Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

1.0K
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...
1.0K
Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

269
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...
269
Endocarditis II: Clinical Features of Infective Endocarditis01:25

Endocarditis II: Clinical Features of Infective Endocarditis

929
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...
929
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

1.3K
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...
1.3K
Hypersensitivity Reactions: Delayed Hypersensitivity Reactions01:29

Hypersensitivity Reactions: Delayed Hypersensitivity Reactions

359
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...
359
Other Disorders of Digestive System01:30

Other Disorders of Digestive System

1.7K
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...
1.7K

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Association between cumulative glucocorticoid exposure and epicardial adipose tissue volume in systemic lupus erythematosus.

EULAR rheumatology open·2026
Same author

Clonal expansion of CAR-T cells harboring lentivector integration in the PLAAT4 gene following anti-CD19 CAR T-cell therapy in a context of cutaneous T-cell lymphoma.

Haematologica·2026
Same author

Effect of a defatting pharmacologic cocktail with rapamycin during cold-to-warm ex situ perfusion of discarded human livers: A comparative study.

Surgery·2026
Same author

Efficacy and Tolerance of Cladribine for Non-Langerhans Cell Histiocytosis.

European journal of haematology·2026
Same author

Serum lipidomic and metabolomic signatures link epicardial adipose tissue to cardiovascular diseases in SLE: a post-hoc analysis.

Lupus science & medicine·2026
Same author

[Bispecific T-cell engagers for the treatment of autoimmune diseases: Current status and therapeutic perspectives].

La Revue de medecine interne·2026

Related Experiment Video

Updated: May 3, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
10:27

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Published on: December 15, 2011

23.9K

Erdheim-Chester disease.

Julien Haroche1, Laurent Arnaud, Fleur Cohen-Aubart

  • 1Department of Internal Medicine and French Reference Center for Rare Autoimmune and Systemic Diseases, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Paris, France, julien.haroche@psl.aphp.fr.

Current Rheumatology Reports
|February 18, 2014
PubMed
Summary

Erdheim-Chester disease (ECD) is a rare histiocytosis. BRAF(V600E) mutation is common, and BRAF inhibitors like vemurafenib show promise for treating severe cases.

More Related Videos

Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity
11:12

Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity

Published on: April 11, 2019

7.8K
Granulocyte-dependent Autoantibody-induced Skin Blistering
12:23

Granulocyte-dependent Autoantibody-induced Skin Blistering

Published on: October 12, 2012

9.9K

Related Experiment Videos

Last Updated: May 3, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
10:27

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Published on: December 15, 2011

23.9K
Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity
11:12

Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity

Published on: April 11, 2019

7.8K
Granulocyte-dependent Autoantibody-induced Skin Blistering
12:23

Granulocyte-dependent Autoantibody-induced Skin Blistering

Published on: October 12, 2012

9.9K

Area of Science:

  • Rare diseases
  • Histiocytosis
  • Oncology

Background:

  • Erdheim-Chester disease (ECD) is a rare, non-inherited histiocytosis of unknown origin.
  • Characterized by tissue infiltration of foamy histiocytes and fibrosis.
  • Distinctive diagnostic markers include CD68+ CD1a- histiocytes, specific bone scintigraphy, and CT findings.

Purpose of the Study:

  • To summarize the key features, diagnosis, and treatment of Erdheim-Chester disease.
  • To highlight the role of BRAF mutations and targeted therapies.

Main Methods:

  • Review of histiocyte analysis in tissue biopsies (CD68, CD1a markers).
  • Utilizing diagnostic imaging techniques like ⁹⁹Technetium bone scintigraphy and abdominal CT scans.
  • Analysis of systemic immune activation markers (IFNα, ILs, MCP-1) and BRAF mutation status.

Main Results:

  • ECD diagnosis relies on characteristic histiocyte markers and imaging.
  • Central nervous system involvement is a critical prognostic factor.
  • Interferon-α is an optimal initial therapy, improving survival despite potential tolerance issues.
  • Over half of ECD patients harbor the BRAF(V600E) mutation.
  • Vemurafenib, a BRAF inhibitor, demonstrated significant benefit in refractory ECD cases with this mutation.

Conclusions:

  • ECD is a complex systemic disease with significant morbidity.
  • Targeting the BRAF(V600E) mutation with vemurafenib offers a promising therapeutic avenue for select patients.
  • Further research into ECD pathogenesis and treatment is warranted.