CAPS
MCID: CTS005
MIFTS: 44

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 53 59 73
Catastrophic Antiphospholipid Antibody Syndrome 53
Cryopyrin-Associated Periodic Syndromes 73
Thrombotic Storm 53
Catastrophic Aps 59
Caps 59

Classifications:



External Ids:

Orphanet 59 ORPHA464343
ICD10 via Orphanet 34 D68.6

Summaries for Catastrophic Antiphospholipid Syndrome

NIH Rare Diseases : 53 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 Antibodies, Antiphospholipid and Immunologic Factors have been mentioned in the context of this disorder. Affiliated tissues include bone, liver and bone marrow, and related phenotypes are homeostasis/metabolism and hematopoietic system

Wikipedia : 76 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 163)
# Related Disease Score Top Affiliating Genes
1 antiphospholipid syndrome 31.1 ADAMTS13 APOH CD40LG F3 F5 MTHFR
2 heparin-induced thrombocytopenia 30.5 CD40LG F3
3 thrombocytopenia 30.4 ADAMTS13 APOH F3
4 systemic lupus erythematosus 30.3 ADAMTS13 APOH CD40LG CFH F3
5 hepatic infarction 30.1 APOH F3 MTHFR
6 purpura fulminans 30.0 F3 PROC
7 hemolytic-uremic syndrome 29.9 ADAMTS13 CFH F3
8 thrombotic thrombocytopenic purpura 29.9 ADAMTS13 APOH CFH F3
9 branch retinal artery occlusion 29.8 APOH F3 MTHFR
10 coronary thrombosis 29.7 F3 SERPINE1
11 protein s deficiency 29.6 APOH F3 F5 MTHFR
12 purpura 29.6 ADAMTS13 APOH F3 PROC
13 disseminated intravascular coagulation 29.4 ADAMTS13 F3 F5 SERPINE1
14 pulmonary embolism 29.4 APOH F3 F5 MTHFR SERPINE1
15 retinal artery occlusion 29.4 APOH F5 MTHFR SERPINE1
16 thrombosis 29.2 ADAMTS13 APOH F3 F5 MTHFR PROC
17 stroke, ischemic 29.0 ADAMTS13 APOH F3 F5 MTHFR SERPINE1
18 hellp syndrome 28.8 ADAMTS13 APOH CFH F3 F5 MTHFR
19 lupus erythematosus 10.3
20 erythema infectiosum 10.2 APOH CD40LG
21 fournier gangrene 10.2 CD40LG F3
22 thrombotic thrombocytopenic purpura, congenital 10.2 ADAMTS13 CFH
23 may-thurner syndrome 10.2 APOH F5
24 acanthamoeba keratitis 10.2 APOH F5
25 pregnancy loss, recurrent 1 10.2 APOH F5
26 syphilis 10.2 APOH CD40LG
27 prothrombin deficiency, congenital 10.2 APOH F5
28 primary thrombocytopenia 10.2 ADAMTS13 CD40LG
29 acute respiratory distress syndrome 10.2
30 carotid artery occlusion 10.2 APOH F5
31 acquired hemophilia 10.2 F3 F5
32 acquired hemophilia a 10.2 F3 F5
33 hemorrhagic fever 10.2 CD40LG F3
34 hemophilia a 10.2 F3 F5
35 sagittal sinus thrombosis 10.1 F3 F5
36 von willebrand disease, type 2 10.1 ADAMTS13 F3
37 mixed connective tissue disease 10.1 ADAMTS13 APOH
38 endocarditis 10.1
39 lymphoma 10.1
40 connective tissue disease 10.1 ADAMTS13 APOH CD40LG
41 porencephaly 10.1 F5 MTHFR
42 cerebral sinovenous thrombosis 10.1 APOH F3 F5
43 grange syndrome 10.1 F3 MTHFR
44 gastroschisis 10.1 F5 MTHFR
45 carotid artery dissection 10.1 ADAMTS13 MTHFR
46 peripheral vertigo 10.1 APOH F3 F5
47 factor xii deficiency 10.1 APOH F3 F5
48 nephrosclerosis 10.1 ADAMTS13 MTHFR
49 arteritic anterior ischemic optic neuropathy 10.1 F5 MTHFR
50 factor x deficiency 10.0 F3 F5

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:

46
# 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 23)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Antibodies, Antiphospholipid Phase 2
2 Immunologic Factors Phase 2,Phase 1
3 Immunoglobulins Phase 2
4 Antibodies Phase 2
5
Cyclophosphamide Approved, Investigational Phase 1 6055-19-2, 50-18-0 2907
6
Fludarabine Approved Phase 1 75607-67-9, 21679-14-1 30751
7
Promethazine Approved, Investigational Phase 1 60-87-7 4927
8
Prednisone Approved, Vet_approved Phase 1 53-03-2 5865
9
Hydrocortisone Approved, Vet_approved Phase 1 50-23-7 5754
10
Benzocaine Approved, Investigational Phase 1 94-09-7, 1994-09-7 2337
11
Acetaminophen Approved Phase 1 103-90-2 1983
12
Diphenhydramine Approved, Investigational Phase 1 147-24-0, 58-73-1 3100
13 tannic acid Approved Phase 1
14
Hydrocortisone acetate Approved, Vet_approved Phase 1 50-03-3
15
alemtuzumab Approved, Investigational Phase 1 216503-57-0
16 Antineoplastic Agents, Alkylating Phase 1
17 Alkylating Agents Phase 1
18 Antirheumatic Agents Phase 1
19 Hydrocortisone 17-butyrate 21-propionate Phase 1
20 Antigen-Antibody Complex Phase 1
21 diuretics Phase 1
22 Immunosuppressive Agents Phase 1
23 Cortisol succinate 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 73 / NDF-RT 51 :


Genetic Tests for Catastrophic Antiphospholipid Syndrome

Anatomical Context for Catastrophic Antiphospholipid Syndrome

MalaCards organs/tissues related to Catastrophic Antiphospholipid Syndrome:

41
Bone, Liver, Bone Marrow, Kidney, Spinal Cord, Myeloid, Placenta

Publications for Catastrophic Antiphospholipid Syndrome

Articles related to Catastrophic Antiphospholipid Syndrome:

(show top 50) (show all 304)
# Title Authors Year
1
Catastrophic antiphospholipid syndrome and pregnancy. Clinical report. ( 29284338 )
2018
2
Catastrophic antiphospholipid syndrome and pregnancy. ( 29179957 )
2018
3
The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review. ( 29779928 )
2018
4
Microthrombotic renal involvement in an SLE patient with concomitant catastrophic antiphospholipid syndrome: the beneficial effect of rituximab treatment. ( 29635999 )
2018
5
Therapeutic apheresis in the treatment of catastrophic antiphospholipid syndrome. ( 29478799 )
2018
6
Rare Presentation of Catastrophic Antiphospholipid Syndrome with Myocarditis in Post-partum Period: Case Report and Review of Literature. ( 29391679 )
2018
7
McMaster RARE-Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome. ( 29978552 )
2018
8
Clinical Report of Probable Catastrophic Antiphospholipid Syndrome in Pregnancy. ( 29808136 )
2018
9
Immunomodulation in the Treatment of Refractory Catastrophic Antiphospholipid Syndrome. ( 29805821 )
2018
10
Eculizumab in a pregnant patient with laboratory onset of catastrophic antiphospholipid syndrome: A case report. ( 30290624 )
2018
11
Bilateral central retinal artery occlusion from catastrophic antiphospholipid syndrome. ( 30420561 )
2018
12
Post-partum catastrophic antiphospholipid syndrome presenting with shock and digital ischaemia - A diagnostic and management challenge. ( 30515245 )
2018
13
Co-existing Bilateral Pulmonary Embolism and Intra-cardiac Mass: A Case of Catastrophic Antiphospholipid Syndrome-like Disease. ( 30546985 )
2018
14
The role of hydroxychloroquine in catastrophic antiphospholipid syndrome case: Series of two case reports and review of literature. ( 30046446 )
2018
15
Eculizumab for catastrophic antiphospholipid syndrome-a case report and literature review. ( 30101319 )
2018
16
Catastrophic antiphospholipid syndrome: Lessons from 14 cases successfully treated in a single center. A narrative report. ( 30146007 )
2018
17
Catastrophic antiphospholipid antibody syndrome presenting as acute vascular occlusion in a young female patient. ( 27745843 )
2018
18
The Wolf Hidden behind the Clots: Catastrophic Antiphospholipid Antibody Syndrome. ( 30123280 )
2018
19
Complement System Abnormalities in Patients with Atypical Hemolytic Uremic Syndrome and Catastrophic Antiphospholipid Syndrome. ( 29308852 )
2017
20
Lupus-Negative Libman-Sacks Endocarditis Complicated by Catastrophic Antiphospholipid Syndrome. ( 28515823 )
2017
21
Catastrophic antiphospholipid syndrome: A case with unusual findings revealed in autopsy and a brief literature update. ( 29308285 )
2017
22
Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology. ( 28829758 )
2017
23
Diagnosis and management of catastrophic antiphospholipid syndrome. ( 28277850 )
2017
24
Catastrophic antiphospholipid syndrome (CAPS)-induced ischemic pancreatic ducts injury mimicking intraductal papillary mucinous neoplasm (IPMN). ( 28802775 )
2017
25
Catastrophic antiphospholipid syndrome: an update. ( 28488841 )
2017
26
Catastrophic Antiphospholipid Syndrome: Candidate Therapies for a Potentially Lethal Disease. ( 28099080 )
2017
27
Probable Catastrophic Antiphospholipid Syndrome with Intracerebral Hemorrhage Secondary to Epstein-Barr Viral Infection. ( 28357636 )
2017
28
Catastrophic antiphospholipid syndrome on switching from warfarin to rivaroxaban. ( 28319823 )
2017
29
The catastrophic antiphospholipid syndrome in children. ( 28632503 )
2017
30
Catastrophic antiphospholipid syndrome treated with rituximab: A case report. ( 28638691 )
2017
31
Successful Treatment of Catastrophic Antiphospholipid Antibody Syndrome Associated with MALT Lymphoma by Autologous Hematopoietic Stem Cell Transplantation. ( 28502938 )
2017
32
Catastrophic antiphospholipid antibody syndrome presenting as severe ischaemic colitis. ( 27939948 )
2017
33
Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies. ( 27380476 )
2016
34
Plasma Exchange in the Management of Catastrophic Antiphospholipid Syndrome. ( 27833765 )
2016
35
Hepatic infarction in a pregnant woman with antiphospholipid syndrome and triple antibody positivity: A case report focusing on catastrophic antiphospholipid syndrome. ( 27668204 )
2016
36
Cutaneous gangrene of the arms and legs after cardiopulmonary resuscitation: A rare presentation of catastrophic antiphospholipid syndrome. ( 27396539 )
2016
37
Catastrophic antiphospholipid syndrome: The current management approach. ( 27886797 )
2016
38
Catastrophic Antiphospholipid Syndrome Triggered by Fulminant Chikungunya Infection in a Patient With Systemic Lupus Erythematosus. ( 26748818 )
2016
39
Catastrophic antiphospholipid syndrome in pregnancy, a diagnosis that should not be missed. ( 27023104 )
2016
40
Complement activation is a crucial pathogenic factor in catastrophic antiphospholipid syndrome. ( 27105662 )
2016
41
Uterine Artery Embolization Resulting in Catastrophic Antiphospholipid Syndrome in a Patient with Primary Antiphospholipid Syndrome. ( 27106650 )
2016
42
Probable catastrophic antiphospholipid syndrome (CAPS) in case of SLE. ( 27728684 )
2016
43
Catastrophic antiphospholipid syndrome after cardiac surgery. ( 27455170 )
2016
44
Treatment of catastrophic antiphospholipid syndrome. ( 26927441 )
2016
45
Catastrophic Antiphospholipid Syndrome. ( 27375916 )
2016
46
Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry. ( 27639837 )
2016
47
Seronegative catastrophic antiphospholipid syndrome in a young female presenting with a headache. ( 25559391 )
2015
48
A case of catastrophic antiphospholipid syndrome: first report with advanced cardiac imaging using MRI. ( 26014099 )
2015
49
Atypical Subacute Recurrence of Catastrophic Antiphospholipid Syndrome in a Japanese Female Patient. ( 26568011 )
2015
50
1272: CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME COMPLICATED BY TAKOTSUBO CARDIOMYOPATHY. ( 26570933 )
2015

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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
Content
Loading form....