Related Experiment Video
Updated: Mar 9, 2026
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
21.0K
Idiopathic systemic capillary leak syndrome (Clarkson disease)
Kirk M Druey1, Samir M Parikh2
1Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases/National Institutes of Health, Bethesda, Md.
The Journal of Allergy and Clinical Immunology
|December 26, 2016
Summary
Systemic capillary leak syndrome (SCLS) causes sudden fluid and protein leakage, mimicking anaphylaxis. Early diagnosis and treatment are crucial due to high mortality, despite its rarity and unknown cause.
Area of Science:
- Medicine
- Pathology
- Immunology
Background:
- Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent episodes of shock, hemoconcentration, and hypoalbuminemia.
- First described by Dr. Bayard Clarkson in 1960, SCLS involves the unexplained leakage of plasma from the vasculature into tissues.
- The condition is likely underdiagnosed due to low awareness and can be mistaken for more common allergic reactions.
Purpose of the Study:
- To highlight the key features of Systemic Capillary Leak Syndrome (SCLS).
- To emphasize the importance of recognizing SCLS in clinical practice.
- To underscore the need for increased awareness and timely diagnosis of SCLS.
Main Methods:
- Literature review of reported cases since 1960.
- Analysis of clinical presentation, including shock and fluid shifts.
- Comparison of SCLS symptoms with other plasma leakage syndromes.
Main Results:
- SCLS presents with transient, severe, yet reversible hemoconcentration and hypoalbuminemia.
- Plasma from affected individuals can induce shock-like symptoms in animal models.
- Despite fewer than 500 reported cases, SCLS has a high mortality rate if untreated.
Conclusions:
- Allergists and clinicians should consider SCLS in patients presenting with symptoms similar to angioedema or anaphylaxis.
- Increased awareness is critical for improving the diagnosis and management of SCLS.
- While the molecular basis of SCLS is still under investigation, recent research has advanced understanding of its pathogenesis.
Related Concept Videos
01:24Nephrotic Syndrome I : Introduction
790
Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
790
01:25Pleural Effusion I: Introduction
5.0K
Pleural effusion is an abnormal fluid accumulation in the pleural cavity, a narrow space between the lungs and the chest wall. It is not a disease per se but rather a symptom or indication of an underlying disease. In normal circumstances, this space contains a small amount of fluid (5 to 15 mL), a lubricant facilitating the non-frictional movement of the pleural surfaces.
There are two main types of pleural effusion: transudative and exudative. They are differentiated using Light's...
There are two main types of pleural effusion: transudative and exudative. They are differentiated using Light's...
5.0K
01:19Hypersensitivity Reactions: Immune-Complex Reactions
76
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...
76
01:26Nephrotic Syndrome II : Assessment and Medical Management
292
IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document...
292
01:25Endocarditis II: Clinical Features of Infective Endocarditis
668
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...
668
01:24Nephrotic Syndrome III : Nursing Management
403
Nursing management for nephrotic syndrome adapts as the disease progresses, with strategies evolving to address advancing symptoms and complications.Early-Stage Management In the early stages, nursing interventions for nephrotic syndrome resemble those used in managing acute glomerulonephritis, focusing on symptom monitoring, fluid balance, and managing mild to moderate edema.Vital Signs: Regularly monitor blood pressure, pulse, respiratory rate, and temperature to promptly identify...
403