MCID: SYS007
MIFTS: 29

Systemic Capillary Leak Syndrome

Categories: Rare diseases, Bone diseases, Cardiovascular diseases

Aliases & Classifications for Systemic Capillary Leak Syndrome

MalaCards integrated aliases for Systemic Capillary Leak Syndrome:

Name: Systemic Capillary Leak Syndrome 50 56
Capillary Leak Syndrome 50 56 69
Capillary Leak Syndrome with Monoclonal Gammopathy 50 69
Clarkson Disease 50 56
Scls 50 56
Periodic Systemic Capillary Leak Syndrome 50
Capillary Hyperpermeability Syndrome 56
Idiopathic Capillary Leak Syndrome 56

Characteristics:

Orphanet epidemiological data:

56
systemic capillary leak syndrome
Inheritance: Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: Adult; Age of death: adult;

Classifications:



External Ids:

Orphanet 56 ORPHA188
MESH via Orphanet 43 D019559
UMLS via Orphanet 70 C0343084
ICD10 via Orphanet 34 I78.8

Summaries for Systemic Capillary Leak Syndrome

NIH Rare Diseases : 50 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 capillary leak syndrome and superior limbic keratoconjunctivitis. An important gene associated with Systemic Capillary Leak Syndrome is EDNRA (Endothelin Receptor Type A). The drugs Aldesleukin and Isotretinoin 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 29)
id Related Disease Score Top Affiliating Genes
1 capillary leak syndrome 10.8
2 superior limbic keratoconjunctivitis 10.8
3 compartment syndrome 10.3
4 endotheliitis 10.2
5 influenza 10.0
6 hemolytic anemia 10.0
7 cerebritis 9.9
8 lymphoma 9.9
9 colorectal cancer 9.8
10 dermatomyositis 9.8
11 breast cancer 9.8
12 pulmonary edema 9.8
13 renal cell carcinoma 9.8
14 multiple myeloma 9.8
15 hereditary angioedema 9.8
16 aspergillosis 9.8
17 angioedema 9.8
18 kawasaki disease 9.8
19 adult respiratory distress syndrome 9.8
20 anaplastic large cell lymphoma 9.8
21 peritonitis 9.8
22 b-cell lymphomas 9.8
23 polycythemia 9.8
24 chronic lymphocytic leukemia 9.8
25 diffuse large b-cell lymphoma 9.8
26 leukemia 9.8
27 alk-negative anaplastic large cell lymphoma 9.8
28 c1 inhibitor deficiency 9.8
29 cardiac arrest 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 58)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Aldesleukin Approved Phase 3,Phase 2 85898-30-2, 110942-02-4
2
Isotretinoin Approved Phase 3,Phase 2 4759-48-2 5538 5282379
3
Acetaminophen Approved Phase 3 103-90-2 1983
4
Codeine Approved, Illicit Phase 3 76-57-3 5284371
5
Dopamine Approved Phase 3 51-61-6, 62-31-7 681
6
Guaifenesin Approved, Vet_approved Phase 3 93-14-1 3516
7
Ibuprofen Approved Phase 3 15687-27-1 3672
8
Metoclopramide Approved, Investigational Phase 3 364-62-5 4168
9
Ondansetron Approved Phase 3 99614-02-5 4595
10
Tretinoin Approved, Investigational, Nutraceutical Phase 3,Phase 2 302-79-4 5538
11
Vitamin A Approved, Nutraceutical, Vet_approved Phase 3 11103-57-4, 68-26-8 445354
12 Analgesics Phase 3,Phase 2
13 Analgesics, Non-Narcotic Phase 3,Phase 2
14 Antibodies Phase 3,Phase 2
15 Antibodies, Monoclonal Phase 3
16 Anti-HIV Agents Phase 3,Phase 2
17 Anti-Infective Agents Phase 3,Phase 2
18 Anti-Retroviral Agents Phase 3,Phase 2
19 Antiviral Agents Phase 3,Phase 2
20 Dermatologic 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 Peripheral Nervous System Agents Phase 3,Phase 2
25 Retinol palmitate Phase 3
26 Vitamins Phase 3
27 Analgesics, Opioid Phase 3
28 Anti-Anxiety Agents Phase 3
29 Antiemetics Phase 3
30 Anti-Inflammatory Agents Phase 3
31 Anti-Inflammatory Agents, Non-Steroidal Phase 3
32 Antipruritics Phase 3
33 Antipsychotic Agents Phase 3
34 Antipyretics Phase 3
35 Antirheumatic Agents Phase 3
36 Antitussive Agents Phase 3
37 Autonomic Agents Phase 3
38 Central Nervous System Depressants Phase 3
39 Chlorpheniramine, phenylpropanolamine drug combination Phase 3
40 Cyclooxygenase Inhibitors Phase 3
41 Dopamine Agents Phase 3
42 Dopamine Antagonists Phase 3
43 Dopamine D2 Receptor Antagonists Phase 3
44 Gastrointestinal Agents Phase 3
45 Narcotics Phase 3
46 Neurotransmitter Agents Phase 3
47 Psychotropic Drugs Phase 3
48 Respiratory System Agents Phase 3
49
Serotonin Phase 3 50-67-9 5202
50 Serotonin Agents Phase 3

Interventional clinical trials:

(show all 11)

id 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 Evaluation of Endothelial Dysfunction During Aortic Valvular Replacement by Bioprosthesis With and Without Extracorporeal Circulation Completed NCT03135496
8 Donor Lymphocyte Infusion (DLI) for Relapsed (Post Transplant) Leukemia Completed NCT00167167
9 Studies in the Pathogenesis of Systemic Capillary Leak Syndrome Recruiting NCT00936325
10 Characterization of Endothelial Dysfunction as a Function of Hyperaemia of the Brachial Artery During Cardiac Surgery Recruiting NCT02853682
11 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:

39
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 141)
id Title Authors Year
1
Mimicker of hereditary angioedema: Idiopathic systemic capillary leak syndrome successfully treated with intravenous immunoglobulin. ( 28377172 )
2017
2
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: comment. ( 28122158 )
2017
3
Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome. ( 28602874 )
2017
4
The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission. ( 28622216 )
2017
5
Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review. ( 28939140 )
2017
6
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: reply. ( 28182327 )
2017
7
Gemcitabine-induced chronic systemic capillary leak syndrome. ( 28855215 )
2017
8
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
9
Systemic capillary leak syndrome and autoimmune diseases: A case series. ( 27637319 )
2016
10
Isoform D of vascular endothelial growth factor in systemic capillary leak syndrome: a case report. ( 27386947 )
2016
11
Idiopathic systemic capillary leak syndrome (Clarkson disease). ( 28012935 )
2016
12
Severe cerebral involvement due to idiopathic systemic capillary leak syndrome. ( 27099745 )
2016
13
A Case of Refractory Systemic Capillary Leak Syndrome (Clarkson's Disease) during Pregnancy. ( 28003676 )
2016
14
Idiopathic Systemic Capillary Leak Syndrome: A Case Report. ( 27195144 )
2016
15
Subcutaneous Immunoglobulin Therapy Prevents Systemic Capillary Leak Syndrome Attack. ( 27059380 )
2016
16
Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome. ( 27688917 )
2016
17
Sudden cardiac arrest in a 73-year-old woman caused by systemic capillary leak syndrome. ( 27056382 )
2016
18
Microangiopathic Hemolytic Anemia Due to ADAMTS-13 Loss in Idiopathic Systemic Capillary Leak Syndrome. ( 27622772 )
2016
19
1197: ACUTE THERAPY WITH IVIG IN A PEDIATRIC PATIENT WITH IDIOPATHIC SYSTEMIC CAPILLARY LEAK SYNDROME. ( 26570858 )
2015
20
97: PEDIATRIC SYSTEMIC CAPILLARY LEAK SYNDROME ENDOTHELIAL CELLS ARE HYPERSENSITIVE TO TNF-I9 AND LPS. ( 26569758 )
2015
21
Influenza A virus infection is associated with systemic capillary leak syndrome: case report and systematic review of the literature. ( 26330157 )
2015
22
An uncommon cause of distributive shock: Lessons from two consecutive cases of idiopathic systemic capillary leak syndrome (Clarkson's disease). ( 26300309 )
2015
23
Idiopathic systemic capillary leak syndrome in children. ( 25713284 )
2015
24
Increased myocardial extracellular volume in active idiopathic systemic capillary leak syndrome. ( 26310790 )
2015
25
1222: SUCCESSFUL MANAGEMENT OF RECURRENT IDIOPATHIC SYSTEMIC CAPILLARY LEAK SYNDROME IN A 4-YEAR OLD GIRL. ( 26570883 )
2015
26
Endothelial Expression of Endothelin Receptor A in the Systemic Capillary Leak Syndrome. ( 26176954 )
2015
27
Multiglandular hormone deficiency in a patient with systemic capillary leak syndrome. ( 25685157 )
2015
28
Correction: Endothelial Expression of Endothelin Receptor A in the Systemic Capillary Leak Syndrome. ( 26325591 )
2015
29
Systemic capillary leak syndrome in a patient receiving adjuvant oxaliplatin for locally advanced colon cancer. ( 26071595 )
2015
30
Recurrent undifferentiated shock: Idiopathic Systemic Capillary Leak Syndrome. ( 26273434 )
2015
31
A case report of idiopathic systemic capillary leak syndrome that occurred during the postoperative period of abdominoperineal resection for colorectal cancer. ( 25594640 )
2015
32
Systemic capillary leak syndrome: a case-report. ( 26117513 )
2015
33
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
34
Clinical and pathological findings of a fatal systemic capillary leak syndrome (Clarkson disease): a case report. ( 25738482 )
2015
35
Systemic Capillary Leak Syndrome after Influenza Vaccination in a Peritoneal Dialysis Patient. ( 26703855 )
2015
36
Spinal Anesthesia in a Patient With a History of Systemic Capillary Leak Syndrome (Clarkson Disease). ( 26237002 )
2015
37
Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature. ( 24490827 )
2014
38
Inflammatory Markers of the Systemic Capillary Leak Syndrome (Clarkson Disease). ( 25405070 )
2014
39
Systemic capillary leak syndrome in an 85-year-old man (Clarkson's syndrome). ( 25287734 )
2014
40
Systemic capillary leak syndrome revealing a diffuse large B-cell lymphoma. ( 24942976 )
2014
41
Two Episodes of Systemic Capillary Leak Syndrome in an 8-year-old Boy, Following Influenza A Virus Infection. ( 23995592 )
2014
42
Systemic capillary leak syndrome under general anesthesia: a case report. ( 25006371 )
2014
43
10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report. ( 24885961 )
2014
44
Fulminant systemic capillary leak syndrome due to C1 inhibitor deficiency complicating acute dermatomyositis: a case report. ( 24467750 )
2014
45
Systemic capillary leak syndrome associated with a rare abdominal and four-limb compartment syndrome: a case report. ( 24934689 )
2014
46
Systemic capillary leak syndrome: is methylene blue the silver bullet? ( 25544902 )
2014
47
Invasive pulmonary aspergillosis in a patient presenting with idiopathic systemic capillary leak syndrome. ( 24859554 )
2014
48
Mechanistic classification of the systemic capillary leak syndrome: Clarkson disease. ( 24787070 )
2014
49
Clarkson disease - systemic capillary leak syndrome in a 6-year-old girl: case report. ( 25311880 )
2014
50
High dose intravenous immunoglobulin therapy of the Systemic Capillary Leak Syndrome (Clarkson disease). ( 25193271 )
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

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67 TGDB
68 Tocris
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70 UMLS via Orphanet
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