MCID: SYS007
MIFTS: 41

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 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 superior limbic keratoconjunctivitis and capillary leak syndrome. An important gene associated with Systemic Capillary Leak Syndrome is EDNRA (Endothelin Receptor Type A). The drugs Amoxicillin and Clavulanic Acid have been mentioned in the context of this disorder. Affiliated tissues include kidney, endothelial and liver, and related phenotypes are leukocytosis and edema of the lower limbs

Related Diseases for Systemic Capillary Leak Syndrome

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 leukocytosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0001974
2 edema of the lower limbs 59 32 hallmark (90%) Very frequent (99-80%) HP:0010741
3 pancreatitis 59 32 frequent (33%) Frequent (79-30%) HP:0001733
4 weight loss 59 32 frequent (33%) Frequent (79-30%) HP:0001824
5 diarrhea 59 32 frequent (33%) Frequent (79-30%) HP:0002014
6 abdominal pain 59 32 frequent (33%) Frequent (79-30%) HP:0002027
7 hypotension 59 32 frequent (33%) Frequent (79-30%) HP:0002615
8 myalgia 59 32 frequent (33%) Frequent (79-30%) HP:0003326
9 fatigue 59 32 frequent (33%) Frequent (79-30%) HP:0012378
10 rhinorrhea 59 32 frequent (33%) Frequent (79-30%) HP:0031417
11 pulmonary edema 59 32 frequent (33%) Frequent (79-30%) HP:0100598
12 renal insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0000083
13 abnormality of the renal tubule 59 32 occasional (7.5%) Occasional (29-5%) HP:0000091
14 pericarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001701
15 pleural effusion 59 32 occasional (7.5%) Occasional (29-5%) HP:0002202
16 venous thrombosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0004936
17 cardiorespiratory arrest 59 32 occasional (7.5%) Occasional (29-5%) HP:0006543
18 multiple myeloma 59 32 occasional (7.5%) Occasional (29-5%) HP:0006775
19 arrhythmia 59 32 occasional (7.5%) Occasional (29-5%) HP:0011675
20 cough 59 32 occasional (7.5%) Occasional (29-5%) HP:0012735
21 myocarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012819
22 oliguria 59 32 occasional (7.5%) Occasional (29-5%) HP:0100520
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 83)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Amoxicillin Approved, Vet_approved Phase 4 26787-78-0 33613
2 Clavulanic Acid Phase 4
3 Anti-Bacterial Agents Phase 4
4 Antibiotics, Antitubercular Phase 4
5 Anti-Infective Agents Phase 4,Phase 3,Phase 2
6 Antitubercular Agents Phase 4
7 Amoxicillin-Potassium Clavulanate Combination Phase 4
8 beta-Lactamase Inhibitors Phase 4
9
Isotretinoin Approved Phase 3,Phase 2 4759-48-2 5538 5282379
10
Aldesleukin Approved Phase 3,Phase 2,Phase 1 85898-30-2, 110942-02-4
11
Dopamine Approved Phase 3 51-61-6, 62-31-7 681
12
Acetaminophen Approved Phase 3 103-90-2 1983
13
Ibuprofen Approved Phase 3 15687-27-1 3672
14
Metoclopramide Approved, Investigational Phase 3 364-62-5 4168
15
Codeine Approved, Illicit Phase 3 76-57-3 5284371
16
Ondansetron Approved Phase 3 99614-02-5 4595
17
Guaifenesin Approved, Investigational, Vet_approved Phase 3 93-14-1 3516
18
Vitamin A Approved, Nutraceutical, Vet_approved Phase 3 11103-57-4, 68-26-8 445354
19
Serine Approved, Nutraceutical Phase 3 56-45-1 5951
20
Tretinoin Approved, Investigational, Nutraceutical Phase 3,Phase 2 302-79-4 5538 444795
21 Interleukin-2 Phase 3,Phase 2,Phase 1
22 Analgesics Phase 3,Phase 2
23 Retinol palmitate Phase 3
24 Analgesics, Non-Narcotic Phase 3,Phase 2
25 Dermatologic Agents Phase 3,Phase 2
26 Peripheral Nervous System Agents Phase 3,Phase 2
27 Vitamins Phase 3
28 Antibodies Phase 3,Phase 2,Phase 1
29 Immunoglobulins Phase 3,Phase 2,Phase 1
30 Antibodies, Monoclonal Phase 3,Phase 1
31 Anti-HIV Agents Phase 3,Phase 2
32 Keratolytic Agents Phase 3,Phase 2
33 Anti-Retroviral Agents Phase 3,Phase 2
34 Antiviral Agents Phase 3,Phase 2
35 Respiratory System Agents Phase 3
36 Central Nervous System Depressants Phase 3
37 Chlorpheniramine, phenylpropanolamine drug combination Phase 3
38 Serotonin Agents Phase 3
39 Serotonin Antagonists Phase 3
40 Gastrointestinal Agents Phase 3
41 Narcotics Phase 3
42 Neurotransmitter Agents Phase 3
43 Cyclooxygenase Inhibitors Phase 3
44 Analgesics, Opioid Phase 3
45 Tranquilizing Agents Phase 3
46 Anti-Anxiety Agents Phase 3
47 Dopamine Agents Phase 3
48 Dopamine Antagonists Phase 3
49 Dopamine D2 Receptor Antagonists Phase 3
50 Antiemetics Phase 3

Interventional clinical trials:

(show all 20)
# 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 Mesenchymal Stromal Cells in Living Donor Kidney Transplantation Recruiting NCT03478215 Phase 2 Tacrolimus;Mycophenolate Mofetil;Basiliximab
6 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
7 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
8 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
9 DT2219ARL for Relapsed or Refractory CD19 (+), CD 22 (+) B-Lineage Leukemia Or Lymphoma Completed NCT00889408 Phase 1
10 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)
11 Human Leukocyte Antigen (HLA) Class I Haplotype Mismatched Natural Killer Cell Infusions Completed NCT00660166 Phase 1
12 Combination Chemotherapy, Monoclonal Antibody, and Natural Killer Cells in Treating Young Patients With Recurrent or Refractory Neuroblastoma Active, not recruiting NCT01576692 Phase 1 Chemotherapy
13 Monitoring the IMmUological TOXicity of Drugs Completed NCT03480529 drug inducing arthritis, lupus or capillary leak syndrom
14 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)
15 Evaluation of Endothelial Dysfunction During Aortic Valvular Replacement by Bioprosthesis With and Without Extracorporeal Circulation Completed NCT03135496
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 Characterization of Endothelial Dysfunction as a Function of Hyperaemia of the Brachial Artery During Cardiac Surgery Recruiting NCT02853682
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

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
Kidney, Endothelial, Liver, Heart, Bone, B Cells, Bone Marrow

Publications for Systemic Capillary Leak Syndrome

Articles related to Systemic Capillary Leak Syndrome:

(show top 50) (show all 256)
# Title Authors Year
1
The Role of Failing Autonomic Nervous System on Life-Threatening Idiopathic Systemic Capillary Leak Syndrome. ( 29732372 )
2018
2
Capillary Leak Syndrome Aggravated by Influenza Type A Infection. ( 29974010 )
2018
3
Intravenous immunoglobulin therapy for dengue capillary leak syndrome in a renal allograft recipient. ( 29925559 )
2018
4
Recognition and management of idiopathic systemic capillary leak syndrome: an evidence-based review. ( 29564922 )
2018
5
Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review. ( 29936591 )
2018
6
Capillary leak syndrome as a complication of antibody-mediated rejection treatment: a case report. ( 29344912 )
2018
7
Adrenomedullin surges are linked to acute episodes of the systemic capillary leak syndrome (Clarkson disease). ( 29360169 )
2018
8
Idiopathic Systemic Capillary Leak Syndrome. ( 29910550 )
2018
9
A Viral Polymyositis Masquerade: Life-Threatening Case of Juvenile Dermatomyositis Complicated by Systemic Capillary Leak Syndrome. ( 29720802 )
2018
10
Idiopathic systemic capillary leak syndrome presenting as septic shock: A case report. ( 29779703 )
2018
11
Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson's Disease). ( 29449526 )
2018
12
Capillary leak syndrome and aseptic meningitis in a patient with Kawasaki disease: A case report. ( 29879013 )
2018
13
Myocardial dysfunction is frequent in systemic capillary-leak syndrome (Clarkson disease) severe episodes. ( 29477581 )
2018
14
Capillary leak syndrome in Daboia russelii bite-a complication associated with poor outcome. ( 29584906 )
2018
15
Anesthetic Considerations for a Patient With Chronic Systemic Capillary Leak Syndrome: A Case Report. ( 29894349 )
2018
16
How relevant are vascular endothelial growth factor and intercellular adhesion molecule in the systemic capillary leak syndrome of psoriasis? ( 29364440 )
2017
17
Capillary Leak Syndrome Following Snakebite Envenomation. ( 29142382 )
2017
18
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: reply. ( 28182327 )
2017
19
Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review. ( 28939140 )
2017
20
A p190BRhoGAP mutation and prolonged RhoB activation in fatal systemic capillary leak syndrome. ( 29097442 )
2017
21
The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission. ( 28622216 )
2017
22
Capillary leak syndrome: etiologies, pathophysiology, and management. ( 28318633 )
2017
23
Life-threatening systemic capillary leak syndrome in juvenile dermatomyositis. ( 28957546 )
2017
24
Capillary leak syndrome in neuromyelitis optica treated with rituximab. ( 28755680 )
2017
25
Mimicker of hereditary angioedema: Idiopathic systemic capillary leak syndrome successfully treated with intravenous immunoglobulin. ( 28377172 )
2017
26
Gemcitabine-induced chronic systemic capillary leak syndrome. ( 28855215 )
2017
27
Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome. ( 28602874 )
2017
28
Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: comment. ( 28122158 )
2017
29
Distributive Shock in the Emergency Department: Sepsis, Anaphylaxis, or Capillary Leak Syndrome? ( 28238385 )
2017
30
Epidemiology, risk factors, and prognosis of capillary leak syndrome in pediatric recipients of stem cell transplants: a retrospective single-center cohort study. ( 27734564 )
2016
31
Severe cerebral involvement due to idiopathic systemic capillary leak syndrome. ( 27099745 )
2016
32
Idiopathic systemic capillary leak syndrome (Clarkson disease). ( 28012935 )
2016
33
Subcutaneous Immunoglobulin Therapy Prevents Systemic Capillary Leak Syndrome Attack. ( 27059380 )
2016
34
Fulminant capillary leak syndrome in a patient with systemic sclerosis treated with imatinib mesylate. ( 27338086 )
2016
35
Sudden cardiac arrest in a 73-year-old woman caused by systemic capillary leak syndrome. ( 27056382 )
2016
36
Microangiopathic Hemolytic Anemia Due to ADAMTS-13 Loss in Idiopathic Systemic Capillary Leak Syndrome. ( 27622772 )
2016
37
Isoform D of vascular endothelial growth factor in systemic capillary leak syndrome: a case report. ( 27386947 )
2016
38
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
39
Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome. ( 27688917 )
2016
40
Rhabdomyolysis and capillary leak syndrome in a patient treated with topical imiquimod. ( 27061736 )
2016
41
Scrub typhus presenting as capillary leak syndrome. ( 27728427 )
2016
42
Systemic capillary leak syndrome and autoimmune diseases: A case series. ( 27637319 )
2016
43
Fatal Primary Capillary Leak Syndrome in a Late Preterm Newborn. ( 27142601 )
2016
44
Idiopathic Systemic Capillary Leak Syndrome: A Case Report. ( 27195144 )
2016
45
A Case of Refractory Systemic Capillary Leak Syndrome (Clarkson's Disease) during Pregnancy. ( 28003676 )
2016
46
1197: ACUTE THERAPY WITH IVIG IN A PEDIATRIC PATIENT WITH IDIOPATHIC SYSTEMIC CAPILLARY LEAK SYNDROME. ( 26570858 )
2015
47
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
48
A case report of idiopathic systemic capillary leak syndrome that occurred during the postoperative period of abdominoperineal resection for colorectal cancer. ( 25594640 )
2015
49
An uncommon cause of distributive shock: Lessons from two consecutive cases of idiopathic systemic capillary leak syndrome (Clarkson's disease). ( 26300309 )
2015
50
Clinical and pathological findings of a fatal systemic capillary leak syndrome (Clarkson disease): a case report. ( 25738482 )
2015

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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