Related Experiment Video
Updated: Jul 20, 2026
06:08
Assessment of Chimeric Antigen Receptor T Cell-Associated Toxicities Using an Acute Lymphoblastic Leukemia Patient-Derived Xenograft Mouse Model
Published on: February 10, 2023
Relapsing polychondritis: a chameleon among orphan diseases
Sabine Schumacher1, Herwig Pieringer2,3
1Academic Research Unit, 2nd Department of Medicine, Kepler University Hospital Linz, Krankenhausstraße 9, 4020, Linz, Austria.
Wiener Medizinische Wochenschrift (1946)
|April 2, 2017
Summary
Relapsing polychondritis (RPC) is a rare autoimmune disease causing cartilage inflammation and organ damage. Early diagnosis and interdisciplinary management are key for better patient outcomes.
Area of Science:
- Rheumatology
- Immunology
- Genetics
Background:
- Relapsing polychondritis (RPC) is a rare systemic autoimmune disease characterized by recurrent inflammation of cartilage.
- Etiology is largely unknown, but genetic predisposition is suspected, and associations with other autoimmune disorders exist.
- RPC affects cartilage in various organs, including ears, nose, joints, trachea, aorta, and coronary arteries, leading to fibrosis and organ damage.
Purpose of the Study:
- To provide a comprehensive overview of the current knowledge on relapsing polychondritis.
- To elucidate the pathogenesis, clinical presentation, diagnostic challenges, and therapeutic strategies for RPC.
- To emphasize the importance of an interdisciplinary approach in managing RPC.
Main Methods:
- Review of existing literature on relapsing polychondritis.
- Synthesis of information regarding etiology, clinical manifestations, and diagnostic criteria.
- Summary of current treatment options and prognostic factors.
Main Results:
- RPC presents heterogeneously, affecting multiple organ systems with cartilage.
- Diagnosis can be challenging due to the diverse clinical picture and potential overlap with other autoimmune conditions.
- Treatment involves a range of options, including glucocorticoids, dapsone, DMARDs, and biologics.
Conclusions:
- An interdisciplinary approach is crucial for accurate diagnosis and effective management of RPC.
- Treatment strategies should be tailored to individual patient needs and disease severity.
- Prognosis varies significantly based on the extent of organ damage, highlighting the need for ongoing research and improved therapies.
Related Concept Videos
01:29Autoimmune Disorders
Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune system...
Concept and Mechanism of Autoimmune Diseases
The immune system...
01:17Chronic Bowel Disorders: Introduction
Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
01:24Chronic Obstructive Pulmonary Disease
COPD is defined as a heterogeneous lung condition marked by persistent respiratory symptoms such as dyspnea, cough, and sputum production, caused by abnormalities in the airways that cause airflow obstruction.
Smoking is a primary risk factor for COPD, with over 80% of patients having a history of it. Patients typically experience progressive dyspnea or labored breathing, frequent coughing, and recurrent pulmonary infections. Many eventually succumb to respiratory failure, characterized by...
Smoking is a primary risk factor for COPD, with over 80% of patients having a history of it. Patients typically experience progressive dyspnea or labored breathing, frequent coughing, and recurrent pulmonary infections. Many eventually succumb to respiratory failure, characterized by...
01:23Rheumatic 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...
01:12Chronic Inflammation: Introduction
Chronic inflammation is a prolonged, dysregulated immune response that persists for weeks to years when the inciting stimulus is difficult to eradicate or when self‑antigens drive ongoing reactivity. Morphologically, it is defined by mononuclear cell infiltration, progressive tissue destruction, and concurrent attempts at healing via angiogenesis and fibrosis. Compared with acute inflammation, edema is less prominent while cellular infiltration predominates; triggers include persistent...
01:25Inflammatory Bowel Disease III: Crohn's Disease
Crohn’s disease is a chronic, relapsing form of inflammatory bowel disease characterized by segmental, transmural inflammation that can affect any part of the gastrointestinal tract. Its pathogenesis arises from a combination of genetic susceptibility, environmental exposures, epithelial barrier dysfunction, and immune dysregulation. Together, these factors lead to an exaggerated immune response against components of the gut microbiome.Genetic and Environmental InfluencesMultiple genetic...