MCID: SYS007
MIFTS: 33

Systemic Capillary Leak Syndrome

Categories: Rare diseases, Cardiovascular diseases, Bone diseases

Aliases & Classifications for Systemic Capillary Leak Syndrome

MalaCards integrated aliases for Systemic Capillary Leak Syndrome:

Name: Systemic Capillary Leak Syndrome 49 55
Capillary Leak Syndrome 49 55 69
Capillary Leak Syndrome with Monoclonal Gammopathy 49 69
Clarkson Disease 49 55
Scls 49 55
Periodic Systemic Capillary Leak Syndrome 49
Capillary Hyperpermeability Syndrome 55
Idiopathic Capillary Leak Syndrome 55

Characteristics:

Orphanet epidemiological data:

55
systemic capillary leak syndrome
Inheritance: Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: Adult; Age of death: adult;

Classifications:



External Ids:

Orphanet 55 ORPHA188
MESH via Orphanet 42 D019559
UMLS via Orphanet 70 C0343084
ICD10 via Orphanet 33 I78.8
UMLS 69 C2930938

Summaries for Systemic Capillary Leak Syndrome

NIH Rare Diseases : 49 Systemic capillary leak syndrome (SCLS) is a condition in which fluid and proteins leak out of tiny blood vessels, into surrounding tissues. This can result in dangerously low blood pressure (hypotensison), hypoalbuminemia, and a decrease in plasma volume (hemoconcentration). Initial symptoms may include fatigue, nausea, abdominal pain, extreme thirst, and sudden increase in body weight. Episodes of SCLS vary in frequency, with some people having one episode in their lifetime, and others having several per year. The severity also varies, and the condition can be fatal. In many cases the cause is not known (idiopathic SCLS). Some people with SCLS report having had an infection (such as a virus) before the episode. Some cases of capillary leak are associated with various conditions of the heart, kidney, or liver. Treatment during an episode may involve stabilizing the airway and breathing, and infusion of fluids, medications, or blood products. Periodic infusions or the use of certain medications may be used to prevent future episodes. Last updated: 7/31/2017

MalaCards based summary : Systemic Capillary Leak Syndrome, also known as capillary leak syndrome, is related to superior limbic keratoconjunctivitis and capillary leak syndrome. An important gene associated with Systemic Capillary Leak Syndrome is EDNRA (Endothelin Receptor Type A). The drugs Isotretinoin and Aldesleukin have been mentioned in the context of this disorder. Affiliated tissues include kidney, heart and liver.

Related Diseases for Systemic Capillary Leak Syndrome

Diseases in the Capillary Leak Syndrome family:

Systemic Capillary Leak Syndrome

Diseases related to Systemic Capillary Leak Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 45)
# Related Disease Score Top Affiliating Genes
1 superior limbic keratoconjunctivitis 10.9
2 capillary leak syndrome 10.9
3 compartment syndrome 10.3
4 polydactyly, postaxial, with progressive myopia 10.3
5 endotheliitis 10.3
6 hemolytic anemia 10.1
7 influenza 10.1
8 thrombotic thrombocytopenic purpura, congenital 10.1
9 lymphoma 10.0
10 dermatomyositis 10.0
11 metabolic encephalomyopathic crises, recurrent, with rhabdomyolysis, cardiac arrhythmias, and neurodegeneration 10.0
12 cerebritis 10.0
13 stroke, ischemic 9.8
14 autoimmune disease 1 9.8
15 cardiac arrest 9.8
16 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.8
17 alk-negative anaplastic large cell lymphoma 9.8
18 kawasaki disease 9.8
19 pulmonary edema 9.8
20 aspergillosis 9.8
21 allergic hypersensitivity disease 9.8
22 b-cell expansion with nfkb and t-cell anergy 9.8
23 autoimmune disease 9.8
24 hereditary angioedema 9.8
25 leukemia, chronic lymphocytic 2 9.8
26 hydrops, lactic acidosis, and sideroblastic anemia 9.8
27 angioedema 9.8
28 colorectal cancer 9.8
29 adult respiratory distress syndrome 9.8
30 complement component 4, partial deficiency of 9.8
31 anaplastic large cell lymphoma 9.8
32 pustulosis of palm and sole 9.8
33 renal cell carcinoma, nonpapillary 9.8
34 b-cell lymphomas 9.8
35 leukemia, chronic lymphocytic 9.8
36 childhood type dermatomyositis 9.8
37 peritonitis 9.8
38 diffuse large b-cell lymphoma 9.8
39 polycythemia 9.8
40 myeloma, multiple 9.8
41 polycythemia vera 9.8
42 leukemia 9.8
43 psoriasis 9.8
44 c1 inhibitor deficiency 9.8
45 leukemia, b-cell, chronic 9.8

Graphical network of the top 20 diseases related to Systemic Capillary Leak Syndrome:



Diseases related to Systemic Capillary Leak Syndrome

Symptoms & Phenotypes for Systemic Capillary Leak Syndrome

Drugs & Therapeutics for Systemic Capillary Leak Syndrome

Drugs for Systemic Capillary Leak Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 67)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Isotretinoin Approved Phase 3,Phase 2 4759-48-2 5282379 5538
2
Aldesleukin Approved Phase 3,Phase 2 110942-02-4, 85898-30-2
3
Dopamine Approved Phase 3 51-61-6, 62-31-7 681
4
Acetaminophen Approved Phase 3 103-90-2 1983
5
Ibuprofen Approved Phase 3 15687-27-1 3672
6
Metoclopramide Approved, Investigational Phase 3 364-62-5 4168
7
Ondansetron Approved Phase 3 99614-02-5 4595
8
Codeine Approved, Illicit Phase 3 76-57-3 5284371
9
Guaifenesin Approved, Investigational, Vet_approved Phase 3 93-14-1 3516
10
Vitamin A Approved, Nutraceutical, Vet_approved Phase 3 68-26-8, 11103-57-4 445354
11
Tretinoin Approved, Investigational, Nutraceutical Phase 3,Phase 2 302-79-4 5538
12 Analgesics Phase 3,Phase 2
13 Retinol palmitate Phase 3
14 Analgesics, Non-Narcotic Phase 3,Phase 2
15 Dermatologic Agents Phase 3,Phase 2
16 Vitamins Phase 3
17 Peripheral Nervous System Agents Phase 3,Phase 2
18 Antibodies, Monoclonal Phase 3
19 Anti-HIV Agents Phase 3,Phase 2
20 Anti-Infective Agents Phase 3,Phase 2
21 Immunoglobulins Phase 3,Phase 2
22 Interleukin-2 Phase 3,Phase 2
23 Keratolytic Agents Phase 3,Phase 2
24 Anti-Retroviral Agents Phase 3,Phase 2
25 Antibodies Phase 3,Phase 2
26 Antiviral Agents Phase 3,Phase 2
27
Serotonin Phase 3 50-67-9 5202
28 Respiratory System Agents Phase 3
29 Central Nervous System Depressants Phase 3
30 Serotonin Agents Phase 3
31 Serotonin Antagonists Phase 3
32 Chlorpheniramine, phenylpropanolamine drug combination Phase 3
33 Narcotics Phase 3
34 Gastrointestinal Agents Phase 3
35 Neurotransmitter Agents Phase 3
36 Tranquilizing Agents Phase 3
37 Cyclooxygenase Inhibitors Phase 3
38 Analgesics, Opioid Phase 3
39 Dopamine Agents Phase 3
40 Anti-Anxiety Agents Phase 3
41 Dopamine Antagonists Phase 3
42 Dopamine D2 Receptor Antagonists Phase 3
43 Antiemetics Phase 3
44 Anti-Inflammatory Agents Phase 3
45 Anti-Inflammatory Agents, Non-Steroidal Phase 3
46 Psychotropic Drugs Phase 3
47 Antipruritics Phase 3
48 Antipsychotic Agents Phase 3
49 Antipyretics Phase 3
50 Autonomic Agents Phase 3

Interventional clinical trials:

(show all 12)

# Name Status NCT ID Phase Drugs
1 Monoclonal Antibody Ch14.18, Sargramostim, Aldesleukin, and Isotretinoin After Autologous Stem Cell Transplant in Treating Patients With Neuroblastoma Completed NCT01041638 Phase 3 Isotretinoin
2 Toxicity Substudy of Evaluation of Subcutaneous Proleukin in a Randomised International Trial (ESPRIT): TOXIL-2 Substudy Terminated NCT00147355 Phase 3 ondansetron, ibuprofen, paracetamol;Ondansetron, ibuprofen, paracetamol;metoclopramide, ibuprofen, paracetamol;Metoclopramide, codeine phosphate, ibuprofen, paracetamol
3 NB2013-HR German (GPOH) / Dutch (DCOG) Trial Terminated NCT02641782 Phase 2 antibody ch14.18;GM-CSF;IL-2 i.v.;IL-2 s.c.;Retinoic acid
4 DT2219ARL for Relapsed or Refractory CD19 (+), CD 22 (+) B-Lineage Leukemia Or Lymphoma Completed NCT00889408 Phase 1
5 CAT-8015 in Children, Adolescents and Young Adults With Acute Lymphoblastic Leukemia or Non-Hodgkin's Lymphoma Completed NCT00659425 Phase 1 CAT-8015 (Moxetumomab Pasudotox);CAT-8015 (Moxetumomab Pasudotox);CAT-8015 (Moxetumomab Pasudotox);CAT-8015 (Moxetumomab Pasudotox);CAT-8015 (Moxetumomab Pasudotox);CAT-8015 (Moxetumomab Pasudotox);CAT-8015 (Moxetumomab Pasudotox);CAT-8015 (Moxetumomab Pasudotox);CAT-8015 (Moxetumomab Pasudotox);CAT-8015 (Moxetumomab Pasudotox)
6 Human Leukocyte Antigen (HLA) Class I Haplotype Mismatched Natural Killer Cell Infusions Completed NCT00660166 Phase 1
7 Bioelectrical Impedance Analysis as a Bedside Tool to Estimate Volume of Distribution of Hydrophilic Antimicrobials in Critically Ill Patients Completed NCT03410771 Monitor antibiotic exposure (PK profile)
8 Evaluation of Endothelial Dysfunction During Aortic Valvular Replacement by Bioprosthesis With and Without Extracorporeal Circulation Completed NCT03135496
9 Donor Lymphocyte Infusion (DLI) for Relapsed (Post Transplant) Leukemia Completed NCT00167167
10 Studies in the Pathogenesis of Systemic Capillary Leak Syndrome Recruiting NCT00936325
11 Characterization of Endothelial Dysfunction as a Function of Hyperaemia of the Brachial Artery During Cardiac Surgery Recruiting NCT02853682
12 Evaluation of the Association Between Pre-existing Endothelial Dysfunction and the Onset of Vasoplegia During Cardiac Surgery With Cardiopulmonary Bypass Not yet recruiting NCT02965339

Search NIH Clinical Center for Systemic Capillary Leak Syndrome

Genetic Tests for Systemic Capillary Leak Syndrome

Anatomical Context for Systemic Capillary Leak Syndrome

MalaCards organs/tissues related to Systemic Capillary Leak Syndrome:

38
Kidney, Heart, Liver, Endothelial, Bone, Skeletal Muscle, B Cells

Publications for Systemic Capillary Leak Syndrome

Articles related to Systemic Capillary Leak Syndrome:

(show top 50) (show all 145)
# Title Authors Year
1
Adrenomedullin surges are linked to acute episodes of the systemic capillary leak syndrome (Clarkson disease). ( 29360169 )
2018
2
Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson's Disease). ( 29449526 )
2018
3
Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome. ( 28602874 )
2017
4
Life-threatening systemic capillary leak syndrome in juvenile dermatomyositis. ( 28957546 )
2017
5
Gemcitabine-induced chronic systemic capillary leak syndrome. ( 28855215 )
2017
6
Mimicker of hereditary angioedema: Idiopathic systemic capillary leak syndrome successfully treated with intravenous immunoglobulin. ( 28377172 )
2017
7
Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review. ( 28939140 )
2017
8
How relevant are vascular endothelial growth factor and intercellular adhesion molecule in the systemic capillary leak syndrome of psoriasis? ( 29364440 )
2017
9
The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission. ( 28622216 )
2017
10
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: comment. ( 28122158 )
2017
11
A p190BRhoGAP mutation and prolonged RhoB activation in fatal systemic capillary leak syndrome. ( 29097442 )
2017
12
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: reply. ( 28182327 )
2017
13
Severe cerebral involvement due to idiopathic systemic capillary leak syndrome. ( 27099745 )
2016
14
Idiopathic Systemic Capillary Leak Syndrome: A Case Report. ( 27195144 )
2016
15
Idiopathic systemic capillary leak syndrome (Clarkson disease). ( 28012935 )
2016
16
Subcutaneous Immunoglobulin Therapy Prevents Systemic Capillary Leak Syndrome Attack. ( 27059380 )
2016
17
Systemic capillary leak syndrome and autoimmune diseases: A case series. ( 27637319 )
2016
18
Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome. ( 27688917 )
2016
19
A Case of Refractory Systemic Capillary Leak Syndrome (Clarkson's Disease) during Pregnancy. ( 28003676 )
2016
20
A Systemic Capillary Leak Syndrome (Clarkson Syndrome) in a Patient with Chronic Lymphocytic Leukemia: A Case Report in an Out-of-Hospital Setting. ( 27069700 )
2016
21
Sudden cardiac arrest in a 73-year-old woman caused by systemic capillary leak syndrome. ( 27056382 )
2016
22
Microangiopathic Hemolytic Anemia Due to ADAMTS-13 Loss in Idiopathic Systemic Capillary Leak Syndrome. ( 27622772 )
2016
23
Endothelial Expression of Endothelin Receptor A in the Systemic Capillary Leak Syndrome. ( 26176954 )
2015
24
Influenza A virus infection is associated with systemic capillary leak syndrome: case report and systematic review of the literature. ( 26330157 )
2015
25
Systemic capillary leak syndrome in a patient receiving adjuvant oxaliplatin for locally advanced colon cancer. ( 26071595 )
2015
26
A fatal case of acute progression of generalized edema and simultaneous flash pulmonary edema in a patient with idiopathic systemic capillary leak syndrome: a case report. ( 25928289 )
2015
27
Clinical and pathological findings of a fatal systemic capillary leak syndrome (Clarkson disease): a case report. ( 25738482 )
2015
28
1222: SUCCESSFUL MANAGEMENT OF RECURRENT IDIOPATHIC SYSTEMIC CAPILLARY LEAK SYNDROME IN A 4-YEAR OLD GIRL. ( 26570883 )
2015
29
97: PEDIATRIC SYSTEMIC CAPILLARY LEAK SYNDROME ENDOTHELIAL CELLS ARE HYPERSENSITIVE TO TNF-I9 AND LPS. ( 26569758 )
2015
30
Spinal Anesthesia in a Patient With a History of Systemic Capillary Leak Syndrome (Clarkson Disease). ( 26237002 )
2015
31
Recurrent undifferentiated shock: Idiopathic Systemic Capillary Leak Syndrome. ( 26273434 )
2015
32
Systemic Capillary Leak Syndrome after Influenza Vaccination in a Peritoneal Dialysis Patient. ( 26703855 )
2015
33
Systemic capillary leak syndrome: a case-report. ( 26117513 )
2015
34
Multiglandular hormone deficiency in a patient with systemic capillary leak syndrome. ( 25685157 )
2015
35
1197: ACUTE THERAPY WITH IVIG IN A PEDIATRIC PATIENT WITH IDIOPATHIC SYSTEMIC CAPILLARY LEAK SYNDROME. ( 26570858 )
2015
36
A case report of idiopathic systemic capillary leak syndrome that occurred during the postoperative period of abdominoperineal resection for colorectal cancer. ( 25594640 )
2015
37
Increased myocardial extracellular volume in active idiopathic systemic capillary leak syndrome. ( 26310790 )
2015
38
Correction: Endothelial Expression of Endothelin Receptor A in the Systemic Capillary Leak Syndrome. ( 26325591 )
2015
39
An uncommon cause of distributive shock: Lessons from two consecutive cases of idiopathic systemic capillary leak syndrome (Clarkson's disease). ( 26300309 )
2015
40
Idiopathic systemic capillary leak syndrome in children. ( 25713284 )
2015
41
Invasive pulmonary aspergillosis in a patient presenting with idiopathic systemic capillary leak syndrome. ( 24859554 )
2014
42
Systemic capillary leak syndrome under general anesthesia: a case report. ( 25006371 )
2014
43
Clarkson disease - systemic capillary leak syndrome in a 6-year-old girl: case report. ( 25311880 )
2014
44
Systemic capillary leak syndrome in an 85-year-old man (Clarkson's syndrome). ( 25287734 )
2014
45
Systemic capillary leak syndrome associated with a rare abdominal and four-limb compartment syndrome: a case report. ( 24934689 )
2014
46
Fulminant systemic capillary leak syndrome due to C1 inhibitor deficiency complicating acute dermatomyositis: a case report. ( 24467750 )
2014
47
High dose intravenous immunoglobulin therapy of the Systemic Capillary Leak Syndrome (Clarkson disease). ( 25193271 )
2014
48
Mechanistic classification of the systemic capillary leak syndrome: Clarkson disease. ( 24787070 )
2014
49
Two Episodes of Systemic Capillary Leak Syndrome in an 8-year-old Boy, Following Influenza A Virus Infection. ( 23995592 )
2014
50
Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease). ( 25405070 )
2014

Variations for Systemic Capillary Leak Syndrome

Expression for Systemic Capillary Leak Syndrome

Search GEO for disease gene expression data for Systemic Capillary Leak Syndrome.

Pathways for Systemic Capillary Leak Syndrome

GO Terms for Systemic Capillary Leak Syndrome

Sources for Systemic Capillary Leak Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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