and parenchymal destruction (emphysema), both driven by chronic inflammation from exposure to harmful particles or gases.The disease includes two main pathological entities: emphysema, marked by destruction of alveolar walls and...
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Chronic bronchitis is a key phenotype of chronic obstructive pulmonary disease (COPD), characterized by airway-centered inflammation and mucus overproduction. It develops from long-term exposure to harmful particles or gases, most commonly cigarette smoke, which triggers a persistent inflammatory response.Cellular and Structural ChangesInflammation initially affects the large bronchi and later the smaller airways, with infiltration by immune cells, including neutrophils, macrophages, and...
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Refined Murine Model of Idiopathic Pulmonary Fibrosis
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Idiopathic pulmonary fibrosis: evolving concepts.

Jay H Ryu1, Teng Moua1, Craig E Daniels1

  • 1Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN.

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Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease often fatal. Current understanding points to genetic and environmental factors driving abnormal repair after lung injury, with limited effective therapies available.

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Area of Science:

  • Pulmonology
  • Pathology
  • Radiology

Background:

  • Idiopathic pulmonary fibrosis (IPF) is a major cause of interstitial lung disease, predominantly affecting older adults.
  • IPF is characterized by usual interstitial pneumonia (UIP) pattern without a known cause of lung injury.
  • The understanding of IPF pathogenesis has shifted towards dysregulated repair mechanisms following alveolar epithelial injury.

Purpose of the Study:

  • To review the current understanding of idiopathic pulmonary fibrosis (IPF).
  • To discuss diagnostic criteria and imaging findings for IPF.
  • To highlight the challenges in IPF management and the need for informed decision-making.

Main Methods:

  • Literature search of PubMed using terms 'idiopathic pulmonary fibrosis' and 'usual interstitial pneumonia'.
  • Inclusion of human studies published from January 1, 2000, to December 31, 2013, plus key earlier references.
  • Review of diagnostic criteria, pathogenesis, imaging, and clinical course of IPF.

Main Results:

  • High-resolution CT (HRCT) can diagnose IPF with typical findings (reticular opacities, traction bronchiectasis, honeycombing).
  • Surgical lung biopsy may be required when HRCT findings are atypical.
  • IPF is considered a heterogeneous disorder influenced by genetic and environmental interactions.

Conclusions:

  • Effective therapies for IPF remain elusive, necessitating informed management decisions, including lung transplantation.
  • The unpredictable clinical course of IPF can include acute exacerbations.
  • Further research into IPF mechanisms is ongoing despite current therapeutic limitations.