Related Experiment Video
Updated: Jun 22, 2026
03:23
Three-Dimensional Cell Culture Models to Investigate the Epithelial Barrier in Eosinophilic Esophagitis
Published on: May 10, 2024
[Eosinophilic fasciitis and asplenia]
N T Baerlecken1, A Melzer, R E Schmidt
1Klinik für Immunologie und Rheumatologie, Medizinische Hochschule Hannover, Hannover, Deutschland. baerlecken.niklas@mh-hannover.de
Zeitschrift Fur Rheumatologie
|June 11, 2009
Summary
Eosinophilic fasciitis, a rare inflammatory condition, is now linked to congenital asplenia, a condition where the spleen is absent from birth. This case study explores this novel association in autoimmune disease research.
Area of Science:
- Rheumatology
- Immunology
- Genetics
Background:
- Eosinophilic fasciitis (Shulman syndrome) is a chronic inflammatory condition affecting fascia and septums, with known associations with various autoimmune and hematologic disorders.
- Systemic sclerosis shares several features with eosinophilic fasciitis, including immune system involvement.
- Congenital asplenia is a rare condition characterized by the absence of a spleen from birth, often associated with other congenital anomalies.
Observation:
- A unique case of a patient presenting with both eosinophilic fasciitis and congenital asplenia is detailed.
- This patient exhibited clinical and histopathological features consistent with Shulman syndrome.
Findings:
- The study reports the first-ever described association between eosinophilic fasciitis and congenital asplenia.
- This finding expands the spectrum of known comorbidities associated with eosinophilic fasciitis.
Implications:
- The association suggests potential shared pathophysiological mechanisms or genetic predispositions between eosinophilic fasciitis and congenital asplenia.
- Further research is warranted to elucidate the underlying causes and clinical significance of this comorbidity.
- Understanding this link may inform diagnostic approaches and management strategies for patients with either condition.
Related Concept Videos
01:28Amebiasis
Entamoeba histolytica, a protozoan parasite, is responsible for intestinal and extraintestinal amebiasis. Though a significant proportion of infections remain asymptomatic, approximately 50 million individuals annually are estimated to present with clinical disease, resulting in up to 100,000 deaths globally. The disease burden is disproportionately high in regions with lower socioeconomic status, such as parts of India, Africa, Mexico, and Latin America.Etiology and TransmissionThe infective...
01:28Asthma I: Introduction
Asthma is a chronic inflammatory disorder of the airways characterized by variable airflow obstruction and heightened bronchial responsiveness to a wide range of triggers. The underlying inflammation leads to airway swelling, mucus hypersecretion, and smooth muscle constriction, all of which narrow the airway lumen and impede airflow. Clinically, asthma presents with recurrent episodes of wheezing, shortness of breath, chest tightness, and coughing, symptoms that typically vary in intensity and...
01:13Asthma III: Clinical Manifestations
Asthma presents with a characteristic pattern of episodic respiratory symptoms that reflect underlying airway inflammation, bronchoconstriction, and mucus hypersecretion. Although severity varies among individuals, certain clinical manifestations are considered hallmarks of the disorder and often guide diagnosis and assessment.Respiratory SymptomsA persistent cough is one of the most common early features of asthma. It is frequently dry and tends to worsen at night or in the early morning,...
01:27Esophageal Achalasia
Esophageal achalasia is a chronic neurogenic disorder characterized by impaired relaxation of the lower esophageal sphincter (LES) and absent or ineffective peristalsis in the distal esophagus. This leads to a functional obstruction without a physical blockage, despite significant disruption of esophageal motility.EtiologyAchalasia is caused by degeneration of the myenteric (Auerbach's) plexus, specifically the loss of inhibitory ganglion cells that produce vasoactive intestinal peptide (VIP)...
01:30Asthma-IV: Diagnostic and Management
The diagnosis and management of asthma are comprehensive, encompassing clinical assessments, lung function tests, and pharmacological interventions. Here's an overview:
Clinical Assessment for Asthma:
This is the first step in diagnosing and managing asthma. It includes:
Clinical Assessment for Asthma:
This is the first step in diagnosing and managing asthma. It includes:
01:26Asthma-II: Pathophysiology and Classification
Asthma is a prevalent chronic respiratory condition marked by inflammation and hyperresponsiveness of the airways. Its pathophysiology involves complex interactions among inflammatory pathways, immune responses, and neural mechanisms.
Additionally, environmental and genetic factors play crucial roles in determining an individual's susceptibility to asthma and the severity of their condition.
Critical processes in asthma pathophysiology include:
Additionally, environmental and genetic factors play crucial roles in determining an individual's susceptibility to asthma and the severity of their condition.
Critical processes in asthma pathophysiology include: