MCID: CTS005
MIFTS: 45

Catastrophic Antiphospholipid Syndrome

Categories: Rare diseases, Blood diseases, Immune diseases

Aliases & Classifications for Catastrophic Antiphospholipid Syndrome

MalaCards integrated aliases for Catastrophic Antiphospholipid Syndrome:

Name: Catastrophic Antiphospholipid Syndrome 49 55 69
Catastrophic Antiphospholipid Antibody Syndrome 49
Cryopyrin-Associated Periodic Syndromes 69
Thrombotic Storm 49
Catastrophic Aps 55
Caps 55

Classifications:



External Ids:

Orphanet 55 ORPHA464343
ICD10 via Orphanet 33 D68.6

Summaries for Catastrophic Antiphospholipid Syndrome

NIH Rare Diseases : 49 Catastrophic antiphospholipid syndrome (CAPS) is a rare form of antiphospholipid syndrome (APS). In CAPS multiple blood clots form throughout the body over a short period of time (usually within a week). CAPS is a medical emergency, as clots can cause life-threatening multi-organ failure. The cause of CAPS is unknown. A widely accepted explanation is that it is caused by a combination of genemutations (making one more susceptible to CAPS) and an environmental trigger, such as an infection, trauma, or surgery. Last updated: 5/25/2016

MalaCards based summary : Catastrophic Antiphospholipid Syndrome, also known as catastrophic antiphospholipid antibody syndrome, is related to thrombocytopenia and antiphospholipid syndrome. An important gene associated with Catastrophic Antiphospholipid Syndrome is APOH (Apolipoprotein H), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and Formation of Fibrin Clot (Clotting Cascade). The drugs Antibodies, Antiphospholipid and Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include liver, bone and endothelial, and related phenotypes are hematopoietic system and homeostasis/metabolism

Wikipedia : 72 Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson\'s syndrome, is an acute and... more...

Related Diseases for Catastrophic Antiphospholipid Syndrome

Diseases related to Catastrophic Antiphospholipid Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 171)
# Related Disease Score Top Affiliating Genes
1 thrombocytopenia 30.3 ADAMTS13 APOH F3
2 antiphospholipid syndrome 30.2 ADAMTS13 APOH CD40LG F3 F5 MTHFR
3 heparin-induced thrombocytopenia 30.2 CD40LG F3
4 thrombotic thrombocytopenic purpura, congenital 30.1 ADAMTS13 CFH
5 hepatic infarction 30.1 APOH F3 MTHFR
6 patent foramen ovale 29.9 APOH F5 MTHFR
7 protein s deficiency 29.4 APOH F3 F5 MTHFR
8 retinal artery occlusion 29.0 APOH F5 MTHFR SERPINE1
9 purpura 28.9 ADAMTS13 APOH CD40LG F3 PROC
10 disseminated intravascular coagulation 28.9 ADAMTS13 F3 F5 SERPINE1
11 thrombosis 28.7 APOH F3 F5 MTHFR PROC SERPINE1
12 pulmonary embolism 28.5 APOH F3 F5 MTHFR SERPINE1
13 stroke, ischemic 28.5 APOH F3 F5 MTHFR SERPINE1
14 hellp syndrome 27.6 ADAMTS13 APOH CFH F3 F5 MTHFR
15 may-thurner syndrome 10.4 APOH F5
16 blood protein disease 10.4 MTHFR PROC
17 acanthamoeba keratitis 10.4 APOH F5
18 prothrombin deficiency, congenital 10.4 APOH F5
19 sneddon syndrome 10.4 APOH F5
20 cataract 32, multiple types 10.4
21 sudden sensorineural hearing loss 10.4 F5 MTHFR
22 carotid artery occlusion 10.3 APOH F5
23 carotid artery dissection 10.3 ADAMTS13 MTHFR
24 porencephaly 10.3 F5 MTHFR
25 purpura fulminans 10.3 F3 PROC
26 acquired hemophilia a 10.3 F3 F5
27 acquired hemophilia 10.3 F3 F5
28 sagittal sinus thrombosis 10.3 F3 F5
29 borderline leprosy 10.3 APOH CD40LG
30 viral exanthem 10.3 APOH CD40LG
31 erythema infectiosum 10.2 APOH CD40LG
32 factor v deficiency 10.2 F3 F5
33 pyoderma 10.2 APOH F3
34 arteritic anterior ischemic optic neuropathy 10.2 F5 MTHFR
35 nephrosclerosis 10.2 ADAMTS13 MTHFR
36 evans' syndrome 10.2 ADAMTS13 CD40LG
37 spinal cord infarction 10.2 APOH F5 MTHFR
38 pregnancy loss, recurrent 1 10.2 APOH F5 MTHFR
39 retinal vascular occlusion 10.2 APOH F5 MTHFR
40 antithrombin iii deficiency 10.2 APOH F5 MTHFR
41 hemophilia a 10.2 F3 F5
42 central retinal vein occlusion 10.2 APOH F5 MTHFR
43 schizencephaly 10.2 F5 MTHFR
44 placental abruption 10.2 APOH F5 MTHFR
45 primary thrombocytopenia 10.2 ADAMTS13 CD40LG
46 ischemic optic neuropathy 10.2 APOH F5 MTHFR
47 systemic lupus erythematosus 10.2
48 lupus erythematosus 10.2
49 autoimmune disease of blood 10.2 ADAMTS13 CD40LG
50 protein c deficiency 10.1 F5 MTHFR PROC

Graphical network of the top 20 diseases related to Catastrophic Antiphospholipid Syndrome:



Diseases related to Catastrophic Antiphospholipid Syndrome

Symptoms & Phenotypes for Catastrophic Antiphospholipid Syndrome

MGI Mouse Phenotypes related to Catastrophic Antiphospholipid Syndrome:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.7 ADAMTS13 CD40LG CFH CRLS1 F3 PROC
2 homeostasis/metabolism MP:0005376 9.61 ADAMTS13 APOH CD40LG CFH F3 F5
3 mortality/aging MP:0010768 9.28 ADAMTS13 APOH CD40LG CFH F3 F5

Drugs & Therapeutics for Catastrophic Antiphospholipid Syndrome

Drugs for Catastrophic Antiphospholipid Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 21)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Antibodies, Antiphospholipid Phase 2
2 Immunoglobulins Phase 2
3 Antibodies Phase 2
4
Cyclophosphamide Approved, Investigational Phase 1 50-18-0, 6055-19-2 2907
5
Acetaminophen Approved Phase 1 103-90-2 1983
6
Fludarabine Approved Phase 1 21679-14-1, 75607-67-9 30751
7
Hydrocortisone Approved, Vet_approved Phase 1 50-23-7 657311 5754
8
Diphenhydramine Approved, Investigational Phase 1 58-73-1, 147-24-0 3100
9
alemtuzumab Approved, Investigational Phase 1 216503-57-0
10
Benzocaine Approved, Investigational Phase 1 1994-09-7, 94-09-7 2337
11
Promethazine Approved, Investigational Phase 1 60-87-7 4927
12
Prednisone Approved, Vet_approved Phase 1 53-03-2 5865
13 tannic acid Approved, Nutraceutical Phase 1
14 Alkylating Agents Phase 1
15 diuretics Phase 1
16 Cortisol succinate Phase 1
17 Hydrocortisone 17-butyrate 21-propionate Phase 1
18 Hydrocortisone acetate Phase 1
19 Antigen-Antibody Complex Phase 1
20 Immunosuppressive Agents Phase 1
21 Antirheumatic Agents Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Eculizumab to Enable Renal Transplantation in Patients With History of Catastrophic Antiphospholipid Antibody Syndrome Active, not recruiting NCT01029587 Phase 2 Eculizumab
2 Nonmyeloablative Allo Stem Cell Transplant for Severe Autoimmune Diseases Terminated NCT00849745 Phase 1

Search NIH Clinical Center for Catastrophic Antiphospholipid Syndrome

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Genetic Tests for Catastrophic Antiphospholipid Syndrome

Anatomical Context for Catastrophic Antiphospholipid Syndrome

MalaCards organs/tissues related to Catastrophic Antiphospholipid Syndrome:

38
Liver, Bone, Endothelial, Bone Marrow, Heart, Skin, Lung

Publications for Catastrophic Antiphospholipid Syndrome

Articles related to Catastrophic Antiphospholipid Syndrome:

(show top 50) (show all 260)
# Title Authors Year
1
Catastrophic antiphospholipid syndrome and pregnancy. Clinical report. ( 29284338 )
2018
2
Catastrophic antiphospholipid syndrome and pregnancy. ( 29179957 )
2018
3
Rare Presentation of Catastrophic Antiphospholipid Syndrome with Myocarditis in Post-partum Period: Case Report and Review of Literature. ( 29391679 )
2018
4
Complement System Abnormalities in Patients with Atypical Hemolytic Uremic Syndrome and Catastrophic Antiphospholipid Syndrome. ( 29308852 )
2017
5
Catastrophic antiphospholipid syndrome treated with rituximab: A case report. ( 28638691 )
2017
6
Catastrophic antiphospholipid syndrome (CAPS)-induced ischemic pancreatic ducts injury mimicking intraductal papillary mucinous neoplasm (IPMN). ( 28802775 )
2017
7
Catastrophic Antiphospholipid Syndrome: Candidate Therapies for a Potentially Lethal Disease. ( 28099080 )
2017
8
Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology. ( 28829758 )
2017
9
Probable Catastrophic Antiphospholipid Syndrome with Intracerebral Hemorrhage Secondary to Epstein-Barr Viral Infection. ( 28357636 )
2017
10
Diagnosis and management of catastrophic antiphospholipid syndrome. ( 28277850 )
2017
11
Lupus-Negative Libman-Sacks Endocarditis Complicated by Catastrophic Antiphospholipid Syndrome. ( 28515823 )
2017
12
Catastrophic antiphospholipid syndrome: an update. ( 28488841 )
2017
13
The catastrophic antiphospholipid syndrome in children. ( 28632503 )
2017
14
Catastrophic antiphospholipid syndrome on switching from warfarin to rivaroxaban. ( 28319823 )
2017
15
Catastrophic antiphospholipid syndrome: A case with unusual findings revealed in autopsy and a brief literature update. ( 29308285 )
2017
16
Treatment of catastrophic antiphospholipid syndrome. ( 26927441 )
2016
17
Catastrophic antiphospholipid syndrome after cardiac surgery. ( 27455170 )
2016
18
Catastrophic antiphospholipid syndrome in pregnancy, a diagnosis that should not be missed. ( 27023104 )
2016
19
Catastrophic Antiphospholipid Syndrome. ( 27375916 )
2016
20
Plasma Exchange in the Management of Catastrophic Antiphospholipid Syndrome. ( 27833765 )
2016
21
Hepatic infarction in a pregnant woman with antiphospholipid syndrome and triple antibody positivity: A case report focusing on catastrophic antiphospholipid syndrome. ( 27668204 )
2016
22
Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies. ( 27380476 )
2016
23
Uterine Artery Embolization Resulting in Catastrophic Antiphospholipid Syndrome in a Patient with Primary Antiphospholipid Syndrome. ( 27106650 )
2016
24
Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry. ( 27639837 )
2016
25
Cutaneous gangrene of the arms and legs after cardiopulmonary resuscitation: A rare presentation of catastrophic antiphospholipid syndrome. ( 27396539 )
2016
26
Complement activation is a crucial pathogenic factor in catastrophic antiphospholipid syndrome. ( 27105662 )
2016
27
Catastrophic Antiphospholipid Syndrome Triggered by Fulminant Chikungunya Infection in a Patient With Systemic Lupus Erythematosus. ( 26748818 )
2016
28
Catastrophic antiphospholipid syndrome: The current management approach. ( 27886797 )
2016
29
Probable catastrophic antiphospholipid syndrome (CAPS) in case of SLE. ( 27728684 )
2016
30
Catastrophic antiphospholipid syndrome (CAPS): A rare and fatal encounter. ( 25983439 )
2015
31
Back from the Brink: Catastrophic Antiphospholipid Syndrome. ( 25596523 )
2015
32
Catastrophic antiphospholipid syndrome presenting as bilateral central retinal artery occlusions. ( 25722904 )
2015
33
Catastrophic Antiphospholipid syndrome in postpartum simulating peritonitis; a diagnostic trap. ( 26367418 )
2015
34
Seronegative catastrophic antiphospholipid syndrome in a young female presenting with a headache. ( 25559391 )
2015
35
Catastrophic Antiphospholipid Syndrome: Scintigraphic Demonstration With Correlated Cross-Sectional Imaging. ( 26447383 )
2015
36
Hyperferritinemic syndrome: Still's disease and catastrophic antiphospholipid syndrome triggered by fulminant Chikungunya infection: a case report of two patients. ( 26233722 )
2015
37
Atypical Subacute Recurrence of Catastrophic Antiphospholipid Syndrome in a Japanese Female Patient. ( 26568011 )
2015
38
The role of infectious diseases in the catastrophic antiphospholipid syndrome. ( 26209907 )
2015
39
A case of catastrophic antiphospholipid syndrome: first report with advanced cardiac imaging using MRI. ( 26014099 )
2015
40
Eculizumab Induces Sustained Remission in a Patient With Refractory Primary Catastrophic Antiphospholipid Syndrome. ( 26267719 )
2015
41
1272: CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME COMPLICATED BY TAKOTSUBO CARDIOMYOPATHY. ( 26570933 )
2015
42
Catastrophic antiphospholipid syndrome: an orchestra with several musicians. ( 25351020 )
2014
43
Recurrent thrombosis prevention with intravenous immunoglobulin and hydroxychloroquine during pregnancy in a patient with history of catastrophic antiphospholipid syndrome and pregnancy loss. ( 24549974 )
2014
44
All that seems sepsis is not sepsis: However, is this case report really a case of catastrophic antiphospholipid syndrome? ( 24701076 )
2014
45
Renal transplantation in a patient with catastrophic antiphospholipid syndrome and heparin-induced thrombocytopenia. ( 24575510 )
2014
46
Pediatric catastrophic antiphospholipid syndrome: descriptive analysis of 45 patients from the "CAPS Registry". ( 24145009 )
2014
47
Malignancies: a possible "first hit" in the development of catastrophic antiphospholipid syndrome? ( 25351019 )
2014
48
36-year-old female with catastrophic antiphospholipid syndrome treated with eculizumab: a case report and review of literature. ( 25389502 )
2014
49
14th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome. ( 24657970 )
2014
50
Catastrophic antiphospholipid syndrome: case reports and review of the literature. ( 25470889 )
2014

Variations for Catastrophic Antiphospholipid Syndrome

Expression for Catastrophic Antiphospholipid Syndrome

Search GEO for disease gene expression data for Catastrophic Antiphospholipid Syndrome.

Pathways for Catastrophic Antiphospholipid Syndrome

GO Terms for Catastrophic Antiphospholipid Syndrome

Cellular components related to Catastrophic Antiphospholipid Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.56 ADAMTS13 APOH CD40LG CFH F3 F5
2 cell surface GO:0009986 9.46 ADAMTS13 APOH CD40LG F3
3 extracellular space GO:0005615 9.23 ADAMTS13 APOH CD40LG CFH F3 F5

Biological processes related to Catastrophic Antiphospholipid Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 platelet degranulation GO:0002576 9.54 APOH F5 SERPINE1
2 blood coagulation GO:0007596 9.46 ADAMTS13 F3 F5 PROC
3 blood circulation GO:0008015 9.4 F5 MTHFR
4 positive regulation of blood coagulation GO:0030194 9.32 APOH SERPINE1
5 negative regulation of fibrinolysis GO:0051918 9.26 APOH SERPINE1
6 negative regulation of blood coagulation GO:0030195 9.13 APOH PROC SERPINE1
7 hemostasis GO:0007599 8.92 ADAMTS13 F3 F5 PROC

Sources for Catastrophic Antiphospholipid 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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