MCID: ACQ012
MIFTS: 38

Acquired Angioedema malady

Genetic diseases, Rare diseases, Bone diseases, Immune diseases, Skin diseases categories

Aliases & Classifications for Acquired Angioedema

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Sources:
45NIH Rare Diseases, 51Orphanet, 65UMLS, 28ICD10 via Orphanet, 37MESH via Orphanet, 66UMLS via Orphanet
See all sources

Aliases & Descriptions for Acquired Angioedema:

Name: Acquired Angioedema 45 51 65
Acquired C1 Inhibitor Deficiency 45 51
Acquired Non Histamine-Induced Angioedema 51
Acquired Bradykinine-Induced Angioedema 51
 
Acquired Angioneurotic Edema 51
Angioedema, Acquired 45
Aae 51


Classifications:



Characteristics (Orphanet epidemiological data):

51
acquired angioedema:
Inheritance: Not applicable; Age of onset: Adult


External Ids:

Orphanet51 91385
ICD10 via Orphanet28 T78.3
MESH via Orphanet37 C538173
UMLS via Orphanet66 C2931758

Summaries for Acquired Angioedema

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NIH Rare Diseases:45 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 systemic lupus erythematosus and angioedema, and has symptoms including urticaria, cutaneous edema and acute abdominal pain/colic. An important gene associated with Acquired Angioedema is XPNPEP2 (X-Prolyl Aminopeptidase (Aminopeptidase P) 2, Membrane-Bound), and among its related pathways are Staphylococcus aureus infection and Pertussis. Affiliated tissues include b cells, tongue and skin, and related mouse phenotype cardiovascular system.

Related Diseases for Acquired Angioedema

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Diseases in the Angioedema family:

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

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

(show top 50)    (show all 75)
idRelated DiseaseScoreTop Affiliating Genes
1systemic lupus erythematosus30.3C1S, C3, CRP
2angioedema11.0
3lupus erythematosus10.6
4c1 inhibitor deficiency10.5
5hereditary angioedema10.4
6acquired angioedema type 110.4
7acquired angioedema type 210.4
8hepatitis10.3
9hepatitis c10.3
10melkersson-rosenthal syndrome10.3
11sjogren's syndrome10.3
12lymphosarcoma10.3
13hepatitis c virus10.2
14angioedema, hereditary, type iii10.2
15angioedema, hereditary, types i and ii10.2
16chronic lymphocytic leukemia10.2
17hepatitis b10.2
18leukemia10.2
19skin conditions10.2
20skin disease10.2
21hepatitis a10.2
22vascular skin disease10.2
23hypersensitivity reaction type ii disease10.2
24hypersensitivity reaction type iii disease10.2
25skin tag10.2
26urticaria10.2
27viral hepatitis10.2
28alopecia antibody deficiency10.2C1S, SERPING1
29mhc class i deficiency10.2C1S, SERPING1
30balanoposthitis10.2C3, SERPING1
31hypercalcemic sarcoidosis10.1C1S, SERPING1
32hypertrichotic osteochondrodysplasia10.1C1S, C3
33complex regional pain syndrome10.1C3, SERPING1
34psoriatic arthritis10.1
35anaplastic large cell lymphoma10.1
36arthritis10.1
37b-cell lymphomas10.1
38hemolytic anemia10.1
39marginal zone b-cell lymphoma10.1
40myelofibrosis10.1
41sinusitis10.1
42antiphospholipid syndrome10.1
43autoimmune hemolytic anemia10.1
44ascher syndrome10.1
45hereditary angioedema type 210.1
46agammaglobulinemia x-linked type 210.1C3, F5
47cicatricial lagophthalmos10.0CRP, SERPING1
48skin glomus tumor10.0F5, HP
49omphalocele10.0CRP, F5
50mediastinum sarcoma10.0CRP, F5

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



Diseases related to acquired angioedema

Symptoms for Acquired Angioedema

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Symptoms:

 51
  • urticaria
  • cutaneous edema
  • acute abdominal pain/colic
  • angioedema/angioneurotic edema
  • intestinal obstruction/ileus
  • severe allergic reaction/atopy

Drugs & Therapeutics for Acquired Angioedema

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Drugs for Acquired Angioedema (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
Desloratadineapproved, investigationalPhase 468100643-71-8124087
Synonyms:
100643-71-8
8-Chloro-11-piperidin-4-ylidene-6,11-dihydro-5H-benzo[5,6]cyclohepta[1,2-b]pyridine
8-Chloro-6,11-dihydro-11-(4-piperidinylidene)-5H-benzo(5,6)cyclohepta(1,2-b)pyridine
8-chloro-11-(piperidin-4-ylidene)-6,11-dihydro-5H-benzo[5,6]cyclohepta[1,2-b]pyridine
8-chloro-6,11-dihydro-11-(4-Piperidinylidene)-5H-benzo(5,6)cyclohepta(1,2-b)pyridine
AC-1279
AC1L3XUD
AKOS000280921
Aerius
Allex
Azomyr
BB_SC-2104
C121345
CHEBI:291342
CHEMBL1172
CID124087
CPD000149358
Claramax
Clarinex
Clarinex (TN)
Clarinex RediTabs
D03693
D3787
DB00967
DESLORATADINE
Denosin
Desalex
Descarboethoxyloratadine
Descarboethoxyoratidine
 
Desloratadine
Desloratadine (USAN/INN)
Desloratadine [USAN]
Desloratidine
Essex brand of desloratadine
HMS2052H05
HMS2090C06
HMS2093F19
I06-1866
L001025
LS-181814
MLS000559042
MLS000759406
MLS001201801
MolPort-000-883-875
NCGC00159325-02
NCGC00159325-03
NCGC00159325-04
NSC675447
Neoclarityn
Opulis
SAM001246545
SMR000149358
STK586537
Sch 34117
Sch-34117
Schering brand of desloratadine
Schering-Plough brand of desloratadine
TL8000065
UNII-FVF865388R
2
Histamine PhosphateapprovedPhase 485251-74-165513
Synonyms:
1H-Imidazole-4-ethanamine, phosphate (1:2)
2-Imidazol-4-ylethylamine orthophosphoric acid (1:2)
4-(2-Aminoethyl)imidazole bis(dihydrogen phosphate)
4-(2-Aminoethyl)imidazole di-acid phosphate
4-2(2-Aminoethyl)Imidazole-Di-Acid Phosphate
51-74-1
53623-99-7
74-56-6
AC1L23E4
CID65513
D04445
DB00667
EINECS 200-118-4
 
H0147
Histamine Positive Skin Test Control
Histamine Positive skin test control
Histamine acid phosphate
Histamine biphosphate
Histamine dihydrogen phosphate
Histamine diphosphate
Histamine phosphate (1:2)
Histamine phosphate (TN)
Histamine phosphate (USP)
Histamine phosphate [USP]
Histamine positive
Histaminum
LS-78569
UNII-QWB37T4WZZ
3
histaminePhase 485275614-87-8, 51-45-6774
Synonyms:
.beta.-Imidazolyl-4-ethylamine
1H-Imidazole-4-ethanamine
1H-Imidazole-5-ethanamine
1avn
1qft
2-(1H-Imidazol-4-yl)ethanamine
2-(1H-Imidazol-4-yl)ethylamine
2-(1H-Imidazol-5-yl)ethanamine
2-(1H-Imidazol-5-yl)ethylamine
2-(1H-imidazol-4-yl)ethan-1-amine
2-(1H-imidazol-5-yl)ethanamine
2-(3H-Imidazol-4-yl)-ethylamine
2-(4-Imidazolyl)ethanamine
2-(4-Imidazolyl)ethylamine
2-Imidazol-4-yl-Ethylamine
2-Imidazol-4-ylethylamine
2-[4-Imidazolyl]ethylamine
4-(2-Aminoethyl)-1H-imidazole
4-(2-Aminoethyl)imidazole
4-Imidazoleethylamine
5-Imidazoleethylamine
51-45-6
53290_FLUKA
64422-25-9
924364-91-0
AC-13185
AC1L19ZT
AC1Q54BV
AC1Q54BW
AKOS000274386
ALBB-005968
BCBcMAP01_000250
BSPBio_001117
BSPBio_002124
Bio1_000487
Bio1_000976
Bio1_001465
Bio2_000389
Bio2_000869
C00388
CCRIS 6535
CHEBI:18295
CHEMBL90
CID774
D08040
DivK1c_000308
EINECS 200-100-6
Eramin
Ergamine
Ergotidine
F411C768-A159-4FC0-A195-291A08BB03AA
Free histamine
H7125_SIGMA
HMS1362G19
HMS1792G19
HMS1990G19
HSDB 3338
HSM
Histamine
Histamine (DCF)
Histamine Base
Histamine [USAN]
Histamine, Free Base
Histaminum
 
Histaminum (TN)
IDI1_000308
IDI1_002144
Imidazole-4-ethylamine
Istamina
Istamina [Italian]
KBio1_000308
KBio2_000457
KBio2_001325
KBio2_003025
KBio2_003893
KBio2_005593
KBio2_006461
KBio3_000853
KBio3_000854
KBio3_001344
KBioGR_000457
KBioGR_001580
KBioSS_000457
KBioSS_001325
L-Histamin base
L-Histamine
L-histamine
L000292
LS-75835
Lopac-H-7250
Lopac0_000595
MLS000069447
MolPort-001-785-597
MolPort-002-042-264
NCGC00015513-01
NCGC00015513-08
NCGC00093371-02
NCGC00093371-03
NCGC00093371-04
NCGC00093371-05
NINDS_000308
NSC 33792
NSC33792
SDCCGMLS-0066601.P001
SMP1_000151
SMR000059091
SPBio_000729
ST073926
STK346752
STOCK5S-55669
Spectrum2_000665
Spectrum3_000452
Spectrum4_000960
Spectrum5_000796
Spectrum_000845
Theramine
UNII-820484N8I3
WLN: T5M CNJ D2Z
ZERO/004089
[3H]histamine
b-Imidazolyl-4-ethylamine
beta-Aminoethylglyoxaline
beta-Aminoethylimidazole
beta-Aminothethylglyoxaline
beta-Imidazolyl-4-ethylamine
beta-aminothethylglyoxaline
bmse000744
histamine
nchembio.87-comp54
nchembio714-comp1
4Complement System ProteinsPhase 2, Phase 3171

Interventional clinical trials:

idNameStatusNCT IDPhase
1Desloratadine 5, 10 and 20mg in Patients With Cold UrticariaCompletedNCT01444196Phase 4
2C1-Esteraseremmer-N for the Treatment of Hereditary (and Acquired) AngioedemaCompletedNCT00125541Phase 2, Phase 3
3C1-Esteraseremmer-N for the Treatment of Hereditary (and Acquired) AngioedemaCompletedNCT00125151Phase 3
4Kinetics, Efficacy and Safety of C1-Esteraseremmer-NCompletedNCT00119431Phase 2

Search NIH Clinical Center for Acquired Angioedema

Genetic Tests for Acquired Angioedema

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Anatomical Context for Acquired Angioedema

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MalaCards organs/tissues related to Acquired Angioedema:

33
B cells, Tongue, Skin, Bone, Testes

Animal Models for Acquired Angioedema or affiliated genes

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MGI Mouse Phenotypes related to Acquired Angioedema:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053857.6C3, CRP, F5, HP, SERPING1

Publications for Acquired Angioedema

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Articles related to Acquired Angioedema:

(show top 50)    (show all 66)
idTitleAuthorsYear
1
The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity. (26068904)
2015
2
Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema? (26478549)
2015
3
A UK national audit of hereditary and acquired angioedema. (23786259)
2014
4
Acquired angioedema with C1 inhibitor deficiency secondary to splenic marginal zone B-cell lymphoma. (24970460)
2014
5
Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema. (25506230)
2014
6
A case of multicentric Castleman's disease in HIV infection with the rare complication of acquired angioedema. (24352125)
2013
7
Acquired angioedema with raised serum tryptase. (23244664)
2013
8
Acquired angioedema--occurrence, clinical features and associated disorders in a Danish nationwide patient cohort. (23921495)
2013
9
Chapter 22: Hereditary and acquired angioedema. (22794695)
2012
10
Acquired angioedema and marginal zone lymphoma. (22508826)
2012
11
International consensus on hereditary and acquired angioedema. (23176876)
2012
12
Successful treatment of an acute attack of acquired angioedema with the bradykinin-B2-receptor antagonist icatibant. (20477917)
2011
13
"Surgical" abdomen in a patient with chronic lymphocytic leukemia: a case of acquired angioedema. (21997434)
2011
14
Rapid resolution of erythema marginatum after icatibant in acquired angioedema. (21279303)
2011
15
Acquired angioedema with C1 inhibitor deficiency associated with anticardiolipin antibodies. (22230421)
2011
16
Treatment of acquired angioedema with icatibant: a case report. (20680518)
2011
17
Acquired angioedema: Autoantibody associations and C1q utility as a diagnostic tool. (20929611)
2010
18
Acquired angioedema. (20667117)
2010
19
Successful use of etanercept in acquired angioedema in a patient with psoriatic arthritis. (20040647)
2010
20
Optimal medical management of acute attacks of acquired angioedema. (20042567)
2010
21
Acquired angioedema associated with primary antiphospholipid syndrome in a patient with antithrombin III deficiency. (18204284)
2008
22
Acquired angioedema associated with hereditary angioedema due to C1 inhibitor deficiency. (18447143)
2008
23
Acute upper airway obstruction from acquired angioedema. (17305664)
2007
24
Acquired angioedema in non-Hodgkin's lymphoma. (17317233)
2007
25
Rituximab-induced elimination of acquired angioedema due to C1-inhibitor deficiency. (16887400)
2006
26
Skin venous thromboembolism by combined oral contraceptive in a woman with acquired angioedema and Factor V Leiden mutation. (16472576)
2006
27
Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. (16630950)
2006
28
Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. (15356535)
2004
29
Hereditary and acquired angioedema: experience with patients in Puerto Rico. (15125214)
2004
30
Acquired angioedema: a new target for rituximab? (15339702)
2004
31
Hereditary and acquired angioedema. (15515380)
2004
32
Clinical and biological distinctions between type I and type II acquired angioedema. (14553889)
2003
33
Acquired angioedema and Helicobacter pylori infection in a child. (12898238)
2003
34
Profhylaxis and treatment of hereditary and acquired angioedema at HUB; use of the C1-esterase inhibitor]. (14974880)
2003
35
Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2. (12208377)
2002
36
Anaplastic large cell lymphoma and acquired angioedema: a novel association? (12402660)
2002
37
Protein sgp 120 as a marker of an acquired angioedema. (11029608)
2000
38
Acquired angioedema associated with sinusitis. (10923586)
2000
39
Acquired angioedema associated with chronic hepatitis C. (10200025)
1999
40
Acquired angioedema. (9092748)
1997
41
Activation of the contact system and fibrinolysis in autoimmune acquired angioedema: a rationale for prophylactic use of tranexamic acid. (8182230)
1994
42
Immunoadsorption in acquired angioedema: a therapeutic misadventure. (8453789)
1993
43
Acquired angioedema as the presenting feature of lymphoproliferative disorders of mature B-lymphocytes. (8242559)
1993
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

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Clinvar genetic disease variations for Acquired Angioedema:

5
id Gene Variation Type Significance SNP ID Assembly Location
1XPNPEP2NG_011479.1: g.2846C> Asingle nucleotide variantrisk factorrs3788853GRCh37Chr X, 128870791: 128870791

Expression for genes affiliated with Acquired Angioedema

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Search GEO for disease gene expression data for Acquired Angioedema.

Pathways for genes affiliated with Acquired Angioedema

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GO Terms for genes affiliated with Acquired Angioedema

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Cellular components related to Acquired Angioedema according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granule lumenGO:003109310.1F5, SERPING1
2blood microparticleGO:00725628.7C1S, C3, HP, SERPING1
3extracellular spaceGO:00056157.3C3, CRP, F5, HP, SERPING1
4extracellular exosomeGO:00700627.3C1S, C3, CRP, HP, SERPING1, XPNPEP2
5extracellular regionGO:00055767.0C1S, C3, CRP, F5, HP, SERPING1

Biological processes related to Acquired Angioedema according to GeneCards Suite gene sharing:

(show all 7)
idNameGO IDScoreTop Affiliating Genes
1platelet degranulationGO:000257610.0F5, SERPING1
2blood circulationGO:00080159.8F5, SERPING1
3complement activationGO:00069569.8C1S, C3
4acute-phase responseGO:00069539.6CRP, HP
5complement activation, classical pathwayGO:00069589.4C1S, C3, SERPING1
6proteolysisGO:00065089.0C1S, HP, XPNPEP2
7blood coagulationGO:00075968.9C3, F5, SERPING1

Sources for Acquired Angioedema

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet