CAPS
MCID: CTS005
MIFTS: 42

Catastrophic Antiphospholipid Syndrome (CAPS) malady

Categories: Rare diseases, Blood diseases, Immune diseases

Aliases & Classifications for Catastrophic Antiphospholipid Syndrome

Aliases & Descriptions for Catastrophic Antiphospholipid Syndrome:

Name: Catastrophic Antiphospholipid Syndrome 50 56 69
Catastrophic Antiphospholipid Antibody Syndrome 50
Cryopyrin-Associated Periodic Syndromes 69
Thrombotic Storm 50
Catastrophic Aps 56
Caps 56

Classifications:



External Ids:

Orphanet 56 ORPHA464343
ICD10 via Orphanet 34 D68.6

Summaries for Catastrophic Antiphospholipid Syndrome

NIH Rare Diseases : 50 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 gene mutations (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 central retinal artery occlusion 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 Immunoglobulins and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include liver, bone and heart, and related phenotypes are homeostasis/metabolism and hematopoietic system

Wikipedia : 71 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 160)
id Related Disease Score Top Affiliating Genes
1 central retinal artery occlusion 29.4 APOH CD40LG F3 MTHFR
2 antiphospholipid syndrome 10.9
3 med13l syndrome 10.3 APOH F5
4 achard thiers syndrome 10.3 APOH F5
5 mixed epithelial tumor of ovary 10.2 MTHFR PROC
6 multiple familial trichoepithelioma 10.2 F5 MTHFR
7 stomatocytosis i 10.2 ADAMTS13 CFH
8 myosclerosis, congenital 10.2 F5 MTHFR
9 photosensitive epilepsy 10.2 F5 MTHFR
10 dmd-related dilated cardiomyopathy 10.2 F3 PROC
11 acrodysplasia with ossification abnormalities, short stature and fibular hypoplasia 10.2 F3 F5
12 pancreatic ductal carcinoma 10.2 F3 F5
13 acrodysplasia scoliosis 10.2 F3 F5
14 myeloid leukemia 10.2 APOH CD40LG
15 stroke, ischemic 10.2 F3 F5
16 exhibitionism 10.2 APOH CD40LG
17 intermediate coronary syndrome 10.2 APOH CD40LG
18 bladder hepatoid adenocarcinoma 10.2 APOH F5
19 vascular erectile tumor 10.1 APOH F5 MTHFR
20 supraumbilical midabdominal raphe and facial cavernous hemangiomas 10.1 APOH F5 MTHFR
21 lung cancer 10.1 APOH F5 MTHFR
22 left-right axis malformations 10.1 APOH F5 MTHFR
23 tonsillar fossa cancer 10.1 ADAMTS13 CD40LG
24 cryptosporidiosis 10.1 APOH F5 MTHFR
25 angular blepharoconjunctivitis 10.1 APOH F5 MTHFR
26 fletcher factor deficiency 10.1 F3 F5
27 ophthalmia neonatorum 10.1 APOH F5 MTHFR
28 dentine erosion 10.1 APOH F5 MTHFR
29 obstructive jaundice 10.1 APOH F5 MTHFR
30 spinal cord lipoma 10.1 APOH F3 MTHFR
31 vaginal discharge 10.1 F5 MTHFR PROC
32 tracheal disease 10.1 ADAMTS13 APOH F3
33 ductal carcinoma in situ 10.1 F3 MTHFR PROC
34 myotonic dystrophy 10.1 F3 F5
35 hendra virus infection 10.1 APOH F3 F5
36 systemic lupus erythematosus 10.1
37 lupus erythematosus 10.1
38 neuronopathy, distal hereditary motor, type iid 10.1 APOH F3 F5
39 freiberg's disease 10.1 CD40LG F3
40 hereditary endotheliopathy, retinopathy, nephropathy, and stroke 10.1 CD40LG F3
41 intestinal disaccharidase deficiency 10.1 APOH F3 F5
42 houlston ironton temple syndrome 10.1 F5 MTHFR
43 pregnancy loss, recurrent 2 10.1 APOH F3 F5
44 atrophic nonflaccid tympanic membrane 10.1 ADAMTS13 F3 F5
45 sporotrichosis 10.1 CD40LG F3
46 chronic inflammatory demyelinating polyneuritis 10.1 CD40LG F3
47 severe nonproliferative diabetic retinopathy 10.1 ADAMTS13 CD40LG
48 stt3a-cdg and stt3b-cdg 10.0 F5 SERPINE1
49 clivus meningioma 10.0 ADAMTS13 APOH CD40LG
50 thrombosis 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:

44
id 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 CD40LG CFH F3 F5 MTHFR PROC

Drugs & Therapeutics for Catastrophic Antiphospholipid Syndrome

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

(show all 22)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1 Immunoglobulins Phase 2
2 Antibodies Phase 2
3 Antibodies, Antiphospholipid 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 5754 657311
8
Diphenhydramine Approved Phase 1 58-73-1, 147-24-0 3100
9
alemtuzumab Approved, Investigational Phase 1 216503-57-0
10
Benzocaine Approved Phase 1 1994-09-7, 94-09-7 2337
11
Promethazine Approved 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 Immunosuppressive Agents Phase 1
20 Antigen-Antibody Complex Phase 1
21 Antirheumatic Agents Phase 1
22 Antineoplastic Agents, Alkylating Phase 1

Interventional clinical trials:


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

Search NIH Clinical Center for Catastrophic Antiphospholipid Syndrome

Genetic Tests for Catastrophic Antiphospholipid Syndrome

Anatomical Context for Catastrophic Antiphospholipid Syndrome

MalaCards organs/tissues related to Catastrophic Antiphospholipid Syndrome:

39
Liver, Bone, Heart, Endothelial, Skin, Bone Marrow, Lung

Publications for Catastrophic Antiphospholipid Syndrome

Articles related to Catastrophic Antiphospholipid Syndrome:

(show top 50) (show all 245)
id Title Authors Year
1
Plasma Exchange in the Management of Catastrophic Antiphospholipid Syndrome. ( 27833765 )
2016
2
Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry. ( 27639837 )
2016
3
Catastrophic antiphospholipid syndrome in pregnancy, a diagnosis that should not be missed. ( 27023104 )
2016
4
Treatment of catastrophic antiphospholipid syndrome. ( 26927441 )
2016
5
Hepatic infarction in a pregnant woman with antiphospholipid syndrome and triple antibody positivity: A case report focusing on catastrophic antiphospholipid syndrome. ( 27668204 )
2016
6
Cutaneous gangrene of the arms and legs after cardiopulmonary resuscitation: A rare presentation of catastrophic antiphospholipid syndrome. ( 27396539 )
2016
7
Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies. ( 27380476 )
2016
8
Catastrophic antiphospholipid syndrome after cardiac surgery. ( 27455170 )
2016
9
Complement activation is a crucial pathogenic factor in catastrophic antiphospholipid syndrome. ( 27105662 )
2016
10
Catastrophic antiphospholipid syndrome: The current management approach. ( 27886797 )
2016
11
Uterine Artery Embolization Resulting in Catastrophic Antiphospholipid Syndrome in a Patient with Primary Antiphospholipid Syndrome. ( 27106650 )
2016
12
Probable catastrophic antiphospholipid syndrome (CAPS) in case of SLE. ( 27728684 )
2016
13
Catastrophic Antiphospholipid Syndrome. ( 27375916 )
2016
14
Catastrophic Antiphospholipid Syndrome Triggered by Fulminant Chikungunya Infection in a Patient With Systemic Lupus Erythematosus. ( 26748818 )
2016
15
Atypical Subacute Recurrence of Catastrophic Antiphospholipid Syndrome in a Japanese Female Patient. ( 26568011 )
2015
16
Catastrophic Antiphospholipid syndrome in postpartum simulating peritonitis; a diagnostic trap. ( 26367418 )
2015
17
Hyperferritinemic syndrome: Still's disease and catastrophic antiphospholipid syndrome triggered by fulminant Chikungunya infection: a case report of two patients. ( 26233722 )
2015
18
A case of catastrophic antiphospholipid syndrome: first report with advanced cardiac imaging using MRI. ( 26014099 )
2015
19
Catastrophic antiphospholipid syndrome presenting as bilateral central retinal artery occlusions. ( 25722904 )
2015
20
Catastrophic antiphospholipid syndrome (CAPS): A rare and fatal encounter. ( 25983439 )
2015
21
Catastrophic Antiphospholipid Syndrome: Scintigraphic Demonstration With Correlated Cross-Sectional Imaging. ( 26447383 )
2015
22
Back from the Brink: Catastrophic Antiphospholipid Syndrome. ( 25596523 )
2015
23
Eculizumab Induces Sustained Remission in a Patient With Refractory Primary Catastrophic Antiphospholipid Syndrome. ( 26267719 )
2015
24
Seronegative catastrophic antiphospholipid syndrome in a young female presenting with a headache. ( 25559391 )
2015
25
The role of infectious diseases in the catastrophic antiphospholipid syndrome. ( 26209907 )
2015
26
1272: CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME COMPLICATED BY TAKOTSUBO CARDIOMYOPATHY. ( 26570933 )
2015
27
Recurrent thrombosis prevention with intravenous immunoglobulin and hydroxychloroquine during pregnancy in a patient with history of catastrophic antiphospholipid syndrome and pregnancy loss. ( 24549974 )
2014
28
CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME ASSOCIATED WITH SYSTEMIC LUPUS ERYTHEMATOSUS TREATED WITH RITUXIMAB: CASE REPORT AND A REVIEW OF THE LITERATURE. ( 25782914 )
2014
29
Catastrophic antiphospholipid syndrome and heparin-induced thrombocytopenia presenting with adrenal insufficiency caused by bilateral hemorrhagic adrenal infarction during sepsis. ( 24709482 )
2014
30
Catastrophic antiphospholipid syndrome: case reports and review of the literature. ( 25508516 )
2014
31
Multiple osteonecrotic lesions mimicking a piano keyboard in the spine of a patient with catastrophic antiphospholipid syndrome. ( 24838647 )
2014
32
All that seems sepsis is not sepsis: However, is this case report really a case of catastrophic antiphospholipid syndrome? ( 24701076 )
2014
33
Catastrophic antiphospholipid syndrome presenting with bilateral massive adrenal haemorrhage. A case report. ( 25553836 )
2014
34
Malignancies: a possible "first hit" in the development of catastrophic antiphospholipid syndrome? ( 25351019 )
2014
35
Catastrophic antiphospholipid syndrome: a clinical review. ( 24644537 )
2014
36
Efficacy of eculizumab in a patient with immunoadsorption-dependent catastrophic antiphospholipid syndrome: a case report. ( 25474424 )
2014
37
14th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome. ( 24657970 )
2014
38
36-year-old female with catastrophic antiphospholipid syndrome treated with eculizumab: a case report and review of literature. ( 25389502 )
2014
39
Catastrophic antiphospholipid syndrome presenting with pulmonary hemorrhage: case report. ( 24894406 )
2014
40
Catastrophic antiphospholipid syndrome: task force report summary. ( 25228727 )
2014
41
Catastrophic antiphospholipid syndrome: an orchestra with several musicians. ( 25351020 )
2014
42
Catastrophic antiphospholipid syndrome in cancer patients: an Interaction of clotting, autoimmunity and tumor growth? ( 25351010 )
2014
43
Cutaneous necrosis of lower extremity as the first manifestation of catastrophic antiphospholipid syndrome. ( 24533540 )
2014
44
A case of catastrophic antiphospholipid syndrome, which presented an acute interstitial pneumonia-like image on chest CT scan. ( 24533549 )
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
Catastrophic antiphospholipid syndrome: case reports and review of the literature. ( 25470889 )
2014
48
Catastrophic antiphospholipid syndrome and heparin-induced thrombocytopenia-related diseases or chance association? ( 25304013 )
2014
49
Recurrent episodes of hemorrhagic alveolitis in relapsing catastrophic antiphospholipid syndrome: the same side of the dark moon. ( 24362787 )
2013
50
Vascular endothelial cell function in catastrophic antiphospholipid syndrome: a case report and review of the literature. ( 23762673 )
2013

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:

id 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:

id Name GO ID Score Top Affiliating Genes
1 platelet degranulation GO:0002576 9.58 APOH F5 SERPINE1
2 platelet activation GO:0030168 9.5 ADAMTS13 CD40LG F5
3 blood coagulation GO:0007596 9.46 ADAMTS13 F3 F5 PROC
4 positive regulation of blood coagulation GO:0030194 9.37 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
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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....