MCID: CTS005
MIFTS: 45

Catastrophic Antiphospholipid Syndrome

Categories: Blood diseases, Bone diseases, Immune diseases, Rare diseases

Aliases & Classifications for Catastrophic Antiphospholipid Syndrome

MalaCards integrated aliases for Catastrophic Antiphospholipid Syndrome:

Name: Catastrophic Antiphospholipid Syndrome 54 60 74
Catastrophic Antiphospholipid Antibody Syndrome 54
Cryopyrin-Associated Periodic Syndromes 74
Thrombotic Storm 54
Catastrophic Aps 60
Caps 60

Classifications:



External Ids:

ICD10 via Orphanet 35 D68.6
Orphanet 60 ORPHA464343

Summaries for Catastrophic Antiphospholipid Syndrome

NIH Rare Diseases : 54 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.

MalaCards based summary : Catastrophic Antiphospholipid Syndrome, also known as catastrophic antiphospholipid antibody syndrome, is related to heparin-induced thrombocytopenia and thrombocytopenia. 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 and Antibodies, Antiphospholipid have been mentioned in the context of this disorder. Affiliated tissues include bone, bone marrow and heart, and related phenotypes are homeostasis/metabolism and hematopoietic system

Wikipedia : 77 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 166)
# Related Disease Score Top Affiliating Genes
1 heparin-induced thrombocytopenia 30.6 CD40LG F3
2 thrombocytopenia 30.3 ADAMTS13 APOH F3
3 antiphospholipid syndrome 30.3 ADAMTS13 APOH CD40LG F3 F5 MTHFR
4 purpura fulminans 30.0 F3 PROC
5 hepatic infarction 29.9 APOH F3 MTHFR
6 coronary thrombosis 29.8 F3 SERPINE1
7 hemolytic-uremic syndrome 29.8 ADAMTS13 CFH F3
8 thrombotic thrombocytopenic purpura 29.7 ADAMTS13 APOH CFH F3
9 branch retinal artery occlusion 29.7 APOH F3 MTHFR
10 purpura 29.5 ADAMTS13 APOH F3 PROC
11 patent foramen ovale 29.5 APOH F5 MTHFR
12 retinal artery occlusion 29.1 APOH F5 MTHFR SERPINE1
13 disseminated intravascular coagulation 29.0 ADAMTS13 F3 F5 SERPINE1
14 protein s deficiency 29.0 APOH F3 F5 MTHFR
15 pulmonary embolism 28.8 APOH F3 F5 MTHFR SERPINE1
16 thrombosis 28.5 ADAMTS13 APOH F3 F5 MTHFR PROC
17 stroke, ischemic 28.3 ADAMTS13 APOH F3 F5 MTHFR SERPINE1
18 myocardial infarction 28.3 ADAMTS13 CD40LG F3 F5 MTHFR SERPINE1
19 hellp syndrome 28.0 ADAMTS13 APOH CFH F3 F5 MTHFR
20 lupus erythematosus 10.4
21 systemic lupus erythematosus 10.4
22 erythema infectiosum 10.3 APOH CD40LG
23 thrombotic thrombocytopenic purpura, congenital 10.3 ADAMTS13 CFH
24 primary thrombocytopenia 10.3 ADAMTS13 CD40LG
25 syphilis 10.3 APOH CD40LG
26 mixed connective tissue disease 10.2 ADAMTS13 APOH
27 central serous chorioretinopathy 10.2 CFH SERPINE1
28 lymphoma 10.2
29 acute respiratory distress syndrome 10.2
30 infective endocarditis 10.2 APOH SERPINE1
31 fournier gangrene 10.1 CD40LG F3
32 carotid artery dissection 10.1 ADAMTS13 MTHFR
33 connective tissue disease 10.1 ADAMTS13 APOH CD40LG
34 endocarditis 10.1
35 nephrosclerosis 10.1 ADAMTS13 MTHFR
36 intermittent claudication 10.1 APOH SERPINE1
37 von willebrand disease, type 2 10.1 ADAMTS13 F3
38 may-thurner syndrome 10.1 APOH F5
39 acanthamoeba keratitis 10.1 APOH F5
40 pregnancy loss, recurrent 1 10.1 APOH F5
41 blue toe syndrome 10.1 F3 SERPINE1
42 carotid artery thrombosis 10.0 F3 SERPINE1
43 prothrombin deficiency, congenital 10.0 APOH F5
44 dysbaric osteonecrosis 10.0 F3 SERPINE1
45 down syndrome 10.0
46 hemorrhage, intracerebral 10.0
47 glomerulonephritis 10.0
48 chikungunya 10.0
49 typhoid fever 10.0
50 pancreatitis 10.0

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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.81 ADAMTS13 APOH CD40LG CFH F3 F5
2 hematopoietic system MP:0005397 9.8 ADAMTS13 CD40LG CFH CRLS1 F3 PROC
3 immune system MP:0005387 9.5 ADAMTS13 CD40LG CFH CRLS1 F3 PROC
4 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 24)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Antibodies Phase 2
2 Antibodies, Antiphospholipid Phase 2
3 Immunoglobulins Phase 2
4 Immunologic Factors Phase 2,Phase 1
5
Fludarabine Approved Phase 1 75607-67-9, 21679-14-1 30751
6
Benzocaine Approved, Investigational Phase 1 1994-09-7, 94-09-7 2337
7
Diphenhydramine Approved, Investigational Phase 1 147-24-0, 58-73-1 3100
8
Prednisone Approved, Vet_approved Phase 1 53-03-2 5865
9
tannic acid Approved Phase 1 1401-55-4
10
alemtuzumab Approved, Investigational Phase 1 216503-57-0
11
Cyclophosphamide Approved, Investigational Phase 1 50-18-0, 6055-19-2 2907
12
Hydrocortisone Approved, Vet_approved Phase 1 50-23-7 5754
13
Hydrocortisone acetate Approved, Vet_approved Phase 1 50-03-3
14
Promethazine Approved, Investigational Phase 1 60-87-7 4927
15
Acetaminophen Approved Phase 1 103-90-2 1983
16 Hydrocortisone 17-butyrate 21-propionate Phase 1
17 Antigen-Antibody Complex Phase 1
18 Antineoplastic Agents, Alkylating Phase 1
19 Alkylating Agents Phase 1
20 Antineoplastic Agents, Immunological Phase 1
21 Antirheumatic Agents Phase 1
22 Immunosuppressive Agents Phase 1
23 Hydrocortisone hemisuccinate Phase 1
24 diuretics 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 74 / NDF-RT 52 :


Genetic Tests for Catastrophic Antiphospholipid Syndrome

Anatomical Context for Catastrophic Antiphospholipid Syndrome

MalaCards organs/tissues related to Catastrophic Antiphospholipid Syndrome:

42
Bone, Bone Marrow, Heart, Liver, Skin, Endothelial, Lung

Publications for Catastrophic Antiphospholipid Syndrome

Articles related to Catastrophic Antiphospholipid Syndrome:

(show top 50) (show all 319)
# Title Authors Year
1
Catastrophic antiphospholipid syndrome causing ST-segment elevation myocardial infarction with non-obstructive coronary arteries. ( 30635301 )
2019
2
Revisiting the role of therapeutic plasma exchange in the management of catastrophic antiphospholipid syndrome. ( 30720923 )
2019
3
Tumid lupus erythematosus-like pseudovasculitic lesions in catastrophic antiphospholipid syndrome. ( 30733989 )
2019
4
Catastrophic Antiphospholipid Syndrome Presenting as Congestive Heart Failure in a Patient with Thrombotic Microangiopathy. ( 30833839 )
2019
5
Evidence of complement activation in the thrombotic small vessels of a patient with catastrophic antiphospholipid syndrome treated with eculizumab. ( 30844561 )
2019
6
Rare case of catastrophic antiphospholipid syndrome with spontaneous intracranial haemorrhage. ( 30898956 )
2019
7
Catastrophic Antiphospholipid Syndrome as a Cause of Severe Gastrointestinal Bleeding. ( 30904512 )
2019
8
A probable case of catastrophic antiphospholipid syndrome: Should high-dose steroids be given in the setting of polymicrobial sepsis? ( 30967955 )
2019
9
A case-report of successful use of thromboelastography to manage anticoagulation therapy in a patient with concomitant catastrophic antiphospholipid syndrome and heparin-induced thrombocytopenia. ( 31090599 )
2019
10
Catastrophic antiphospholipid syndrome and pregnancy. Clinical report. ( 29284338 )
2019
11
Catastrophic antiphospholipid antibody syndrome in a young child. ( 30932176 )
2019
12
The role of hydroxychloroquine in catastrophic antiphospholipid syndrome case: Series of two case reports and review of literature. ( 30046446 )
2018
13
Eculizumab for catastrophic antiphospholipid syndrome-a case report and literature review. ( 30101319 )
2018
14
Catastrophic antiphospholipid syndrome: Lessons from 14 cases successfully treated in a single center. A narrative report. ( 30146007 )
2018
15
Eculizumab in a pregnant patient with laboratory onset of catastrophic antiphospholipid syndrome: A case report. ( 30290624 )
2018
16
Bilateral central retinal artery occlusion from catastrophic antiphospholipid syndrome. ( 30420561 )
2018
17
Post-partum catastrophic antiphospholipid syndrome presenting with shock and digital ischaemia - A diagnostic and management challenge. ( 30515245 )
2018
18
Co-existing Bilateral Pulmonary Embolism and Intra-cardiac Mass: A Case of Catastrophic Antiphospholipid Syndrome-like Disease. ( 30546985 )
2018
19
Rare Presentation of Catastrophic Antiphospholipid Syndrome with Myocarditis in Post-partum Period: Case Report and Review of Literature. ( 29391679 )
2018
20
Therapeutic apheresis in the treatment of catastrophic antiphospholipid syndrome. ( 29478799 )
2018
21
Microthrombotic renal involvement in an SLE patient with concomitant catastrophic antiphospholipid syndrome: the beneficial effect of rituximab treatment. ( 29635999 )
2018
22
The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review. ( 29779928 )
2018
23
Immunomodulation in the Treatment of Refractory Catastrophic Antiphospholipid Syndrome. ( 29805821 )
2018
24
Clinical Report of Probable Catastrophic Antiphospholipid Syndrome in Pregnancy. ( 29808136 )
2018
25
McMaster RARE-Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome. ( 29978552 )
2018
26
Catastrophic antiphospholipid syndrome (CAPS)-induced ischemic pancreatic ducts injury mimicking intraductal papillary mucinous neoplasm (IPMN). ( 28802775 )
2018
27
Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology. ( 28829758 )
2018
28
Catastrophic antiphospholipid syndrome and pregnancy. ( 29179957 )
2018
29
Probable Catastrophic Antiphospholipid Syndrome with Intracerebral Hemorrhage Secondary to Epstein-Barr Viral Infection. ( 28357636 )
2018
30
Catastrophic Antiphospholipid Antibody Syndrome After Cobra Bite in a Patient With Systemic Lupus Erythematosus. ( 30585994 )
2018
31
Catastrophic antiphospholipid antibody syndrome presenting as acute vascular occlusion in a young female patient. ( 27745843 )
2018
32
The Wolf Hidden behind the Clots: Catastrophic Antiphospholipid Antibody Syndrome. ( 30123280 )
2018
33
Catastrophic antiphospholipid syndrome: A case with unusual findings revealed in autopsy and a brief literature update. ( 29308285 )
2017
34
Catastrophic antiphospholipid syndrome: an update. ( 28488841 )
2017
35
Lupus-Negative Libman-Sacks Endocarditis Complicated by Catastrophic Antiphospholipid Syndrome. ( 28515823 )
2017
36
The catastrophic antiphospholipid syndrome in children. ( 28632503 )
2017
37
Catastrophic antiphospholipid syndrome treated with rituximab: A case report. ( 28638691 )
2017
38
Catastrophic Antiphospholipid Syndrome: Candidate Therapies for a Potentially Lethal Disease. ( 28099080 )
2017
39
Diagnosis and management of catastrophic antiphospholipid syndrome. ( 28277850 )
2017
40
Catastrophic antiphospholipid syndrome on switching from warfarin to rivaroxaban. ( 28319823 )
2017
41
Cutaneous gangrene of the arms and legs after cardiopulmonary resuscitation: A rare presentation of catastrophic antiphospholipid syndrome. ( 27396539 )
2017
42
Successful Treatment of Catastrophic Antiphospholipid Antibody Syndrome Associated with MALT Lymphoma by Autologous Hematopoietic Stem Cell Transplantation. ( 28502938 )
2017
43
Catastrophic antiphospholipid antibody syndrome presenting as severe ischaemic colitis. ( 27939948 )
2017
44
[Complement System Abnormalities in Patients with Atypical Hemolytic Uremic Syndrome and Catastrophic Antiphospholipid Syndrome]. ( 29308852 )
2017
45
Catastrophic Antiphospholipid Syndrome. ( 27375916 )
2016
46
Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies. ( 27380476 )
2016
47
Catastrophic antiphospholipid syndrome after cardiac surgery. ( 27455170 )
2016
48
Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry. ( 27639837 )
2016
49
Hepatic infarction in a pregnant woman with antiphospholipid syndrome and triple antibody positivity: A case report focusing on catastrophic antiphospholipid syndrome. ( 27668204 )
2016
50
Plasma Exchange in the Management of Catastrophic Antiphospholipid Syndrome. ( 27833765 )
2016

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 collagen-containing extracellular matrix GO:0062023 9.43 APOH F3 SERPINE1
4 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
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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