MCID: ACQ012
MIFTS: 42

Acquired Angioedema malady

Genetic diseases, Rare diseases, Skin diseases, Bone diseases, Immune diseases categories
Download this MalaCard

Summaries for Acquired Angioedema

About this section


Fully expand this MalaCard
NIH Rare Diseases:42 Acquired angioedema (aae) is a rare condition characterized by recurrent episodes of swelling (edema). affected people may have swelling of the face, lips, tongue, limbs, and genitals. sometimes there is abdominal pain due to edema of the gastrointestinal mucosa, and life-threatening edema of the upper respiratory tract. swelling episodes may be triggered by mild trauma (e.g., dental work), viral illness, cold exposure, pregnancy, ingestion of certain foods, or emotional stress. there are two forms of aae: type i and type ii. aae type i is most commonly associated with b-cell lymphoproliferative disorders and consumption of c1-inh.  aae type ii is an autoimmune condition associated with the presence of an autoantibody against c1-inh. treatment aims to control episodes and normalize c1-inh levels. when possible, the underlying disorder should be treated. last updated: 8/25/2014

MalaCards based summary: Acquired Angioedema, also known as acquired c1 inhibitor deficiency, is related to angioedema and c1 inhibitor deficiency. An important gene associated with Acquired Angioedema is C1S (complement component 1, s subcomponent), and among its related pathways are Pertussis and Complement Pathway. The compounds inogatran and hirugen have been mentioned in the context of this disorder. Affiliated tissues include b cells, tongue and skin.

Aliases & Classifications for Acquired Angioedema

About this section

Acquired Angioedema, Aliases & Descriptions:

Name: Acquired Angioedema 42 62
Acquired C1 Inhibitor Deficiency 42
 
Angioedema, Acquired 42


Classifications:



Related Diseases for Acquired Angioedema

About this section

Diseases in the Hereditary Angioedema Type Iii family:

Hereditary Angioedema Angioedema
acquired angioedema Angioedema, Hereditary, Types I and Ii
Acquired Angioedema Type 1 Acquired Angioedema Type 2

Diseases related to Acquired Angioedema via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 75)
idRelated DiseaseScoreTop Affiliating Genes
1angioedema31.6C1S, SERPING1
2c1 inhibitor deficiency31.3SERPING1
3hereditary angioedema30.7C1S, SERPING1
4lupus erythematosus30.7C1S, SERPINC1, F5
5antithrombin iii deficiency30.2SERPINC1, F5
6antiphospholipid syndrome30.1SERPINC1, F5
7thromboembolism30.1SERPINC1, F5
8venous thrombosis30.1SERPINC1, F5
9systemic lupus erythematosus10.6
10branch retinal artery occlusion10.4F5
11central retinal vein occlusion10.4F5
12acquired angioedema type 210.3
13melkersson-rosenthal syndrome10.3
14sjogren's syndrome10.3
15lymphosarcoma10.3
16sneddon syndrome10.3SERPINC1
17coronary thrombosis10.3SERPINC1
18eclampsia10.3SERPINC1
19chronic lymphocytic leukemia10.2
20leukemia10.2
21angioedema induced by ace inhibitors10.2
22acquired angioedema type 110.2
23capillary leak syndrome10.1C1S, SERPING1
24anaplastic large cell lymphoma10.1
25arthritis10.1
26b-cell lymphomas10.1
27hemolytic anemia10.1
28hepatitis10.1
29hodgkin's lymphoma10.1
30marginal zone b-cell lymphoma10.1
31myelofibrosis10.1
32sinusitis10.1
33hepatitis c10.1
34autoimmune hemolytic anemia10.1
35psoriatic arthritis10.1
36castleman's disease10.1
37venous thromboembolism10.1
38urticaria10.1C1S, SERPING1
39post-thrombotic syndrome10.1SERPINC1, F5
40legg-calve-perthes disease10.1SERPINC1, F5
41prothrombin deficiency10.1F5, SERPINC1
42sagittal sinus thrombosis10.1SERPINC1, F5
43hepatic vein thrombosis10.1SERPINC1, F5
44retinal vein occlusion10.1SERPINC1, F5
45blood protein disease10.1F5, SERPINC1
46portal vein thrombosis10.1SERPINC1, F5
47intracranial thrombosis10.1SERPINC1, F5
48factor xii deficiency10.1SERPINC1, F5
49severe pre-eclampsia10.1SERPINC1, F5
50protein s deficiency10.1SERPINC1, F5

Graphical network of the top 20 diseases related to Acquired Angioedema:



Diseases related to acquired angioedema

Symptoms for Acquired Angioedema

About this section

Drugs & Therapeutics for Acquired Angioedema

About this section

Drug clinical trials:

Search ClinicalTrials for Acquired Angioedema

Search NIH Clinical Center for Acquired Angioedema

Genetic Tests for Acquired Angioedema

About this section

Anatomical Context for Acquired Angioedema

About this section

MalaCards organs/tissues related to Acquired Angioedema:

32
B cells, Tongue, Skin, Testes

Animal Models for Acquired Angioedema or affiliated genes

About this section

Publications for Acquired Angioedema

About this section

Articles related to Acquired Angioedema:

(show top 50)    (show all 63)
idTitleAuthorsYear
1
A UK national audit of hereditary and acquired angioedema. (23786259)
2014
2
Acquired angioedema with C1 inhibitor deficiency secondary to splenic marginal zone B-cell lymphoma. (24970460)
2014
3
A case of multicentric Castleman's disease in HIV infection with the rare complication of acquired angioedema. (24352125)
2013
4
Acquired angioedema with raised serum tryptase. (23244664)
2013
5
Acquired angioedema--occurrence, clinical features and associated disorders in a Danish nationwide patient cohort. (23921495)
2013
6
Chapter 22: Hereditary and acquired angioedema. (22794695)
2012
7
Acquired angioedema and marginal zone lymphoma. (22508826)
2012
8
International consensus on hereditary and acquired angioedema. (23176876)
2012
9
Successful treatment of an acute attack of acquired angioedema with the bradykinin-B2-receptor antagonist icatibant. (20477917)
2011
10
"Surgical" abdomen in a patient with chronic lymphocytic leukemia: a case of acquired angioedema. (21997434)
2011
11
Rapid resolution of erythema marginatum after icatibant in acquired angioedema. (21279303)
2011
12
Acquired angioedema with C1 inhibitor deficiency associated with anticardiolipin antibodies. (22230421)
2011
13
Treatment of acquired angioedema with icatibant: a case report. (20680518)
2011
14
Acquired angioedema: Autoantibody associations and C1q utility as a diagnostic tool. (20929611)
2010
15
Acquired angioedema. (20667117)
2010
16
Successful use of etanercept in acquired angioedema in a patient with psoriatic arthritis. (20040647)
2010
17
Optimal medical management of acute attacks of acquired angioedema. (20042567)
2010
18
Acquired angioedema associated with primary antiphospholipid syndrome in a patient with antithrombin III deficiency. (18204284)
2008
19
Acquired angioedema associated with hereditary angioedema due to C1 inhibitor deficiency. (18447143)
2008
20
Acute upper airway obstruction from acquired angioedema. (17305664)
2007
21
Acquired angioedema in non-Hodgkin's lymphoma. (17317233)
2007
22
Rituximab-induced elimination of acquired angioedema due to C1-inhibitor deficiency. (16887400)
2006
23
Skin venous thromboembolism by combined oral contraceptive in a woman with acquired angioedema and Factor V Leiden mutation. (16472576)
2006
24
Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. (16630950)
2006
25
Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. (15356535)
2004
26
Hereditary and acquired angioedema: experience with patients in Puerto Rico. (15125214)
2004
27
Acquired angioedema: a new target for rituximab? (15339702)
2004
28
Hereditary and acquired angioedema. (15515380)
2004
29
Clinical and biological distinctions between type I and type II acquired angioedema. (14553889)
2003
30
Acquired angioedema and Helicobacter pylori infection in a child. (12898238)
2003
31
Profhylaxis and treatment of hereditary and acquired angioedema at HUB; use of the C1-esterase inhibitor]. (14974880)
2003
32
Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2. (12208377)
2002
33
Anaplastic large cell lymphoma and acquired angioedema: a novel association? (12402660)
2002
34
Polyclonal autoantibodies against C1 inhibitor in a case of acquired angioedema. (12086372)
2002
35
Protein sgp 120 as a marker of an acquired angioedema. (11029608)
2000
36
Acquired angioedema associated with sinusitis. (10923586)
2000
37
Acquired angioedema associated with chronic hepatitis C. (10200025)
1999
38
Hereditary or acquired angioedema caused by functional deficiency of C1 inhibitor--a still unfamiliar disease picture]. (10093876)
1999
39
Acquired angioedema. (9092748)
1997
40
Activation of the contact system and fibrinolysis in autoimmune acquired angioedema: a rationale for prophylactic use of tranexamic acid. (8182230)
1994
41
Immunoadsorption in acquired angioedema: a therapeutic misadventure. (8453789)
1993
42
Acquired angioedema as the presenting feature of lymphoproliferative disorders of mature B-lymphocytes. (8242559)
1993
43
Replacement therapy in hereditary and acquired angioedema. (1409286)
1992
44
C1 inhibitor deficiency: molecular and immunologic basis of hereditary and acquired angioedema. (1921329)
1991
45
Acquired angioedema: observations on the mechanism of action of autoantibodies directed against C1 esterase inhibitor. (2454251)
1988
46
Acquired angioedema. (3203091)
1988
47
Electrophoretic differentiation of acquired angioedema from hereditary angioedema. (3568427)
1987
48
Acquired angioedema and hypocompleentemia in a patient with myelofibrosis. Effect of danazol treatment. (6899952)
1980
49
Acquired angioedema associated with rectal carcinoma and its response to danazol therapy. Acquired angioedema treated with danazol. (701655)
1978
50
Acquired angioedema with lymphoproliferative disorder: association of C1 inhibitor deficiency with cellular abnormality. (1085645)
1976

Variations for Acquired Angioedema

About this section

Expression for genes affiliated with Acquired Angioedema

About this section
Expression patterns in normal tissues for genes affiliated with Acquired Angioedema

Search GEO for disease gene expression data for Acquired Angioedema.

Pathways for genes affiliated with Acquired Angioedema

About this section

Pathways related to Acquired Angioedema according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.3SERPING1, C1S
2
Show member pathways
Immune response Alternative complement pathway60
Immune response Lectin Induced complement pathway60
Immune response Classic complement pathway60
9.3SERPING1, C1S
3
Show member pathways
9.3F5, SERPING1
4
Show member pathways
9.1F5, SERPINC1
5
Show member pathways
8.5F5, SERPINC1, SERPING1
6
Show member pathways
8.5SERPING1, SERPINC1, F5
7
Show member pathways
Complement Activation, Classical Pathway37
Complement and Coagulation Cascades37
8.0F5, SERPINC1, SERPING1, C1S

Compounds for genes affiliated with Acquired Angioedema

About this section

Compounds related to Acquired Angioedema according to GeneCards/GeneDecks:

(show all 47)
idCompoundScoreTop Affiliating Genes
1inogatran449.6SERPINC1, F5
2hirugen449.6F5, SERPINC1
3ecarin449.6F5, SERPINC1
4spectrozyme449.6SERPINC1, F5
5nafamostat mesilate449.6F5, SERPINC1
6danaparoid449.6F5, SERPINC1
7ximelagatran44 1110.5SERPINC1, F5
8ancrod449.5SERPINC1, F5
9heparinoids449.5SERPINC1, F5
10fondaparinux449.5F5, SERPINC1
11bivalirudin44 1110.5SERPINC1, F5
12argatroban44 1110.5F5, SERPINC1
13desogestrel44 1110.5F5, SERPINC1
14gabexate mesilate449.5F5, SERPINC1
15tranexamic acid44 1110.5C1S, SERPINC1
16protamine sulfate449.5SERPINC1, F5
17gestodene44 1110.5F5, SERPINC1
18eltrombopag50 1110.5SERPINC1, F5
19benzamidine44 1110.5F5, SERPINC1
20ristocetin449.5SERPINC1, F5
21tibolone449.5F5, SERPINC1
22levonorgestrel44 61 28 1112.5F5, SERPINC1
23abciximab44 1110.4SERPINC1, C1S
24desmopressin44 61 28 1112.4SERPINC1, F5
25coumarin44 2 50 2412.4SERPINC1, F5
26fluorescein44 1110.4F5, SERPINC1
27hirudin449.4F5, SERPINC1
28protamine449.4SERPINC1, F5
29dermatan sulfate449.4F5, SERPINC1
30raloxifene44 50 28 1112.4F5, SERPINC1
31dextran sulfate449.3SERPINC1, F5
32warfarin44 50 24 1112.3F5, SERPINC1
33progestin449.2SERPINC1, F5
34polyethylene glycol449.1F5, SERPINC1
35folate449.1F5, SERPINC1
36kininogen449.1F5, SERPINC1, C1S
37aprotinin44 1110.0F5, SERPINC1, C1S
38homocysteine44 2410.0SERPINC1, F5
39prostacyclin449.0F5, SERPINC1, C1S
40aspirin44 50 28 2412.0F5, SERPINC1, C1S
41fibrinogen449.0F5, SERPINC1, C1S
42danazol44 24 1111.0SERPINC1, SERPING1, C1S
43citrate448.8F5, SERPINC1
44arginine448.8F5, SERPINC1, C1S
45alanine448.6F5, SERPINC1, SERPING1
46heparin44 28 24 1111.4C1S, SERPING1, SERPINC1, F5
47serine448.3C1S, SERPING1, SERPINC1, F5

GO Terms for genes affiliated with Acquired Angioedema

About this section

Cellular components related to Acquired Angioedema according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granule lumenGO:0310939.0F5, SERPING1
2blood microparticleGO:0725628.5SERPINC1, SERPING1, C1S
3extracellular spaceGO:0056158.5F5, SERPINC1, SERPING1
4extracellular regionGO:0055767.9C1S, SERPING1, SERPINC1, F5

Biological processes related to Acquired Angioedema according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1blood circulationGO:0080159.4F5, SERPING1
2complement activation, classical pathwayGO:0069589.3C1S, SERPING1
3platelet activationGO:0301689.3F5, SERPING1
4platelet degranulationGO:0025769.2F5, SERPING1
5negative regulation of endopeptidase activityGO:0109518.7SERPINC1, SERPING1
6blood coagulationGO:0075968.6F5, SERPINC1, SERPING1

Molecular functions related to Acquired Angioedema according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1serine-type endopeptidase activityGO:0042529.4F5, C1S
2serine-type endopeptidase inhibitor activityGO:0048678.7SERPINC1, SERPING1

Products for genes affiliated with Acquired Angioedema

About this section
  • Antibodies
  • Proteins
  • Lysates

Sources for Acquired Angioedema

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet