SCLS
MCID: SYS007
MIFTS: 42

Systemic Capillary Leak Syndrome (SCLS)

Categories: Bone diseases, Cardiovascular diseases, Immune diseases, Rare diseases

Aliases & Classifications for Systemic Capillary Leak Syndrome

MalaCards integrated aliases for Systemic Capillary Leak Syndrome:

Name: Systemic Capillary Leak Syndrome 53 59
Capillary Leak Syndrome 53 59 73
Capillary Leak Syndrome with Monoclonal Gammopathy 53 73
Clarkson Disease 53 59
Scls 53 59
Periodic Systemic Capillary Leak Syndrome 53
Capillary Hyperpermeability Syndrome 59
Idiopathic Capillary Leak Syndrome 59

Characteristics:

Orphanet epidemiological data:

59
systemic capillary leak syndrome
Inheritance: Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: Adult; Age of death: adult;

Classifications:



Summaries for Systemic Capillary Leak Syndrome

NIH Rare Diseases : 53 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.

MalaCards based summary : Systemic Capillary Leak Syndrome, also known as capillary leak syndrome, is related to dengue disease and superior limbic keratoconjunctivitis. An important gene associated with Systemic Capillary Leak Syndrome is EDNRA (Endothelin Receptor Type A). The drugs Amoxicillin and Anti-Bacterial Agents have been mentioned in the context of this disorder. Affiliated tissues include liver, endothelial and kidney, and related phenotypes are hypotension and renal insufficiency

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 top 50) (show all 91)
# Related Disease Score Top Affiliating Genes
1 dengue disease 11.2
2 superior limbic keratoconjunctivitis 11.1
3 capillary leak syndrome 11.0
4 compartment syndrome 10.5
5 influenza 10.2
6 pelger-huet anomaly 10.2
7 acute t cell leukemia 10.2
8 hemolytic anemia 10.2
9 dermatomyositis 10.2
10 pustular psoriasis 10.1
11 myositis 10.1
12 childhood type dermatomyositis 10.1
13 lymphoma 10.1
14 viral infectious disease 10.1
15 scleroderma, familial progressive 10.0
16 psoriasis 14, pustular 10.0
17 thrombocytopenia 10.0
18 cutaneous t cell lymphoma 10.0
19 brucellosis 10.0
20 encephalopathy 10.0
21 interstitial lung disease 10.0
22 lung disease 10.0
23 autoimmune disease 9.9
24 leukemia, chronic lymphocytic 2 9.9
25 breast cancer 9.9
26 colorectal cancer 9.9
27 complement component 4, partial deficiency of 9.9
28 renal cell carcinoma, nonpapillary 9.9
29 leukemia, chronic lymphocytic 9.9
30 myeloma, multiple 9.9
31 polycythemia vera 9.9
32 sjogren syndrome 9.9
33 stroke, ischemic 9.9
34 autoimmune disease 1 9.9
35 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.9
36 kawasaki disease 9.9
37 aspergillosis 9.9
38 adult respiratory distress syndrome 9.9
39 anaplastic large cell lymphoma 9.9
40 b-cell lymphomas 9.9
41 diffuse large b-cell lymphoma 9.9
42 leukemia 9.9
43 c1 inhibitor deficiency 9.9
44 cardiac arrest 9.9
45 lymphocytic leukemia 9.9
46 pulmonary edema 9.9
47 hereditary angioedema 9.9
48 angioedema 9.9
49 agammaglobulinemia 9.9
50 polycythemia 9.9

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

Human phenotypes related to Systemic Capillary Leak Syndrome:

59 32 (show all 23)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hypotension 59 32 frequent (33%) Frequent (79-30%) HP:0002615
2 renal insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0000083
3 fatigue 59 32 frequent (33%) Frequent (79-30%) HP:0012378
4 arrhythmia 59 32 occasional (7.5%) Occasional (29-5%) HP:0011675
5 abdominal pain 59 32 frequent (33%) Frequent (79-30%) HP:0002027
6 weight loss 59 32 frequent (33%) Frequent (79-30%) HP:0001824
7 venous thrombosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0004936
8 abnormality of the renal tubule 59 32 occasional (7.5%) Occasional (29-5%) HP:0000091
9 pancreatitis 59 32 frequent (33%) Frequent (79-30%) HP:0001733
10 myalgia 59 32 frequent (33%) Frequent (79-30%) HP:0003326
11 leukocytosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0001974
12 diarrhea 59 32 frequent (33%) Frequent (79-30%) HP:0002014
13 pericarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001701
14 myocarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012819
15 cough 59 32 occasional (7.5%) Occasional (29-5%) HP:0012735
16 edema of the lower limbs 59 32 hallmark (90%) Very frequent (99-80%) HP:0010741
17 pleural effusion 59 32 occasional (7.5%) Occasional (29-5%) HP:0002202
18 oliguria 59 32 occasional (7.5%) Occasional (29-5%) HP:0100520
19 rhinorrhea 59 32 frequent (33%) Frequent (79-30%) HP:0031417
20 cardiorespiratory arrest 59 32 occasional (7.5%) Occasional (29-5%) HP:0006543
21 multiple myeloma 59 32 occasional (7.5%) Occasional (29-5%) HP:0006775
22 pulmonary edema 59 32 frequent (33%) Frequent (79-30%) HP:0100598
23 constitutional symptom 59 Frequent (79-30%)

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 91)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Amoxicillin Approved, Vet_approved Phase 4 26787-78-0 33613
2 Anti-Bacterial Agents Phase 4,Phase 2
3 Anti-Infective Agents Phase 4,Phase 3,Phase 2
4 Antibiotics, Antitubercular Phase 4,Phase 2
5 Clavulanic Acids Phase 4
6 Antitubercular Agents Phase 4,Phase 2
7 Amoxicillin-Potassium Clavulanate Combination Phase 4
8 beta-Lactamase Inhibitors Phase 4
9
Aldesleukin Approved Phase 3,Phase 2,Phase 1 85898-30-2, 110942-02-4
10
Isotretinoin Approved Phase 3,Phase 2 4759-48-2 5538 5282379
11
Guaifenesin Approved, Investigational, Vet_approved Phase 3 93-14-1 3516
12
Codeine Approved, Illicit Phase 3 76-57-3 5284371
13
Ondansetron Approved Phase 3 99614-02-5 4595
14
Acetaminophen Approved Phase 3 103-90-2 1983
15
Dopamine Approved Phase 3 51-61-6, 62-31-7 681
16
Ibuprofen Approved Phase 3 15687-27-1 3672
17
Metoclopramide Approved, Investigational Phase 3 364-62-5 4168
18
Vitamin A Approved, Nutraceutical, Vet_approved Phase 3 11103-57-4, 68-26-8 445354
19
Tretinoin Approved, Investigational, Nutraceutical Phase 3,Phase 2 302-79-4 5538 444795
20
Serine Approved, Nutraceutical Phase 3 56-45-1 5951
21 Analgesics Phase 3,Phase 2
22 Analgesics, Non-Narcotic Phase 3,Phase 2
23 Antibodies, Monoclonal Phase 3,Phase 1
24 Anti-HIV Agents Phase 3,Phase 2
25 Keratolytic Agents Phase 3,Phase 2
26 Vitamins Phase 3
27 Retinol palmitate Phase 3
28 Anti-Retroviral Agents Phase 3,Phase 2
29 retinol Phase 3
30 Antiviral Agents Phase 3,Phase 2
31 Peripheral Nervous System Agents Phase 3,Phase 2
32 Antibodies Phase 3,Phase 2,Phase 1
33 Immunoglobulins Phase 3,Phase 2,Phase 1
34 Interleukin-2 Phase 3,Phase 2,Phase 1
35 Immunologic Factors Phase 3,Phase 2,Phase 1
36 Dermatologic Agents Phase 3,Phase 2
37 Psychotropic Drugs Phase 3
38 Anti-Inflammatory Agents Phase 3
39 Dopamine D2 Receptor Antagonists Phase 3
40 Antirheumatic Agents Phase 3,Phase 2
41 Central Nervous System Depressants Phase 3
42 Serotonin Agents Phase 3
43 Autonomic Agents Phase 3
44 Tranquilizing Agents Phase 3
45 Dopamine Agents Phase 3
46 Chlorpheniramine, phenylpropanolamine drug combination Phase 3
47 Dopamine Antagonists Phase 3
48 Serotonin Antagonists Phase 3
49 Gastrointestinal Agents Phase 3
50 Cyclooxygenase Inhibitors Phase 3

Interventional clinical trials:

(show all 21)
# Name Status NCT ID Phase Drugs
1 Routine Antibiotic vs. Directed Antibiotic Treatment in Snake Bite Recruiting NCT02570347 Phase 4 Co-amoxiclav
2 Monoclonal Antibody Ch14.18, Sargramostim, Aldesleukin, and Isotretinoin After Autologous Stem Cell Transplant in Treating Patients With Neuroblastoma Completed NCT01041638 Phase 3 Isotretinoin
3 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
4 Safety Study of CAT-8015 to Treat Advanced B-cell Non-Hodgkin Lymphoma and Chronic Lymphocytic Leukemia (NHL or CLL) Completed NCT01030536 Phase 1, Phase 2 CAT-8015 20 mcg/kg;CAT-8015 30 mcg/kg;CAT-8015 40 mcg/kg;CAT-8015 50 mcg/kg;CAT-8015 60 mcg/kg
5 A Study Of The Safety And Effects Of One Or More Doses Of HSP-130 Injected Under The Skin In Women With Breast Cancer That Has Not Spread To Distant Sites In The Body. Completed NCT02650193 Phase 2 HSP-130
6 Mesenchymal Stromal Cells in Living Donor Kidney Transplantation Recruiting NCT03478215 Phase 2
7 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
8 Moxetumomab Pasudotox (CAT-8015, HA22) in Children With B-lineage Acute Lymphoblastic Leukemia and Minimal Residual Disease Prior to Allogeneic Hematopoietic Stem Cell Transplantation Terminated NCT02338050 Phase 2
9 CD3/CD19 Depleted or CD3 Depleted/CD56 Selected Haploid Donor Natural Killer Cell Treatment in Older AML in First Complete Remission Withdrawn NCT01639456 Phase 2 Cyclophosphamide;Fludarabine;Aldesleukin
10 DT2219ARL for Relapsed or Refractory CD19 (+), CD 22 (+) B-Lineage Leukemia Or Lymphoma Completed NCT00889408 Phase 1
11 Combination Chemotherapy, Monoclonal Antibody, and Natural Killer Cells in Treating Young Patients With Recurrent or Refractory Neuroblastoma Completed NCT01576692 Phase 1 Chemotherapy
12 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)
13 Human Leukocyte Antigen (HLA) Class I Haplotype Mismatched Natural Killer Cell Infusions Completed NCT00660166 Phase 1
14 Monitoring the IMmUological TOXicity of Drugs Completed NCT03480529 drug inducing arthritis, lupus or capillary leak syndrom
15 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)
16 Donor Lymphocyte Infusion (DLI) for Relapsed (Post Transplant) Leukemia Completed NCT00167167 Not Applicable
17 Studies in the Pathogenesis of Systemic Capillary Leak Syndrome Recruiting NCT00936325
18 Evaluation of Endothelial Dysfunction During Aortic Valvular Replacement by Bioprosthesis With and Without Extracorporeal Circulation Recruiting NCT03135496
19 Evaluation of the Association Between Pre-existing Endothelial Dysfunction and the Onset of Vasoplegia During Cardiac Surgery With Cardiopulmonary Bypass Recruiting NCT02965339
20 Observational Study of Blinatumomab Recruiting NCT03117621
21 Characterization of Endothelial Dysfunction as a Function of Hyperaemia of the Brachial Artery During Cardiac Surgery Active, not recruiting NCT02853682

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:

41
Liver, Endothelial, Kidney, Heart, Bone, Lung, T Cells

Publications for Systemic Capillary Leak Syndrome

Articles related to Systemic Capillary Leak Syndrome:

(show top 50) (show all 265)
# Title Authors Year
1
Chronic systemic capillary leak syndrome treatment with intravenous immune globulin: Case report and review of the literature
. ( 30431430 )
2019
2
The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome. ( 29732372 )
2018
3
Capillary Leak Syndrome Aggravated by Influenza Type A Infection. ( 29974010 )
2018
4
Intravenous immunoglobulin therapy for dengue capillary leak syndrome in a renal allograft recipient. ( 29925559 )
2018
5
Recognition and management of idiopathic systemic capillary leak syndrome: an evidence-based review. ( 29564922 )
2018
6
Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review. ( 29936591 )
2018
7
Capillary leak syndrome as a complication of antibody-mediated rejection treatment: a case report. ( 29344912 )
2018
8
Adrenomedullin surges are linked to acute episodes of the systemic capillary leak syndrome (Clarkson disease). ( 29360169 )
2018
9
Idiopathic Systemic Capillary Leak Syndrome. ( 29910550 )
2018
10
A Viral Polymyositis Masquerade: Life-Threatening Case of Juvenile Dermatomyositis Complicated by Systemic Capillary Leak Syndrome. ( 29720802 )
2018
11
Idiopathic systemic capillary leak syndrome presenting as septic shock: A case report. ( 29779703 )
2018
12
Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson's Disease). ( 29449526 )
2018
13
Capillary leak syndrome and aseptic meningitis in a patient with Kawasaki disease: A case report. ( 29879013 )
2018
14
Myocardial dysfunction is frequent in systemic capillary-leak syndrome (Clarkson disease) severe episodes. ( 29477581 )
2018
15
Capillary leak syndrome in Daboia russelii bite-a complication associated with poor outcome. ( 29584906 )
2018
16
Anesthetic Considerations for a Patient With Chronic Systemic Capillary Leak Syndrome: A Case Report. ( 29894349 )
2018
17
Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection. ( 30158273 )
2018
18
Sjögren's syndrome concurrent with protein-losing gastroenteropathy with secondary systemic capillary leak syndrome : A case report. ( 30214773 )
2018
19
Correction: Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection. ( 30287629 )
2018
20
Probable Mechanisms Involved in Immunotoxin Mediated Capillary Leak Syndrome (CLS) and Recently Developed Countering Strategies. ( 30289072 )
2018
21
Whole Exome Sequencing of Adult and Pediatric Cohorts of the Rare Vascular Disorder Systemic Capillary Leak Syndrome. ( 30289850 )
2018
22
Systemic Capillary Leak Syndrome (Clarkson Syndrome) in Cancer Patients: A Systematic Review. ( 30404164 )
2018
23
How relevant are vascular endothelial growth factor and intercellular adhesion molecule in the systemic capillary leak syndrome of psoriasis? ( 29364440 )
2017
24
Capillary Leak Syndrome Following Snakebite Envenomation. ( 29142382 )
2017
25
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: reply. ( 28182327 )
2017
26
Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review. ( 28939140 )
2017
27
A p190BRhoGAP mutation and prolonged RhoB activation in fatal systemic capillary leak syndrome. ( 29097442 )
2017
28
The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission. ( 28622216 )
2017
29
Capillary leak syndrome: etiologies, pathophysiology, and management. ( 28318633 )
2017
30
Life-threatening systemic capillary leak syndrome in juvenile dermatomyositis. ( 28957546 )
2017
31
Capillary leak syndrome in neuromyelitis optica treated with rituximab. ( 28755680 )
2017
32
Mimicker of hereditary angioedema: Idiopathic systemic capillary leak syndrome successfully treated with intravenous immunoglobulin. ( 28377172 )
2017
33
Gemcitabine-induced chronic systemic capillary leak syndrome. ( 28855215 )
2017
34
Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome. ( 28602874 )
2017
35
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: comment. ( 28122158 )
2017
36
Distributive Shock in the Emergency Department: Sepsis, Anaphylaxis, or Capillary Leak Syndrome? ( 28238385 )
2017
37
Epidemiology, risk factors, and prognosis of capillary leak syndrome in pediatric recipients of stem cell transplants: a retrospective single-center cohort study. ( 27734564 )
2016
38
Severe cerebral involvement due to idiopathic systemic capillary leak syndrome. ( 27099745 )
2016
39
Idiopathic systemic capillary leak syndrome (Clarkson disease). ( 28012935 )
2016
40
Subcutaneous Immunoglobulin Therapy Prevents Systemic Capillary Leak Syndrome Attack. ( 27059380 )
2016
41
Fulminant capillary leak syndrome in a patient with systemic sclerosis treated with imatinib mesylate. ( 27338086 )
2016
42
Sudden cardiac arrest in a 73-year-old woman caused by systemic capillary leak syndrome. ( 27056382 )
2016
43
Microangiopathic Hemolytic Anemia Due to ADAMTS-13 Loss in Idiopathic Systemic Capillary Leak Syndrome. ( 27622772 )
2016
44
Isoform D of vascular endothelial growth factor in systemic capillary leak syndrome: a case report. ( 27386947 )
2016
45
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
46
Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome. ( 27688917 )
2016
47
Rhabdomyolysis and capillary leak syndrome in a patient treated with topical imiquimod. ( 27061736 )
2016
48
Scrub typhus presenting as capillary leak syndrome. ( 27728427 )
2016
49
Systemic capillary leak syndrome and autoimmune diseases: A case series. ( 27637319 )
2016
50
Fatal Primary Capillary Leak Syndrome in a Late Preterm Newborn. ( 27142601 )
2016

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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