MCID: SYS007
MIFTS: 45

Systemic Capillary Leak Syndrome

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 54 60 17
Capillary Leak Syndrome 54 60 74
Capillary Leak Syndrome with Monoclonal Gammopathy 54 74
Clarkson Disease 54 60
Scls 54 60
Periodic Systemic Capillary Leak Syndrome 54
Capillary Hyperpermeability Syndrome 60
Idiopathic Capillary Leak Syndrome 60

Characteristics:

Orphanet epidemiological data:

60
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 : 54 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 pulmonary hypertension and dengue disease. An important gene associated with Systemic Capillary Leak Syndrome is ADM (Adrenomedullin). The drugs Amoxicillin and Antibiotics, Antitubercular have been mentioned in the context of this disorder. Affiliated tissues include kidney, t cells and endothelial, and related phenotypes are leukocytosis and pedal edema

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 101)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension 29.3 ADM EDNRA
2 dengue disease 11.2
3 superior limbic keratoconjunctivitis 11.1
4 capillary leak syndrome 11.1
5 compartment syndrome 10.5
6 pelger-huet anomaly 10.3
7 acute t cell leukemia 10.2
8 influenza 10.2
9 hemolytic anemia 10.2
10 dermatomyositis 10.2
11 myositis 10.1
12 systemic scleroderma 10.1
13 t-cell leukemia 10.1
14 pustular psoriasis 10.1
15 deficiency anemia 10.1
16 childhood type dermatomyositis 10.1
17 lymphoma 10.1
18 agammaglobulinemia 10.1
19 systemic lupus erythematosus 10.0
20 hemangioma 10.0
21 interstitial lung disease 10.0
22 lung disease 10.0
23 lupus erythematosus 10.0
24 psoriasis 14, pustular 10.0
25 thrombocytopenia 10.0
26 cutaneous t cell lymphoma 10.0
27 brucellosis 10.0
28 diarrhea 10.0
29 encephalopathy 10.0
30 autoimmune disease 9.9
31 leukemia, chronic lymphocytic 2 9.9
32 breast cancer 9.9
33 colorectal cancer 9.9
34 complement component 4, partial deficiency of 9.9
35 renal cell carcinoma, nonpapillary 9.9
36 leukemia, chronic lymphocytic 9.9
37 scleroderma, familial progressive 9.9
38 myeloma, multiple 9.9
39 polycythemia vera 9.9
40 sjogren syndrome 9.9
41 stroke, ischemic 9.9
42 autoimmune disease 1 9.9
43 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.9
44 kawasaki disease 9.9
45 aspergillosis 9.9
46 adult respiratory distress syndrome 9.9
47 anaplastic large cell lymphoma 9.9
48 diffuse large b-cell lymphoma 9.9
49 leukemia 9.9
50 c1 inhibitor deficiency 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:

60 33 (show all 25)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 leukocytosis 60 33 hallmark (90%) Very frequent (99-80%) HP:0001974
2 pedal edema 33 hallmark (90%) HP:0010741
3 hypotension 60 33 frequent (33%) Frequent (79-30%) HP:0002615
4 fatigue 60 33 frequent (33%) Frequent (79-30%) HP:0012378
5 abdominal pain 60 33 frequent (33%) Frequent (79-30%) HP:0002027
6 weight loss 60 33 frequent (33%) Frequent (79-30%) HP:0001824
7 pancreatitis 60 33 frequent (33%) Frequent (79-30%) HP:0001733
8 myalgia 60 33 frequent (33%) Frequent (79-30%) HP:0003326
9 diarrhea 60 33 frequent (33%) Frequent (79-30%) HP:0002014
10 rhinorrhea 60 33 frequent (33%) Frequent (79-30%) HP:0031417
11 pulmonary edema 60 33 frequent (33%) Frequent (79-30%) HP:0100598
12 renal insufficiency 60 33 occasional (7.5%) Occasional (29-5%) HP:0000083
13 arrhythmia 60 33 occasional (7.5%) Occasional (29-5%) HP:0011675
14 venous thrombosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0004936
15 pericarditis 60 33 occasional (7.5%) Occasional (29-5%) HP:0001701
16 myocarditis 60 33 occasional (7.5%) Occasional (29-5%) HP:0012819
17 cough 60 33 occasional (7.5%) Occasional (29-5%) HP:0012735
18 pleural effusion 60 33 occasional (7.5%) Occasional (29-5%) HP:0002202
19 oliguria 60 33 occasional (7.5%) Occasional (29-5%) HP:0100520
20 cardiorespiratory arrest 60 33 occasional (7.5%) Occasional (29-5%) HP:0006543
21 multiple myeloma 60 33 occasional (7.5%) Occasional (29-5%) HP:0006775
22 abnormal renal tubule morphology 33 occasional (7.5%) HP:0000091
23 abnormality of the renal tubule 60 Occasional (29-5%)
24 edema of the lower limbs 60 Very frequent (99-80%)
25 constitutional symptom 60 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 95)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Amoxicillin Approved, Vet_approved Phase 4 26787-78-0 33613
2 Antibiotics, Antitubercular Phase 4,Phase 2
3 Antitubercular Agents Phase 4,Phase 2
4 Anti-Infective Agents Phase 4,Phase 3,Phase 2
5 Anti-Bacterial Agents Phase 4,Phase 2
6 Clavulanic Acids Phase 4
7 beta-Lactamase Inhibitors Phase 4
8 Amoxicillin-Potassium Clavulanate Combination Phase 4
9
Isotretinoin Approved Phase 3,Phase 2 4759-48-2 5282379 5538
10
Sargramostim Approved, Investigational Phase 3,Phase 2,Phase 1 123774-72-1, 83869-56-1
11
Aldesleukin Approved Phase 3,Phase 2,Phase 1 110942-02-4, 85898-30-2
12
Dopamine Approved Phase 3 62-31-7, 51-61-6 681
13
Ibuprofen Approved Phase 3 15687-27-1 3672
14
Guaifenesin Approved, Investigational, Vet_approved Phase 3 93-14-1 3516
15
Acetaminophen Approved Phase 3 103-90-2 1983
16
Codeine Approved, Illicit Phase 3 76-57-3 5284371
17
Metoclopramide Approved, Investigational Phase 3 364-62-5 4168
18
Ondansetron Approved Phase 3 99614-02-5 4595
19
Tretinoin Approved, Investigational, Nutraceutical Phase 3,Phase 2 302-79-4 444795 5538
20
Serine Approved, Nutraceutical Phase 3 56-45-1 5951
21
Vitamin A Approved, Nutraceutical, Vet_approved Phase 3 68-26-8, 22737-96-8, 11103-57-4 9904001 445354
22 Retinol palmitate Phase 3
23 Analgesics, Non-Narcotic Phase 3,Phase 2
24 Immunoglobulins Phase 3,Phase 2,Phase 1
25 Dermatologic Agents Phase 3,Phase 2
26 Anti-Retroviral Agents Phase 3,Phase 2
27 Analgesics Phase 3,Phase 2
28 Peripheral Nervous System Agents Phase 3,Phase 2
29 Anti-HIV Agents Phase 3,Phase 2
30 Immunologic Factors Phase 3,Phase 2,Phase 1
31 Antibodies, Monoclonal Phase 3,Phase 2,Phase 1
32 Antiviral Agents Phase 3,Phase 2
33 Vitamins Phase 3
34 Antineoplastic Agents, Immunological Phase 3
35 retinol Phase 3
36 Keratolytic Agents Phase 3,Phase 2
37 Interleukin-2 Phase 3,Phase 2,Phase 1
38 Ch14.18 monoclonal antibody Phase 3,Phase 2
39 Antibodies Phase 3,Phase 2,Phase 1
40 Tranquilizing Agents Phase 3
41 Dopamine Antagonists Phase 3
42 Autonomic Agents Phase 3
43 Antipyretics Phase 3
44 Central Nervous System Depressants Phase 3
45 Chlorpheniramine, phenylpropanolamine drug combination Phase 3
46 Antiemetics Phase 3
47 Serotonin Antagonists Phase 3
48 Antirheumatic Agents Phase 3,Phase 2
49 Serotonin Agents Phase 3
50 Dopamine D2 Receptor Antagonists 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, hepatitis, 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:

42
Kidney, T Cells, Endothelial, B Cells, Skin, Bone Marrow, Brain

Publications for Systemic Capillary Leak Syndrome

Articles related to Systemic Capillary Leak Syndrome:

(show top 50) (show all 273)
# Title Authors Year
1
Chronic systemic capillary leak syndrome treatment with intravenous immune globulin: Case report and review of the literature
. ( 30431430 )
2019
2
Clinical communication: systemic capillary leak syndrome due to mast cell activation? ( 30639435 )
2019
3
Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome. ( 30665393 )
2019
4
Incidence of Capillary Leak Syndrome as an Adverse Effect of Drugs in Cancer Patients: A Systematic Review and Meta-Analysis. ( 30691103 )
2019
5
First experience with Tolvaptan for the treatment of neonates and infants with capillary leak syndrome after cardiac surgery. ( 30755181 )
2019
6
Chemotherapy-induced skin toxicity and capillary leak syndrome. ( 30847892 )
2019
7
Capillary leak-syndrome triggered by Maripa virus in French Guiana: case report and implication for pathogenesis. ( 30876401 )
2019
8
Successful Treatment of Sjögren's Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and Glucocorticoid. ( 30956836 )
2019
9
Colchicine: An Impressive Effect on Posttransplant Capillary Leak Syndrome and Renal Failure. ( 31000685 )
2019
10
Capillary leak syndrome: a cytokine and catecholamine storm? ( 31010471 )
2019
11
Systemic capillary leak syndrome following bosentan treatment in a patient with systemic sclerosis. ( 31012372 )
2019
12
Capillary leak syndrome and aseptic meningitis in a patient with Kawasaki disease: A case report. ( 29879013 )
2018
13
Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review. ( 28939140 )
2018
14
Capillary leak syndrome as a complication of antibody-mediated rejection treatment: a case report. ( 29344912 )
2018
15
Adrenomedullin surges are linked to acute episodes of the systemic capillary leak syndrome (Clarkson disease). ( 29360169 )
2018
16
Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson's Disease). ( 29449526 )
2018
17
Myocardial dysfunction is frequent in systemic capillary-leak syndrome (Clarkson disease) severe episodes. ( 29477581 )
2018
18
Recognition and management of idiopathic systemic capillary leak syndrome: an evidence-based review. ( 29564922 )
2018
19
Capillary leak syndrome in Daboia russelii bite-a complication associated with poor outcome. ( 29584906 )
2018
20
A Viral Polymyositis Masquerade: Life-Threatening Case of Juvenile Dermatomyositis Complicated by Systemic Capillary Leak Syndrome. ( 29720802 )
2018
21
The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome. ( 29732372 )
2018
22
Idiopathic systemic capillary leak syndrome presenting as septic shock: A case report. ( 29779703 )
2018
23
Anesthetic Considerations for a Patient With Chronic Systemic Capillary Leak Syndrome: A Case Report. ( 29894349 )
2018
24
Idiopathic Systemic Capillary Leak Syndrome. ( 29910550 )
2018
25
Intravenous immunoglobulin therapy for dengue capillary leak syndrome in a renal allograft recipient. ( 29925559 )
2018
26
Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review. ( 29936591 )
2018
27
Capillary Leak Syndrome Aggravated by Influenza Type A Infection. ( 29974010 )
2018
28
Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection. ( 30158273 )
2018
29
Sjögren's syndrome concurrent with protein-losing gastroenteropathy with secondary systemic capillary leak syndrome : A case report. ( 30214773 )
2018
30
Correction: Severe capillary leak syndrome with cardiac arrest triggered by influenza virus infection. ( 30287629 )
2018
31
Probable Mechanisms Involved in Immunotoxin Mediated Capillary Leak Syndrome (CLS) and Recently Developed Countering Strategies. ( 30289072 )
2018
32
Whole Exome Sequencing of Adult and Pediatric Cohorts of the Rare Vascular Disorder Systemic Capillary Leak Syndrome. ( 30289850 )
2018
33
Systemic Capillary Leak Syndrome (Clarkson Syndrome) in Cancer Patients: A Systematic Review. ( 30404164 )
2018
34
Diagnosis of Systemic Capillary Leak Syndrome in a Young Child Treated with Intravenous Immunoglobulin in the Acute Phase. ( 31073478 )
2018
35
Mimicker of hereditary angioedema: Idiopathic systemic capillary leak syndrome successfully treated with intravenous immunoglobulin. ( 28377172 )
2017
36
Systemic capillary leak syndrome and autoimmune diseases: A case series. ( 27637319 )
2017
37
Idiopathic systemic capillary leak syndrome (Clarkson disease). ( 28012935 )
2017
38
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: comment. ( 28122158 )
2017
39
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: reply. ( 28182327 )
2017
40
Distributive Shock in the Emergency Department: Sepsis, Anaphylaxis, or Capillary Leak Syndrome? ( 28238385 )
2017
41
Capillary leak syndrome: etiologies, pathophysiology, and management. ( 28318633 )
2017
42
Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome. ( 28602874 )
2017
43
The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission. ( 28622216 )
2017
44
Capillary leak syndrome in neuromyelitis optica treated with rituximab. ( 28755680 )
2017
45
Gemcitabine-induced chronic systemic capillary leak syndrome. ( 28855215 )
2017
46
Life-threatening systemic capillary leak syndrome in juvenile dermatomyositis. ( 28957546 )
2017
47
A p190BRhoGAP mutation and prolonged RhoB activation in fatal systemic capillary leak syndrome. ( 29097442 )
2017
48
Capillary Leak Syndrome Following Snakebite Envenomation. ( 29142382 )
2017
49
How relevant are vascular endothelial growth factor and intercellular adhesion molecule in the systemic capillary leak syndrome of psoriasis? ( 29364440 )
2017
50
Systemic capillary leak syndrome in a patient receiving adjuvant oxaliplatin for locally advanced colon cancer. ( 26071595 )
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

Biological processes related to Systemic Capillary Leak Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 heart development GO:0007507 9.16 ADM EDNRA
2 response to hypoxia GO:0001666 8.96 ADM EDNRA
3 positive regulation of cytosolic calcium ion concentration GO:0007204 8.62 ADM EDNRA

Sources for Systemic Capillary Leak Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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