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Disease Profile
Systemic capillary leak syndrome
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
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Age of onset
Adult
ICD-10
I78.8
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
SCLS; Clarkson disease; Capillary leak syndrome;
Summary
Systemic capillary leak
In many cases the cause is not known (idiopathic SCLS).[2] Some people with SCLS report having had an infection (such as a
Symptoms
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
Medical Terms | Other Names |
Learn More:
HPO ID
|
---|---|---|
80%-99% of people have these symptoms | ||
Leukocytosis |
Elevated white blood count
High white blood count
Increased blood leukocyte number
[ more ] |
0001974 |
Pedal edema |
Fluid accumulation in lower limbs
Lower leg swelling
[ more ] |
0010741 |
30%-79% of people have these symptoms | ||
Abdominal pain |
Pain in stomach
Stomach pain
[ more ] |
0002027 |
Diarrhea |
Watery stool
|
0002014 |
Fatigue |
Tired
Tiredness
[ more ] |
0012378 |
Hypotension |
Low blood pressure
|
0002615 |
Myalgia |
Muscle ache
Muscle pain
[ more ] |
0003326 |
Pancreatitis |
Pancreatic inflammation
|
0001733 |
Pulmonary edema |
Excess fluid in lungs
Wet lung
[ more ] |
0100598 |
Rhinorrhea |
Runny Nose
|
0031417 |
Weight loss | 0001824 | |
5%-29% of people have these symptoms | ||
Abnormal renal tubule morphology | 0000091 | |
Arrhythmia |
Abnormal heart rate
Heart rhythm disorders
Irregular heart beat
Irregular heartbeat
[ more ] |
0011675 |
Cardiorespiratory arrest | 0006543 | |
Cough |
Coughing
|
0012735 |
Multiple myeloma | 0006775 | |
Myocarditis |
Inflammation of heart muscle
|
0012819 |
Oliguria | 0100520 | |
Pericarditis |
Swelling or irritation of membrane around heart
|
0001701 |
Pleural effusion |
Fluid around lungs
|
0002202 |
Renal insufficiency |
Renal failure
Renal failure in adulthood
[ more ] |
0000083 |
Venous thrombosis |
Blood clot in vein
|
0004936 |
Diagnosis
Treatment
Periodic infusions or the use of certain medications are used to try to prevent future episodes (
Research into how SCLS develops and factors that affect its course are underway, in hopes that a better understanding of SCLS will lead to more effective treatment options and a lower mortality rate.
Related diseases
Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.
Conditions with similar signs and symptoms from Orphanet
|
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Differential diagnosis includes sepsis, anaphylaxis, any other cause of capillary leakage and inferior vena cavainterruption (see this term). Chronic cases might be misdiagnosed as Gleich syndrome, venous stasis, protein-losing enteropathy and nephrotic syndrome.
Visit the Orphanet disease page for more information.
|
Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Social Networking Websites
- RareShare has a community for systemic capillary leak syndrome. This website connects people affected by rare medical conditions.
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- The National Cancer Institute glossary of terms includes an entry for capillary leak syndrome with monoclonal gammopathy. Click on National Cancer Institute to view the definition.
- Mayo Clinic has an information page on Systemic capillary leak syndrome.
- The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
In-Depth Information
- MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Systemic capillary leak syndrome. Click on the link to view a sample search on this topic.
References
- Dictionary of Cancer Terms: capillary leak syndrome. National Cancer Institute (NCI). https://www.cancer.gov/dictionary?CdrID=426421. Accessed 9/11/2014.
- Rajesh A. Idiopathic systemic capillary leak syndrome. UpToDate. May 20, 2016; https://www.uptodate.com/contents/idiopathic-systemic-capillary-leak-syndrome.
- Druey KM, Parikh SM. Idiopathic systemic capillary leak syndrome (Clarkson disease). J Allergy Clin Immunol. December 22, 2016; https://www.sciencedirect.com/science/article/pii/S0091674916324605.
- Amoura Z. Systemic capillary leak syndrome. Orphanet. November 2012; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=188.
- Pineton de Chambrun M, Gousseff M, Mauhin W, et al. Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome. Am J Med. June 9, 2017; https://www.ncbi.nlm.nih.gov/pubmed/28602874.
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