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Related Concept Videos

Atherosclerosis I: Introduction01:30

Atherosclerosis I: Introduction

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Atherosclerosis is a progressive disorder characterized by the buildup of plaques on the arterial inner wall, causing them to narrow and harden over time. These plaques comprise lipids, calcium, blood components, carbohydrates, and fibrous tissue. The process primarily affects the intima of large and medium-sized arteries, reducing blood flow in any artery.Etiology and risk factorsThe cause of atherosclerosis is multifactorial, involving a complex interplay among endothelial injury, lipid...
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Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

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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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Heart failure (HF) is a progressive syndrome involving ventricles that leads to inadequate cardiac output. It can be classified based on location and output or ejection fraction. Ejection fraction (EF) is an essential measurement in the diagnosis and surveillance of HF. Reduced EF corresponds to systolic heart failure (HFrEF). However, HF with preserved ejection fraction (HFpEF) is becoming increasingly prevalent. Also known as diastolic HF, this form of HF is related to aging. The...
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Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

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Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
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Coronary Artery Disease II: Pathophysiology01:26

Coronary Artery Disease II: Pathophysiology

70
Coronary Artery Disease (CAD) originates from a series of events that impair the function of coronary arteries, the blood vessels responsible for delivering oxygen-rich blood to the heart muscle. The pathophysiology of CAD is closely linked to atherosclerosis, a chronic inflammatory and lipid-driven condition affecting the vascular endothelium.1. Endothelial DamageThe process begins with damage to the vascular endothelium, which serves as a protective barrier between the blood and the vessel...
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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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Related Experiment Video

Updated: Oct 1, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Pathophysiology of systemic sclerosis (scleroderma).

Ann-Helen Rosendahl1, Katrin Schönborn1,2, Thomas Krieg1,2,3,4

  • 1Translational Matrix Biology, University of Cologne, Medical Faculty, Cologne, Germany.

The Kaohsiung Journal of Medical Sciences
|March 2, 2022
PubMed
Summary
This summary is machine-generated.

Systemic sclerosis, an autoimmune fibrotic disease, involves complex pathophysiology including endothelial damage and fibroblast activation. While a cure is elusive, recent breakthroughs offer targeted therapies for organ complications.

Keywords:
extracellular matrixfibroblastfibrosisinflammation

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Area of Science:

  • Immunology
  • Rheumatology
  • Pathophysiology

Background:

  • Systemic sclerosis (scleroderma) is a chronic, autoimmune-triggered fibrotic disease affecting skin and internal organs.
  • Pathophysiology involves endothelial damage, inflammation, and fibroblast activation leading to tissue fibrosis.
  • Genetic predisposition plays a role in immune system dysregulation, cytokine release, and autoantibody production.

Purpose of the Study:

  • To review the complex pathophysiology of systemic sclerosis.
  • To highlight recent advancements in understanding disease mechanisms.
  • To discuss emerging therapeutic strategies targeting specific disease pathways.

Main Methods:

  • Literature review of systemic sclerosis pathophysiology.
  • Analysis of immune system involvement (innate and acquired).
  • Examination of fibroblast activation and extracellular matrix deposition.

Main Results:

  • Identified key steps: endothelial damage, inflammation, fibroblast-to-myofibroblast transition, and fibrosis.
  • Highlighted the role of cytokines, chemokines, and autoantibodies in disease progression.
  • Noted significant progress in managing organ-specific complications.

Conclusions:

  • Systemic sclerosis pathogenesis is multifactorial, involving genetic and immune components.
  • New therapeutic concepts are emerging based on a deeper understanding of pathophysiology.
  • Targeted therapies offer promise for managing systemic sclerosis and its complications.