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Related Concept Videos

Pericarditis I: Introduction01:22

Pericarditis I: Introduction

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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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Pericarditis III: Medical Management01:17

Pericarditis III: Medical Management

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The primary objectives of managing pericarditis are to determine the underlying cause, provide effective therapy for treatment and symptom relief, and promptly detect signs and symptoms of cardiac tamponade. The following outlines the essential aspects of medical management for pericarditis:ObjectivesDetermine the Cause: Identifying the underlying cause of pericarditis is crucial for targeted treatment. Causes include viral infections, autoimmune diseases, post-cardiac injury syndrome, and...
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Rheumatic Heart Disease III: Medical Management01:21

Rheumatic Heart Disease III: Medical Management

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Rheumatic heart disease (RHD) management can be divided into two main strategies: prevention and long-term management.Primary PreventionPrimary prevention focuses on timely diagnosis and management of group A streptococcal pharyngitis to prevent acute rheumatic fever. The most widely used antibiotic for treating this condition is intramuscular benzathine penicillin G.Acute Rheumatic Fever TreatmentThe primary treatment goal for a patient diagnosed with acute rheumatic fever is to suppress the...
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Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

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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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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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Chronic Obstructive Pulmonary Disease-V: Management01:29

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Managing Chronic Obstructive Pulmonary Disease (COPD) involves a multifaceted approach to reduce symptoms, prevent exacerbations, improve overall health status, and slow disease progression. Key strategies include lifestyle modifications, pharmacotherapy, supportive therapies, and, in some cases, surgery. Here is an overview of the primary COPD management strategies:
Smoking Cessation
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[Relapsing polychondritis: What's new in 2017?]

J Dion1, G Leroux2, L Mouthon1

  • 1Service de médecine interne, Centre de référence maladies auto-immunes et systémiques rares d'Île-de-France, hôpital Cochin, université Paris Descartes, AP-HP, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France.

La Revue De Medecine Interne
|September 12, 2017
PubMed
Summary
This summary is machine-generated.

Relapsing polychondritis (RP) is a rare autoimmune disease affecting cartilage. Recent data shows improved survival and identifies distinct phenotypes, guiding treatment strategies from corticosteroids to biologics.

Keywords:
Clinical phenotypesMyelodysplasiaMyélodysplasiePhénotypes cliniquesPolychondrite atrophianteRelapsing polychondritisTracheobronchomalaciaTrachéobronchomalacie

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Area of Science:

  • Rheumatology
  • Immunology
  • Rare Diseases

Background:

  • Relapsing polychondritis (RP) is a rare autoimmune condition causing cartilage inflammation and systemic symptoms.
  • Pathophysiology remains poorly understood, but an autoimmune mechanism is suspected.
  • RP can be classified into three clinical phenotypes: hematological, respiratory, and mild, with varying prognoses.

Purpose of the Study:

  • To summarize current understanding of relapsing polychondritis.
  • To discuss recent findings on pathophysiology, clinical phenotypes, and survival.
  • To review diagnostic and therapeutic approaches for RP.

Main Methods:

  • Review of recent literature and clinical series on relapsing polychondritis.
  • Analysis of survival data and prognostic factors.
  • Evaluation of diagnostic tools like positron emission tomography.
  • Assessment of current and emerging treatment strategies.

Main Results:

  • Survival in RP has improved compared to historical data.
  • Poor prognostic factors include male gender, hematological disorders, and cardiac involvement.
  • Three distinct clinical phenotypes (hematological, respiratory, mild) have been identified.
  • Positron emission tomography shows potential for diagnosis and treatment monitoring.
  • Treatment remains empirical, often involving corticosteroids and immunosuppressants, with biologics showing varied outcomes.

Conclusions:

  • Relapsing polychondritis management is evolving with better understanding of phenotypes and prognosis.
  • Current treatments include corticosteroids, immunosuppressants, and biologics, with colchicine or watchful waiting for mild cases.
  • Further research is needed to establish evidence-based treatment guidelines.