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Related Concept Videos

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...
Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum sickness, a systemic...
Endocarditis II: Clinical Features of Infective Endocarditis01:25

Endocarditis II: Clinical Features of Infective Endocarditis

Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

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...
Hypersensitivity Reactions: Delayed Hypersensitivity Reactions01:29

Hypersensitivity Reactions: Delayed Hypersensitivity Reactions

Delayed-Type Hypersensitivity (DTH), or Type IV hypersensitivity, is a cell-mediated immune response. It occurs when T cells, rather than antibodies, mediate a reaction to specific antigens. It is characterized by a delayed onset (1-2 days) and involves the recruitment of macrophages to the inflammation site.The initiation of a DTH response begins with the sensitization of T cells. During this phase, which lasts at least 1-2 weeks, antigen-specific T cells are activated, clonally expanded, and...
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Other Disorders of Digestive System

The gastrointestinal tract is susceptible to various disorders. If the lower esophageal sphincter is damaged, stomach acid can flow back into the esophagus, causing irritation and inflammation of the lining. This condition is called gastroesophageal reflux disease (known as heartburn) and may cause chest pain and difficulty swallowing. In the stomach, prolonged use of nonsteroidal anti-inflammatory drugs like aspirin, chronic alcohol consumption, bacterial infections such as Helicobacter...

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Related Experiment Video

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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
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Erdheim-Chester disease.

Julien Haroche1, Laurent Arnaud, Fleur Cohen-Aubart

  • 1Department of Internal Medicine, French Reference Center for Rare Autoimmune and Systemic Diseases, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, 47-83 boulevard de l'Hôpital, 75651 Paris Cedex 13, France. julien.haroche@psl.aphp.fr

Rheumatic Diseases Clinics of North America
|April 20, 2013
PubMed
Summary

Erdheim-Chester disease (ECD), a rare histiocytosis, is diagnosed via tissue biopsy. BRAFV600E mutation and central nervous system involvement impact prognosis, while interferon alpha and vemurafenib show therapeutic promise.

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Area of Science:

  • Oncology
  • Pathology
  • Genetics

Background:

  • Erdheim-Chester disease (ECD) is a rare non-Langerhans' cell histiocytosis.
  • Central nervous system involvement is a critical prognostic indicator in ECD.
  • Histiocytes, foamy and CD68+ CD1a-, are diagnostic hallmarks via tissue biopsy.

Purpose of the Study:

  • To summarize diagnostic criteria for Erdheim-Chester disease.
  • To highlight prognostic factors including CNS involvement.
  • To review current and emerging therapeutic strategies for ECD.

Main Methods:

  • Review of diagnostic criteria for ECD.
  • Analysis of prognostic factors in ECD patient cohorts.
  • Evaluation of therapeutic outcomes for interferon alpha and vemurafenib.

Main Results:

  • ECD diagnosis relies on characteristic histiocytes in tissue biopsies.
  • BRAFV600E mutation occurs in over 50% of ECD cases.
  • Interferon alpha improves survival; vemurafenib shows benefit in BRAFV600E-mutated, refractory ECD.

Conclusions:

  • Early diagnosis and management of ECD are crucial.
  • Targeting BRAFV600E mutation with vemurafenib offers a promising therapeutic avenue for severe ECD.
  • Further research into ECD pathogenesis and treatment is warranted.