Related Experiment Video

Updated: Apr 30, 2026

Implantation of Total Artificial Heart in Congenital Heart Disease
07:27

Implantation of Total Artificial Heart in Congenital Heart Disease

Published on: July 18, 2014

28.4K

Rapid progression to cardiac tamponade in Erdheim-Chester disease despite treatment with interferon alpha

Afif Nakhleh1, Gleb Slobodin1, Nizar Elias1

  • 1a Department of Internal Medicine A , Bnai Zion Medical Center, Faculty of Medicine , Technion , Haifa , Israel.

Modern Rheumatology
|April 24, 2014
PubMed

Insights

Erdheim-Chester disease (ECD), a rare histiocytosis, can cause cardiac tamponade. This case highlights progression to cardiac tamponade despite interferon alpha treatment in a patient with multi-organ involvement.

Area of Science:

  • Cardiovascular Pathology
  • Rare Diseases
  • Histiocytosis

Background:

  • Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis with varied symptoms, often presenting as bone pain.
  • Extraskeletal involvement occurs in about 75% of patients, with cardiac issues seen in up to 45%.

Observation:

  • Pericardial involvement is the most frequent cardiac manifestation of ECD.
  • Cardiac tamponade is an exceptionally rare complication of ECD.

Findings:

  • This report details a case of ECD with multi-organ involvement and a small pericardial effusion.
  • The effusion progressed to cardiac tamponade despite treatment with interferon alpha.

Implications:

  • This case underscores the potential for rapid cardiac deterioration in ECD patients.
  • It suggests that interferon alpha may not always prevent the progression of cardiac complications in ECD.
  • Further research into ECD's cardiac manifestations and treatment strategies is warranted.

Keywords:
Erdheim–Chester diseaseInterferon alphaPericarditis tamponade

More Related Videos

Induction and Phenotyping of Acute Right Heart Failure in a Large Animal Model of Chronic Thromboembolic Pulmonary Hypertension07:41

Induction and Phenotyping of Acute Right Heart Failure in a Large Animal Model of Chronic Thromboembolic Pulmonary Hypertension

Published on: March 17, 2022

2.4K
Author Spotlight: A Neonatal Heterotopic Rat Heart Transplantation Model for the Study of Endothelial-to-Mesenchymal Transition08:38

Author Spotlight: A Neonatal Heterotopic Rat Heart Transplantation Model for the Study of Endothelial-to-Mesenchymal Transition

Published on: July 21, 2023

1.7K

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...
1.0K
Myocarditis I: Introduction01:21

Myocarditis I: Introduction

Myocarditis is inflammation of the myocardium, which is the muscular layer of the heart.EtiologyMyocarditis has a diverse etiology, including a wide range of infectious and non-infectious causes:Infectious CausesViral: Common viruses include Coxsackie A and B, adenovirus, parvovirus B19, enteroviruses, and influenza A.Bacterial: Examples include infections caused by Streptococcus, Staphylococcus, and Mycoplasma species.Rickettsial: Infections like Rocky Mountain spotted fever can result in...
645
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
790
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...
1.3K
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...
929
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

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...
952