MCID: CTS005
MIFTS: 52

Catastrophic Antiphospholipid Syndrome malady

Genetic diseases, Rare diseases, Immune diseases, Blood diseases categories

Summaries for Catastrophic Antiphospholipid Syndrome

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66Wikipedia, 34MalaCards
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Wikipedia:66 Catastrophic antiphospholipid syndrome, CAPS, also known as Asherson?s syndrome, is an acute and complex... more...

MalaCards: Catastrophic Antiphospholipid Syndrome, also known as thrombotic storm, is related to antiphospholipid syndrome and lupus erythematosus. An important gene associated with Catastrophic Antiphospholipid Syndrome is HLA-DRB4 (major histocompatibility complex, class II, DR beta 4), and among its related pathways are Selected targets of HNF1 and Collagen biosynthesis and modifying enzymes. The compounds CL(22:6(4Z,7Z,10Z,13Z,16Z,19Z)/18:2(9Z,12Z)/18:2(9Z,12Z)/18:1(11Z)) and ticlopidine have been mentioned in the context of this disorder. Affiliated tissues include liver, bone and skin, and related mouse phenotypes are nervous system and cardiovascular system.

Aliases & Classifications for Catastrophic Antiphospholipid Syndrome

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44NIH Rare Diseases, 21GeneTests
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Genetic diseases, Rare diseases
Anatomical: Immune diseases, Blood diseases


Aliases & Descriptions:

catastrophic antiphospholipid syndrome 44
thrombotic storm 44 21
catastrophic antiphospholipid antibody syndrome 44


Related Diseases for Catastrophic Antiphospholipid Syndrome

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18GeneCards, 19GeneDecks
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Diseases related to Catastrophic Antiphospholipid Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 134)
idRelated DiseaseScoreTop Affiliating Genes
1antiphospholipid syndrome31.6CD40LG, F5, F8, MTHFR, F3, ADAMTS13
2lupus erythematosus30.8CD40LG, F5, F3, APOH
3systemic lupus erythematosus30.7CFH, APOH, ADAMTS13, F3, F8, CD40LG
4purpura fulminans30.5PROC, F5
5purpura30.5ADAMTS13
6sepsis30.4SERPINE1, ADAMTS13, PROC, F3, F5
7hemolytic anemia30.3CFH, APOH, ADAMTS13, F3
8septic shock30.2F5, F3, SERPINE1
9rheumatoid arthritis30.2CD40LG, MTHFR, APOH, HLA-DRB4
10hypertension30.2F5, MTHFR, SERPINE1
11pulmonary embolism30.2APOH, F3, MTHFR, F5
12hellp syndrome30.1APOH, SERPINE1, ADAMTS13, MTHFR, F5
13disseminated intravascular coagulation30.1SERPINE1, ADAMTS13, PROC, F3, F8, F5
14thrombocytopenia30.0CFH, APOH, ADAMTS13, F3, F8, F5
15protein s deficiency30.0F5, F8, MTHFR, F3, SERPINE1, APOH
16thromboembolism30.0F8, MTHFR, F3, PROC, SERPINE1, APOH
17pancreatitis10.4
18polyarteritis nodosa10.3
19typhoid fever10.3
20endocarditis10.3
21glomerulonephritis10.3
22endotheliitis10.3
23cardiogenic shock10.3
24splenic marginal zone lymphoma10.3
25leukemia10.3
26acute myelomonocytic leukemia10.3
27pediatric systemic lupus erythematosus10.3
28graft versus host disease10.2
29acute interstitial pneumonia10.2
30invasive aspergillosis10.2
31choroiditis10.2
32adult respiratory distress syndrome10.2
33cerebritis10.2
34proliferative glomerulonephritis10.2
35pancytopenia10.2
36sclerosing cholangitis10.2
37adult-onset still's disease10.2
38down syndrome10.2
39crescentic glomerulonephritis10.2
40arthritis10.2
41aspergillosis10.2
42cholangitis10.2
43dilated cardiomyopathy10.2
44hepatitis10.2
45herpes simplex10.2
46influenza10.2
47pneumonia10.2
48retinitis10.2
49bone marrow necrosis10.2
50chronic graft versus host disease10.2

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



Diseases related to catastrophic antiphospholipid syndrome

Symptoms for Catastrophic Antiphospholipid Syndrome

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Drugs & Therapeutics for Catastrophic Antiphospholipid Syndrome

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6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Search CenterWatch for Catastrophic Antiphospholipid Syndrome

Genetic Tests for Catastrophic Antiphospholipid Syndrome

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21GeneTests
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Genetic tests related to Catastrophic Antiphospholipid Syndrome:

id Genetic test Affiliating Genes
1 Thrombotic Storm21

Anatomical Context for Catastrophic Antiphospholipid Syndrome

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34MalaCards
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MalaCards organs/tissues related to Catastrophic Antiphospholipid Syndrome:

34
Liver, Bone, Skin, Bone marrow, Heart, Endothelial, Lung, Breast

Animal Models for Catastrophic Antiphospholipid Syndrome or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Catastrophic Antiphospholipid Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00036317.9CD40LG, F5, MTHFR, F3, PROC, CFH
2MP:00053857.6CFH, SERPINE1, PROC, F3, F5, CD40LG
3MP:00053977.5CFH, SERPINE1, ADAMTS13, PROC, F3, F8
4MP:00053877.4CFH, SERPINE1, ADAMTS13, PROC, F3, F8
5MP:00107717.4SERPINE1, F3, MTHFR, F5, CD40LG
6MP:00053766.2CD40LG, CFH, APOH, SERPINE1, ADAMTS13, PROC
7MP:00107686.1CFH, APOH, SERPINE1, ADAMTS13, PROC, F3

Publications for Catastrophic Antiphospholipid Syndrome

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53PubMed
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Articles related to Catastrophic Antiphospholipid Syndrome:

(show top 50)    (show all 194)
idTitleAuthorsYear
1
A case of catastrophic antiphospholipid syndrome, which presented an acute interstitial pneumonia-like image on chest CT scan. (23553659)
2013
2
Rituximab use in the catastrophic antiphospholipid syndrome: Descriptive analysis of the CAPS registry patients receiving rituximab. (23777822)
2013
3
Catastrophic antiphospholipid syndrome and pregnancy: an experience of 13 cases. (23676524)
2013
4
A 13-year-old girl with pancytopenia. Catastrophic antiphospholipid syndrome. (23556525)
2013
5
Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations. (23722230)
2013
6
The Hyperferritinemic Syndrome: macrophage activation syndrome, Still's disease, septic shock and catastrophic antiphospholipid syndrome. (23968282)
2013
7
Catastrophic antiphospholipid syndrome. (23278947)
2013
8
Vascular endothelial cell function in catastrophic antiphospholipid syndrome: a case report and review of the literature. (23762673)
2013
9
N2010 adult-onset Still's disease complicated by hemophagocytic syndrome and catastrophic antiphospholipid syndrome resulting in four limb amputation. (23781758)
2013
10
Relapsing catastrophic antiphospholipid syndrome potential role of microangiopathic hemolytic anemia in disease relapses. (22784445)
2013
11
Neurosurgical management for complicated catastrophic antiphospholipid syndrome. (24269552)
2013
12
8th International Congress on Autoimmunity: new perspectives for refractory catastrophic antiphospholipid syndrome. (23078059)
2012
13
Catastrophic antiphospholipid syndrome in a patient with V Leiden variant. (23319991)
2012
14
Severe abdominal pain as a presenting symptom of probable catastrophic antiphospholipid syndrome. (22711721)
2012
15
Multiple thromboemboli associated to two occult tumors: a case mimicking catastrophic antiphospholipid syndrome. (21459648)
2011
16
Eculizumab and renal transplantation in a patient with catastrophic antiphospholipid syndrome: effect of heparin on complement activation. (21335395)
2011
17
Catastrophic antiphospholipid syndrome (CAPS): update from the 'CAPS Registry'. (20353979)
2010
18
Reversible cardiac valvular disease in catastrophic antiphospholipid syndrome. (20508270)
2010
19
Infant with probable catastrophic antiphospholipid syndrome successfully managed with rituximab. (20478943)
2010
20
Catastrophic antiphospholipid syndrome complicated by cardiogenic shock - a case report]. (19650000)
2009
21
Massive cerebrovascular infarct due to the catastrophic antiphospholipid syndrome in a patient with idiopathic thrombocytopenic purpura. (19617232)
2009
22
Catastrophic antiphospholipid syndrome and rituximab: a new report]. (20187361)
2009
23
Catastrophic antiphospholipid syndrome: treatment, prognosis, and the risk of relapse. (19051065)
2009
24
Catastrophic antiphospholipid syndrome presenting as ischemic pancreatitis in systemic lupus erythematosus. (19734640)
2009
25
The catastrophic antiphospholipid syndrome in Serbia: diagnostic and management problems. (19048415)
2009
26
Catastrophic antiphospholipid syndrome in a patient with Down syndrome. (19762387)
2009
27
Relapsing catastrophic antiphospholipid syndrome: report of three cases. (17977582)
2008
28
A case of catastrophic antiphospholipid syndrome presenting with acute respiratory distress syndrome as the initial manifestation. (18651145)
2008
29
Reversible posterior leukoencephalopathy syndrome in catastrophic antiphospholipid syndrome. (17565204)
2007
30
Catastrophic antiphospholipid syndrome related to severe ovarian hyperstimulation. (16538387)
2007
31
Catastrophic antiphospholipid syndrome associated with malignancies (case report and review of the literature). (17283588)
2007
32
Severe limb necrosis: primary thrombotic microangiopathy or "seronegative" catastrophic antiphospholipid syndrome? A diagnostic dilemma. (17256103)
2007
33
Delayed catastrophic antiphospholipid syndrome after massive cerebral infarction. (17565449)
2007
34
Catastrophic antiphospholipid syndrome presenting with renal thrombotic microangiopathy and diffuse proliferative glomerulonephritis. (16539830)
2006
35
Diffuse large and small bowel necrosis in catastrophic antiphospholipid syndrome. (16894316)
2006
36
Catastrophic antiphospholipid syndrome: a rare cause of disseminated microvascular thrombotic injury - a case report with pathological and molecular correlative studies. (15743323)
2005
37
Refractory leg infection as an inducer of the catastrophic antiphospholipid syndrome. (15249331)
2004
38
Liver involvement in catastrophic antiphospholipid syndrome. (15168783)
2004
39
Combined plasmapheresis and immunosuppression as rescue treatment of a patient with catastrophic antiphospholipid syndrome occurring despite anticoagulation: a case report. (12945882)
2003
40
Catastrophic antiphospholipid syndrome: proposed guidelines for diagnosis and treatment. (17041346)
2002
41
Catastrophic antiphospholipid syndrome associated with typhoid fever: comment on the article by Hayem et al. (11920434)
2002
42
Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. (11704713)
2001
43
Catastrophic antiphospholipid syndrome. (10961585)
2000
44
Pathophysiology of the catastrophic antiphospholipid syndrome (CAPS). (10996834)
2000
45
Systemic lupus erythematosus-associated catastrophic antiphospholipid syndrome occurring after typhoid fever: a possible role of Salmonella lipopolysaccharide in the occurrence of diffuse vasculopathy-coagulopathy. (10323464)
1999
46
Membranous obstruction of the inferior vena cava and extensive bone marrow necrosis associated with catastrophic antiphospholipid syndrome. (19078409)
1999
47
The pathogenesis of catastrophic antiphospholipid syndrome. (19078402)
1999
48
Anti beta 2-glycoprotein I antibodies in a patient with catastrophic antiphospholipid syndrome. (8608682)
1995
49
On the catastrophic antiphospholipid syndrome. A comment on the editorial by AlarcA^n-Segovia. (8070172)
1994
50
Catastrophic antiphospholipid syndrome (CAS): a rare manifestation of the antiphospholipid antibody syndrome. (8299260)
1993

Variations for Catastrophic Antiphospholipid Syndrome

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Expression for genes affiliated with Catastrophic Antiphospholipid Syndrome

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Catastrophic Antiphospholipid Syndrome

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

Pathways for genes affiliated with Catastrophic Antiphospholipid Syndrome

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51PathCards, 13EMD Millipore, 56Reactome, 54QIAGEN, 39NCBI BioSystems Database, 55R&D Systems, 31KEGG
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Compounds for genes affiliated with Catastrophic Antiphospholipid Syndrome

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25HMDB, 46Novoseek, 12DrugBank, 52PharmGKB, 30IUPHAR, 62Tocris Bioscience
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Compounds related to Catastrophic Antiphospholipid Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 207)
idCompoundScoreTop Affiliating Genes
1CL(22:6(4Z,7Z,10Z,13Z,16Z,19Z)/18:2(9Z,12Z)/18:2(9Z,12Z)/18:1(11Z))259.8CRLS1, APOH
2ticlopidine46 1210.8APOH, ADAMTS13, F3
3CL(20:4(8Z,11Z,14Z,17Z)/20:3(8Z,11Z,14Z)/18:2(9Z,12Z)/18:2(9Z,12Z))259.8APOH, CRLS1
4CL(20:4(5Z,8Z,11Z,14Z)/20:1(11Z)/18:2(9Z,12Z)/18:2(9Z,12Z))259.7APOH, CRLS1
5CL(20:1(11Z)/18:2(9Z,12Z)/18:1(11Z)/18:1(11Z))259.7APOH, CRLS1
6CL(18:1(11Z)/18:1(11Z)/18:1(11Z)/18:1(11Z))259.7APOH, CRLS1
7coumarins469.6F5, F3, APOH
8gamma-carboxyglutamic acid469.6PROC, F3, F5
9CL(18:2(9Z,12Z)/18:2(9Z,12Z)/18:1(9Z)/16:0)259.6APOH, CRLS1
10anisodamine469.5SERPINE1, F3
11cacl2469.4F3, F8, F5
12rituximab46 52 1211.4APOH, ADAMTS13, F8, CD40LG
13fluorescein46 1210.4F3, F5, CD40LG
14phosphatidylethanolamine46 1210.4APOH, F8, F5
15CL(18:2(9Z,12Z)/18:2(9Z,12Z)/18:1(11Z)/18:1(9Z))259.4APOH, CRLS1
16dextran sulfate469.3CD40LG, F5, F3, APOH
17kaolin469.2F5, F8, F3, APOH
18ristocetin469.2ADAMTS13, F3, F8, F5
19tranexamic acid46 1210.2SERPINE1, F3, F8
20vitamin b12469.1CD40LG, F5, MTHFR, APOH
21nacl469.0F5, F8, ADAMTS13, CFH
22folate469.0CD40LG, F5, MTHFR, APOH
23p00001468.9SERPINE1, MTHFR, F8
24phospholipid468.9F5, F8, F3, PROC, APOH
25phosphatidylserine46 30 1210.8APOH, F3, F8, F5, CD40LG
26kininogen468.8APOH, SERPINE1, F3, F5
27polysaccharide468.8CFH, F3, F8, F5, CD40LG
28cyclosporin a46 30 6210.7CFH, ADAMTS13, MTHFR, F8, CD40LG
29tacrolimus46 52 1210.6F5, F3, SERPINE1, CFH
30uric acid46 259.6F5, MTHFR, SERPINE1, APOH
31prostacyclin468.4F5, F8, F3, SERPINE1, APOH
32desmopressin46 62 30 1211.4SERPINE1, ADAMTS13, F3, F8, F5
33warfarin46 52 25 1211.3APOH, PROC, F3, MTHFR, F8, F5
34hirudin468.3SERPINE1, F3, F8, F5, CD40LG
35alanine468.1CFH, F3, MTHFR, F8, F5, CD40LG
36cysteine468.0CFH, F3, MTHFR, F8, F5, CD40LG
37cardiolipin46 129.0CD40LG, F5, CRLS1, F8, F3, APOH
38vegf468.0APOH, SERPINE1, F3, F8, F5, CD40LG
39cholesterol46 30 25 1210.8CFH, APOH, SERPINE1, MTHFR, F8, F5
40homocysteine46 258.8APOH, SERPINE1, F3, MTHFR, F8, F5
41endotoxin467.7APOH, SERPINE1, ADAMTS13, F3, F8, F5
42estrogen467.7APOH, SERPINE1, MTHFR, F8, F5, CD40LG
43retinoic acid46 258.7CFH, SERPINE1, F3, MTHFR, F5, CD40LG
44aspartate467.7SERPINE1, F3, MTHFR, F8, F5, CD40LG
45cyclophosphamide46 52 129.5APOH, SERPINE1, ADAMTS13, F3, MTHFR, F8
46aspirin46 52 30 2510.4APOH, SERPINE1, F3, MTHFR, F8, F5
47creatinine467.1APOH, SERPINE1, ADAMTS13, F3, MTHFR, F8
48serine466.7CFH, SERPINE1, ADAMTS13, PROC, F3, MTHFR
49heparin46 30 25 129.5CD40LG, F5, F8, MTHFR, F3, PROC
50fibrinogen466.5PROC, ADAMTS13, SERPINE1, APOH, CFH, F3

GO Terms for genes affiliated with Catastrophic Antiphospholipid Syndrome

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17Gene Ontology
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Cellular components related to Catastrophic Antiphospholipid Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular matrixGO:0310128.9APOH, SERPINE1, F3
2platelet alpha granule lumenGO:0310938.7F5, F8, SERPINE1
3extracellular regionGO:0055768.0F5, F8, PROC, SERPINE1, CFH
4plasma membraneGO:0058867.0HLA-DRB4, SERPINE1, PROC, F3, F8, F5
5extracellular spaceGO:0056157.0CD40LG, CFH, APOH, SERPINE1, ADAMTS13, F3

Biological processes related to Catastrophic Antiphospholipid Syndrome according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1blood coagulation, intrinsic pathwayGO:0075979.4F8, APOH
2negative regulation of fibrinolysisGO:0519189.4APOH, SERPINE1
3blood circulationGO:0080159.3F5, MTHFR
4positive regulation of blood coagulationGO:0301949.3SERPINE1, APOH
5negative regulation of blood coagulationGO:0301959.2APOH, SERPINE1, PROC
6platelet degranulationGO:0025768.7F5, F8, SERPINE1
7platelet activationGO:0301688.2CD40LG, F5, F8, ADAMTS13, SERPINE1
8blood coagulationGO:0075968.1SERPINE1, PROC, F3, F8, F5

Molecular functions related to Catastrophic Antiphospholipid Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1serine-type endopeptidase activityGO:0042529.2F5, F8, PROC
2protein bindingGO:0055156.4F5, HLA-DRB4, CFH, APOH, SERPINE1, ADAMTS13

Products for genes affiliated with Catastrophic Antiphospholipid Syndrome

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Sources for Catastrophic Antiphospholipid Syndrome

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet