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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: 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...
Chronic Obstructive Pulmonary Disease-III: Symptoms and Complications.01:25

Chronic Obstructive Pulmonary Disease-III: Symptoms and Complications.

Understanding the variety of primary symptoms and systemic complications that characterize chronic obstructive pulmonary disease (COPD) is crucial for healthcare professionals.
Symptoms of COPD can be classified as primary or systemic. Primary symptoms relate to reduced airflow, while systemic or extrapulmonary symptoms relate to COPD's broader impact on the body.
Primary Symptoms of COPD:
Pulmonary Edema II: Pathophysiology01:18

Pulmonary Edema II: Pathophysiology

Pulmonary edema is the accumulation of fluid in the interstitial and alveolar spaces of the lungs, impairing gas exchange and oxygen delivery. It may be cardiogenic or noncardiogenic, but both reduce oxygenation and lung compliance.Cardiogenic Pulmonary EdemaCardiogenic edema results from increased hydrostatic pressure in pulmonary capillaries, usually due to left ventricular dysfunction from myocardial infarction, heart failure, or valvular disease. Ineffective cardiac pumping causes blood to...
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure01:16

Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure

Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue, improving...
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...

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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

Pulmonary hypertension in systemic sclerosis.

Soumya Chatterjee1

  • 1Department of Rheumatic and Immunologic Diseases, Orthopedic and Rheumatology Institute, Cleveland Clinic, Cleveland, OH 44195, USA. chattes@ccf.org

Seminars in Arthritis and Rheumatism
|November 5, 2010
PubMed
Summary
This summary is machine-generated.

Pulmonary hypertension in systemic sclerosis (SSc-PH) presents unique pathology and a worse prognosis than idiopathic pulmonary arterial hypertension (IPAH). Current treatments are less effective for SSc-PH, necessitating further research for better therapeutic strategies.

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Area of Science:

  • Rheumatology
  • Pulmonology
  • Pathology

Background:

  • Pulmonary hypertension (PH) in systemic sclerosis (SSc-PH) is a complex condition with distinct characteristics.
  • It can occur independently or alongside interstitial lung disease and left heart disease.

Purpose of the Study:

  • To delineate the clinical subtypes, pathogenesis, pathology, diagnostic evaluation, treatment, and prognosis of SSc-PH.
  • To emphasize its fundamental differences from idiopathic pulmonary arterial hypertension (IPAH).

Main Methods:

  • A comprehensive literature review was conducted using Medline (1969-2010).
  • Keywords included scleroderma, pulmonary hypertension, pathogenesis, pathology, treatment, and prognosis.
  • Key differences in pathogenesis, histopathology, treatment response, and prognosis compared to IPAH were analyzed.

Main Results:

  • SSc-PH exhibits unique histopathologic findings, including intimal fibrosis and absence of plexiform lesions, with a high prevalence of pulmonary veno-occlusive disease-like lesions.
  • Diagnostic tools like the 6-minute walk test and NT-proBNP have limitations in SSc-PH evaluation.
  • Calcium channel blockers are ineffective, anticoagulation requires caution, and approved therapies show poorer efficacy, leading to a worse prognosis compared to IPAH.

Conclusions:

  • SSc-PH is a complex condition with a poorer therapeutic response and worse outcome than IPAH.
  • Recent research offers insights into SSc-PH pathophysiology and pathogenesis.
  • Further investigation is crucial for understanding pathogenesis and developing improved therapeutic strategies.