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Related Concept Videos

Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

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...
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

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 (ECE). Of...
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors01:28

Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors

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...
Portal Hypertension01:22

Portal Hypertension

Portal hypertension is an increase in blood pressure within the portal venous system. Normally, this pressure is less than 5 mmHg. It is considered clinically significant when it rises above 10 mmHg. At this threshold, complications from altered blood flow and venous congestion emerge.EtiologyPortal hypertension arises from conditions that impede blood flow through the liver. The most common cause is cirrhosis, in which chronic liver injury leads to fibrotic scarring. This fibrosis narrows or...
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

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.
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Systemic sclerosis-associated pulmonary arterial hypertension.

Jérôme Le Pavec1, Marc Humbert, Luc Mouthon

  • 1Division of Pulmonary and Critical Care Medicine, Johns Hopkins University Department of Medicine, 1830 East Monument Street, Baltimore, MD 21287, USA.

American Journal of Respiratory and Critical Care Medicine
|March 3, 2010
PubMed
Summary

Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) has a poor prognosis and suboptimal response to therapies approved for idiopathic PAH. New research is needed to understand SSc-PAH pathogenesis and develop targeted treatments.

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Area of Science:

  • Cardiovascular Sciences
  • Rheumatology
  • Pulmonology

Background:

  • Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases like systemic sclerosis (SSc).
  • SSc-associated PAH (SSc-PAH) significantly worsens patient outcomes and is the leading cause of mortality in SSc patients.
  • Current PAH therapies show limited efficacy in SSc-PAH, and survival rates remain poor.

Purpose of the Study:

  • To highlight the critical need for improved understanding and treatment strategies for SSc-PAH.
  • To identify key differences contributing to the poor prognosis of SSc-PAH compared to idiopathic PAH (IPAH).
  • To emphasize the necessity for developing novel diagnostic and therapeutic approaches for SSc-PAH.

Main Methods:

  • Review of current literature on PAH pathogenesis, diagnosis, and treatment, with a focus on SSc-PAH.
  • Comparative analysis of clinical and prognostic factors between SSc-PAH and IPAH.
  • Identification of gaps in current diagnostic tools and therapeutic responses for SSc-PAH.

Main Results:

  • SSc-PAH exhibits distinct clinical and prognostic features compared to IPAH, potentially due to heightened autoimmune responses, comorbidities, and multi-organ involvement.
  • Existing diagnostic markers and therapeutic response assessments for IPAH are inadequate for SSc-PAH.
  • Suboptimal response to established PAH therapies underscores the need for SSc-specific treatment strategies.

Conclusions:

  • Urgent need for enhanced understanding of SSc-PAH pathogenesis, including genetic factors.
  • Development of reliable tools for assessing functional impairment and monitoring treatment response in SSc-PAH is crucial.
  • Novel, targeted therapies are required to improve outcomes for patients with SSc-PAH.