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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.
There are various classifications for PH, each relating to different underlying causes and also...
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Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
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Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

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Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
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Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

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Phosphodiesterase 5 (PDE5) inhibitors are potent enzymes that function to hydrolyze cyclic nucleotides to their corresponding 5' monophosphates. Their unique biochemical properties have been applied in treating Pulmonary Arterial Hypertension (PAH).
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...
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Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
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Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers01:26

Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers

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Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
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Related Experiment Video

Updated: Jun 6, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Pathologic differences between systemic sclerosis-associated and idiopathic pulmonary arterial hypertension.

Samuel H Friedman1, Russell A Harley2, Jacob Williams3

  • 1Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Department of Medicine, Medical University of South Carolina, Charleston, SC, USA.

Journal of Scleroderma and Related Disorders
|December 2, 2024
PubMed
Summary

Systemic sclerosis-associated pulmonary arterial hypertension lungs show distinct interstitial fibrosis and immune cell infiltration compared to idiopathic pulmonary arterial hypertension. These differences may explain varied treatment responses in pulmonary arterial hypertension patients.

Keywords:
IPAHPAHSSc-PAHimmunohistochemistryscleroderma

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Area of Science:

  • Pulmonary Hypertension Research
  • Cardiovascular Pathology
  • Immunohistochemistry

Background:

  • Pulmonary arterial hypertension (PAH) therapies improve outcomes for idiopathic PAH but not systemic sclerosis-associated PAH (SSc-PAH).
  • Understanding histological and immunophenotypic differences between idiopathic PAH and SSc-PAH is crucial for targeted therapies.

Purpose of the Study:

  • To investigate the distinct histological and immunophenotypic characteristics of pulmonary vasculopathy in SSc-PAH versus idiopathic PAH.
  • To identify potential reasons for differential treatment responses and survival rates.

Main Methods:

  • Semi-quantitative lung morphometry analysis of bio-banked tissue from SSc-PAH (n=24), idiopathic PAH (n=9), and control (n=13) subjects.
  • Histological staining (H&E, VVG) and immunohistochemistry for inflammatory and fibrosis mediators.
  • Correlation with baseline demographic and hemodynamic data.

Main Results:

  • Plexiform lesions were absent in SSc-PAH but present in 55% of idiopathic PAH cases.
  • SSc-PAH lungs exhibited significantly greater interstitial fibrosis and cellularity (p<0.001).
  • Increased interstitial infiltration of CD3 T-cells, CD20 B-cells, and CD163 macrophages observed in SSc-PAH.

Conclusions:

  • SSc-PAH lungs possess distinct pulmonary vascular pathology and significant interstitial fibrosis compared to idiopathic PAH.
  • These findings highlight SSc-PAH heterogeneity and may inform future therapeutic strategies.
  • Further research into immune cell roles in SSc-PAH pathogenesis is warranted.