CAPS
MCID: CTS005
MIFTS: 45

Catastrophic Antiphospholipid Syndrome (CAPS)

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 antiphospholipid syndrome and heparin-induced 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 Immunoglobulins and Antibodies, Antiphospholipid have been mentioned in the context of this disorder. Affiliated tissues include bone, liver and heart, and related phenotypes are homeostasis/metabolism and hematopoietic system

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 165)
# Related Disease Score Top Affiliating Genes
1 antiphospholipid syndrome 30.7 ADAMTS13 APOH CD40LG F3 F5 MTHFR
2 heparin-induced thrombocytopenia 30.5 CD40LG F3
3 thrombocytopenia 30.4 ADAMTS13 APOH F3
4 hepatic infarction 30.1 APOH F3 MTHFR
5 purpura fulminans 30.0 F3 PROC
6 hemolytic-uremic syndrome 29.9 ADAMTS13 CFH F3
7 thrombotic thrombocytopenic purpura 29.9 ADAMTS13 APOH CFH F3
8 branch retinal artery occlusion 29.8 APOH F3 MTHFR
9 purpura 29.5 ADAMTS13 APOH F3 PROC
10 protein s deficiency 29.5 APOH F3 F5 MTHFR
11 disseminated intravascular coagulation 29.2 ADAMTS13 F3 F5 SERPINE1
12 retinal artery occlusion 29.1 APOH F5 MTHFR SERPINE1
13 pulmonary embolism 29.0 APOH F3 F5 MTHFR SERPINE1
14 stroke, ischemic 28.5 ADAMTS13 APOH F3 F5 MTHFR SERPINE1
15 thrombosis 28.5 ADAMTS13 APOH F3 F5 MTHFR PROC
16 myocardial infarction 28.4 ADAMTS13 CD40LG F3 F5 MTHFR SERPINE1
17 hellp syndrome 28.2 ADAMTS13 APOH CFH F3 F5 MTHFR
18 lupus erythematosus 10.4
19 systemic lupus erythematosus 10.4
20 thrombotic thrombocytopenic purpura, congenital 10.3 ADAMTS13 CFH
21 erythema infectiosum 10.3 APOH CD40LG
22 may-thurner syndrome 10.3 APOH F5
23 acanthamoeba keratitis 10.3 APOH F5
24 pregnancy loss, recurrent 1 10.3 APOH F5
25 prothrombin deficiency, congenital 10.2 APOH F5
26 fournier gangrene 10.2 CD40LG F3
27 syphilis 10.2 APOH CD40LG
28 carotid artery occlusion 10.2 APOH F5
29 primary thrombocytopenia 10.2 ADAMTS13 CD40LG
30 lymphoma 10.2
31 acquired hemophilia 10.2 F3 F5
32 acquired hemophilia a 10.2 F3 F5
33 hemophilia a 10.2 F3 F5
34 mixed connective tissue disease 10.2 ADAMTS13 APOH
35 acute respiratory distress syndrome 10.2
36 sagittal sinus thrombosis 10.2 F3 F5
37 von willebrand disease, type 2 10.2 ADAMTS13 F3
38 endocarditis 10.1
39 carotid artery dissection 10.1 ADAMTS13 MTHFR
40 porencephaly 10.1 F5 MTHFR
41 grange syndrome 10.1 F3 MTHFR
42 connective tissue disease 10.1 ADAMTS13 APOH CD40LG
43 nephrosclerosis 10.1 ADAMTS13 MTHFR
44 gastroschisis 10.1 F5 MTHFR
45 cerebral sinovenous thrombosis 10.1 APOH F3 F5
46 arteritic anterior ischemic optic neuropathy 10.1 F5 MTHFR
47 peripheral vertigo 10.1 APOH F3 F5
48 factor xii deficiency 10.1 APOH F3 F5
49 hemorrhage, intracerebral 10.0
50 polyarteritis nodosa, childhood-onset 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 Immunoglobulins Phase 2
2 Antibodies, Antiphospholipid Phase 2
3 Antibodies Phase 2
4 Immunologic Factors Phase 2,Phase 1
5
Promethazine Approved, Investigational Phase 1 60-87-7 4927
6
alemtuzumab Approved, Investigational Phase 1 216503-57-0
7
Benzocaine Approved, Investigational Phase 1 94-09-7, 1994-09-7 2337
8
Hydrocortisone Approved, Vet_approved Phase 1 50-23-7 5754
9
tannic acid Approved Phase 1 1401-55-4
10
Hydrocortisone acetate Approved, Vet_approved Phase 1 50-03-3
11
Acetaminophen Approved Phase 1 103-90-2 1983
12
Cyclophosphamide Approved, Investigational Phase 1 6055-19-2, 50-18-0 2907
13
Fludarabine Approved Phase 1 75607-67-9, 21679-14-1 30751
14
Diphenhydramine Approved, Investigational Phase 1 147-24-0, 58-73-1 3100
15
Prednisone Approved, Vet_approved Phase 1 53-03-2 5865
16 Alkylating Agents Phase 1
17 diuretics Phase 1
18 Hydrocortisone hemisuccinate Phase 1
19 Antirheumatic Agents Phase 1
20 Immunosuppressive Agents Phase 1
21 Antineoplastic Agents, Immunological Phase 1
22 Hydrocortisone 17-butyrate 21-propionate Phase 1
23 Antigen-Antibody Complex Phase 1
24 Antineoplastic Agents, Alkylating 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, Liver, Heart, Skin, Endothelial, Bone Marrow, Lung

Publications for Catastrophic Antiphospholipid Syndrome

Articles related to Catastrophic Antiphospholipid Syndrome:

(show top 50) (show all 312)
# 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
Catastrophic antiphospholipid syndrome and pregnancy. Clinical report. ( 29284338 )
2018
9
Catastrophic antiphospholipid syndrome and pregnancy. ( 29179957 )
2018
10
The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review. ( 29779928 )
2018
11
Microthrombotic renal involvement in an SLE patient with concomitant catastrophic antiphospholipid syndrome: the beneficial effect of rituximab treatment. ( 29635999 )
2018
12
Therapeutic apheresis in the treatment of catastrophic antiphospholipid syndrome. ( 29478799 )
2018
13
Rare Presentation of Catastrophic Antiphospholipid Syndrome with Myocarditis in Post-partum Period: Case Report and Review of Literature. ( 29391679 )
2018
14
McMaster RARE-Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome. ( 29978552 )
2018
15
Clinical Report of Probable Catastrophic Antiphospholipid Syndrome in Pregnancy. ( 29808136 )
2018
16
Immunomodulation in the Treatment of Refractory Catastrophic Antiphospholipid Syndrome. ( 29805821 )
2018
17
The role of hydroxychloroquine in catastrophic antiphospholipid syndrome case: Series of two case reports and review of literature. ( 30046446 )
2018
18
Eculizumab for catastrophic antiphospholipid syndrome-a case report and literature review. ( 30101319 )
2018
19
Catastrophic antiphospholipid syndrome: Lessons from 14 cases successfully treated in a single center. A narrative report. ( 30146007 )
2018
20
Eculizumab in a pregnant patient with laboratory onset of catastrophic antiphospholipid syndrome: A case report. ( 30290624 )
2018
21
Bilateral central retinal artery occlusion from catastrophic antiphospholipid syndrome. ( 30420561 )
2018
22
Post-partum catastrophic antiphospholipid syndrome presenting with shock and digital ischaemia - A diagnostic and management challenge. ( 30515245 )
2018
23
Co-existing Bilateral Pulmonary Embolism and Intra-cardiac Mass: A Case of Catastrophic Antiphospholipid Syndrome-like Disease. ( 30546985 )
2018
24
Catastrophic Antiphospholipid Antibody Syndrome After Cobra Bite in a Patient With Systemic Lupus Erythematosus. ( 30585994 )
2018
25
Catastrophic antiphospholipid antibody syndrome presenting as acute vascular occlusion in a young female patient. ( 27745843 )
2018
26
The Wolf Hidden behind the Clots: Catastrophic Antiphospholipid Antibody Syndrome. ( 30123280 )
2018
27
Complement System Abnormalities in Patients with Atypical Hemolytic Uremic Syndrome and Catastrophic Antiphospholipid Syndrome. ( 29308852 )
2017
28
Lupus-Negative Libman-Sacks Endocarditis Complicated by Catastrophic Antiphospholipid Syndrome. ( 28515823 )
2017
29
Catastrophic antiphospholipid syndrome: A case with unusual findings revealed in autopsy and a brief literature update. ( 29308285 )
2017
30
Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology. ( 28829758 )
2017
31
Diagnosis and management of catastrophic antiphospholipid syndrome. ( 28277850 )
2017
32
Catastrophic antiphospholipid syndrome (CAPS)-induced ischemic pancreatic ducts injury mimicking intraductal papillary mucinous neoplasm (IPMN). ( 28802775 )
2017
33
Catastrophic antiphospholipid syndrome: an update. ( 28488841 )
2017
34
Catastrophic Antiphospholipid Syndrome: Candidate Therapies for a Potentially Lethal Disease. ( 28099080 )
2017
35
Probable Catastrophic Antiphospholipid Syndrome with Intracerebral Hemorrhage Secondary to Epstein-Barr Viral Infection. ( 28357636 )
2017
36
Catastrophic antiphospholipid syndrome on switching from warfarin to rivaroxaban. ( 28319823 )
2017
37
The catastrophic antiphospholipid syndrome in children. ( 28632503 )
2017
38
Catastrophic antiphospholipid syndrome treated with rituximab: A case report. ( 28638691 )
2017
39
Successful Treatment of Catastrophic Antiphospholipid Antibody Syndrome Associated with MALT Lymphoma by Autologous Hematopoietic Stem Cell Transplantation. ( 28502938 )
2017
40
Catastrophic antiphospholipid antibody syndrome presenting as severe ischaemic colitis. ( 27939948 )
2017
41
Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies. ( 27380476 )
2016
42
Plasma Exchange in the Management of Catastrophic Antiphospholipid Syndrome. ( 27833765 )
2016
43
Hepatic infarction in a pregnant woman with antiphospholipid syndrome and triple antibody positivity: A case report focusing on catastrophic antiphospholipid syndrome. ( 27668204 )
2016
44
Cutaneous gangrene of the arms and legs after cardiopulmonary resuscitation: A rare presentation of catastrophic antiphospholipid syndrome. ( 27396539 )
2016
45
Catastrophic antiphospholipid syndrome: The current management approach. ( 27886797 )
2016
46
Catastrophic Antiphospholipid Syndrome Triggered by Fulminant Chikungunya Infection in a Patient With Systemic Lupus Erythematosus. ( 26748818 )
2016
47
Catastrophic antiphospholipid syndrome in pregnancy, a diagnosis that should not be missed. ( 27023104 )
2016
48
Complement activation is a crucial pathogenic factor in catastrophic antiphospholipid syndrome. ( 27105662 )
2016
49
Uterine Artery Embolization Resulting in Catastrophic Antiphospholipid Syndrome in a Patient with Primary Antiphospholipid Syndrome. ( 27106650 )
2016
50
Probable catastrophic antiphospholipid syndrome (CAPS) in case of SLE. ( 27728684 )
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
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31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
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46 MESH via Orphanet
47 MGI
50 NCI
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55 NINDS
56 Novoseek
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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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