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Related Concept Videos

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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Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

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The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
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Pericarditis I: Introduction01:22

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Pericarditis is defined as the inflammation of the pericardium, the thin, sac-like membrane surrounding the heart. This condition can cause significant chest pain and other symptoms, often necessitating medical intervention. The pericardium has two layers: the inner visceral layer and the outer parietal layer, separated by a small amount of fluid that reduces friction during heartbeats.Types of PericarditisPericarditis can be classified into several types based on the duration and nature of the...
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Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

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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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Chronic Bowel Disorders: Introduction01:17

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Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
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An Adoptive Transfer Model of Rheumatoid Arthritis in Mice
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[Restatement. Relapsing polychondritis].

Philippe Mertz1, Laurent Arnaud1

  • 1Service de rhumatologie, Centre national de référence des maladies auto-immunes systémiques rares de l'Est et du Sud-Ouest (RESO), hôpital de Hautepierre, Strasbourg, France.

La Revue Du Praticien
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PubMed
Summary
This summary is machine-generated.

Relapsing polychondritis (RP) is a rare systemic disease. Early diagnosis and screening for tracheobronchial issues are vital for managing RP and its severe complications.

Keywords:
Polychondritis, Relapsing

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Area of Science:

  • Rheumatology
  • Immunology
  • Genetics

Context:

  • Relapsing polychondritis (RP) is a systemic autoimmune disease.
  • Diagnosis can be challenging due to variable initial presentations.
  • Tracheobronchial involvement significantly impacts RP morbidity and mortality.

Purpose:

  • To highlight diagnostic challenges in relapsing polychondritis.
  • To emphasize the importance of screening for specific manifestations.
  • To discuss differential diagnoses and associated conditions.

Summary:

  • Relapsing polychondritis (RP) diagnosis is complex, with typical chondritis present in only a third of cases.
  • Three distinct RP phenotypes require tailored management and follow-up.
  • Systematic screening for tracheobronchial disease is crucial due to its high mortality risk.
  • Consider UBA1 mutations for VEXAS syndrome in older males with specific symptoms.
  • Rule out ANCA-associated vasculitis and identify co-existing autoimmune diseases.

Impact:

  • Improved diagnostic strategies for relapsing polychondritis.
  • Enhanced patient management through phenotype-specific approaches.
  • Reduced mortality by prioritizing screening for life-threatening complications.
  • Potential for earlier identification of VEXAS syndrome in at-risk populations.