MCID: ACQ012
MIFTS: 45

Acquired Angioedema

Categories: Rare diseases, Immune diseases, Bone diseases, Skin diseases, Genetic diseases

Aliases & Classifications for Acquired Angioedema

MalaCards integrated aliases for Acquired Angioedema:

Name: Acquired Angioedema 49 69
Acquired C1 Inhibitor Deficiency 49
Angioedema, Acquired 49

Classifications:



Summaries for Acquired Angioedema

NIH Rare Diseases : 49 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, and has symptoms including facial edema, urticaria and ascites. An important gene associated with Acquired Angioedema is SERPING1 (Serpin Family G Member 1), and among its related pathways/superpathways are Creation of C4 and C2 activators and Complement and coagulation cascades. The drugs Complement C1 Inactivator Proteins and Complement C1 Inhibitor Protein have been mentioned in the context of this disorder. Affiliated tissues include b cells, tongue and testes, and related phenotype is cardiovascular system.

Related Diseases for Acquired Angioedema

Diseases in the Angioedema family:

Angioedema, Hereditary, Type I Angioedema, Hereditary, Type Iii
Hereditary Angioedema Acquired Angioedema
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 59)
# Related Disease Score Top Affiliating Genes
1 angioedema 31.0 C1S SERPING1
2 c1 inhibitor deficiency 30.4 C1S SERPING1
3 hereditary angioedema 30.2 C1S SERPING1
4 anemia, autoimmune hemolytic 29.2 C3 HP
5 acquired angioedema type 2 12.2
6 acquired angioedema type 1 12.1
7 angioedema induced by ace inhibitors 11.2
8 hydrops, lactic acidosis, and sideroblastic anemia 10.2 C1S SERPING1
9 louse-borne relapsing fever 10.2 CRP SERPING1
10 mediastinitis 10.1 CRP F5
11 peripheral vertigo 10.1 CRP F5
12 complement deficiency 10.1 C3 SERPING1
13 complement component 4, partial deficiency of 10.1
14 lymphoma 10.1
15 arteritic anterior ischemic optic neuropathy 10.0 CRP F5
16 acute maxillary sinusitis 10.0 C3 CRP
17 hypersensitivity vasculitis 10.0 C3 CRP
18 lemierre's syndrome 10.0 C3 CRP
19 hypersensitivity reaction type iii disease 10.0 C3 CRP
20 homocysteinemia 10.0 CRP F5
21 pulmonary embolism 9.9 CRP F5
22 afibrinogenemia 9.9 C3 F5
23 lyme disease 9.9 C3 CRP
24 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.9
25 melancholia 9.9 CRP HP
26 cerebrovascular disease 9.9 CRP F5
27 leukemia, chronic lymphocytic 2 9.8
28 leukemia, chronic lymphocytic 9.8
29 autoimmune lymphoproliferative syndrome, type v 9.8
30 leukemia 9.8
31 leukemia, b-cell, chronic 9.8
32 dysentery 9.8 CRP HP
33 dirofilariasis 9.8 CRP HP
34 skeletal tuberculosis 9.8 CRP HP
35 autoimmune disease of blood 9.8 C3 HP
36 glomerulonephritis 9.7 C1S C3
37 hemoglobinuria 9.7 C3 HP
38 blepharochalasis and double lip 9.7
39 thrombophilia due to thrombin defect 9.7
40 complement hyperactivation, angiopathic thrombosis, and protein-losing enteropathy 9.7
41 myelofibrosis 9.7
42 helicobacter pylori infection 9.7
43 psoriatic arthritis 9.7
44 antithrombin iii deficiency 9.7
45 anaplastic large cell lymphoma 9.7
46 arthritis 9.7
47 b-cell lymphomas 9.7
48 hemolytic anemia 9.7
49 hepatitis 9.7
50 marginal zone b-cell lymphoma 9.7

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



Diseases related to Acquired Angioedema

Symptoms & Phenotypes for Acquired Angioedema

Human phenotypes related to Acquired Angioedema:

31 (show all 8)
# Description HPO Frequency HPO Source Accession
1 facial edema 31 occasional (7.5%) HP:0000282
2 urticaria 31 occasional (7.5%) HP:0001025
3 ascites 31 occasional (7.5%) HP:0001541
4 abdominal pain 31 hallmark (90%) HP:0002027
5 intestinal obstruction 31 occasional (7.5%) HP:0005214
6 intestinal edema 31 occasional (7.5%) HP:0005225
7 laryngeal edema 31 occasional (7.5%) HP:0012027
8 angioedema 31 hallmark (90%) HP:0100665

MGI Mouse Phenotypes related to Acquired Angioedema:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.02 C3 CRP F5 HP SERPING1

Drugs & Therapeutics for Acquired Angioedema

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

(show all 6)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Complement C1 Inactivator Proteins Phase 2, Phase 3
2 Complement C1 Inhibitor Protein Phase 2, Phase 3
3 Complement Inactivating Agents Phase 2, Phase 3
4 Complement System Proteins Phase 2, Phase 3
5 Immunosuppressive Agents Phase 2, Phase 3
6 Complement C1s

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 C1-Esteraseremmer-N for the Treatment of Hereditary (and Acquired) Angioedema Completed NCT00125541 Phase 2, Phase 3 C1 inhibitor concentrate (C1-esteraseremmer-N)
2 C1-Esteraseremmer-N for the Treatment of Hereditary (and Acquired) Angioedema Completed NCT00125151 Phase 3 C1 inhibitor concentrate (C1-esteraseremmer-N)
3 Biomarker for Hereditary Angioedema Disease Type 1 Recruiting NCT03029728

Search NIH Clinical Center for Acquired Angioedema

Genetic Tests for Acquired Angioedema

Anatomical Context for Acquired Angioedema

MalaCards organs/tissues related to Acquired Angioedema:

38
B Cells, Tongue, Testes, Skin, Colon

Publications for Acquired Angioedema

Articles related to Acquired Angioedema:

(show top 50) (show all 74)
# Title Authors Year
1
Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes. ( 29393169 )
2018
2
Idiopathic non-histaminergic acquired angioedema: a case series and discussion of published clinical trials. ( 28861213 )
2017
3
Acquired Angioedema. ( 28377018 )
2017
4
Rituximab in a patient with splenic marginal zone lymphoma and acquired angioedema. ( 27017557 )
2016
5
Shortened Activated Partial Thromboplastin Time May Help in Diagnosing Hereditary and Acquired Angioedema. ( 27463190 )
2016
6
Perioperative course in patients with hereditary or acquired angioedema. ( 27687418 )
2016
7
A case of acquired angioedema possibly associated with adenocarcinoma of the colon. ( 27026513 )
2016
8
Comparing acquired angioedema with hereditary angioedema (type I/II): Findings from the Icatibant Outcome Survey. ( 27936514 )
2016
9
Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema? ( 26478549 )
2015
10
The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity. ( 26068904 )
2015
11
An evidence based therapeutic approach to hereditary and acquired angioedema. ( 24983294 )
2014
12
Use of recombinant C1 inhibitor in patients with resistant or frequent attacks of hereditary or acquired angioedema. ( 24556385 )
2014
13
A UK national audit of hereditary and acquired angioedema. ( 23786259 )
2014
14
Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema. ( 25506230 )
2014
15
Acquired angioedema with C1 inhibitor deficiency secondary to splenic marginal zone B-cell lymphoma. ( 24970460 )
2014
16
New perspectives in acquired angioedema. ( 24979839 )
2014
17
A patient with chronic lymphocytic leukemia and acquired angioedema: correlation of clinical and biochemical response to CLL therapy. ( 23441138 )
2013
18
A case of multicentric Castleman's disease in HIV infection with the rare complication of acquired angioedema. ( 24352125 )
2013
19
Acquired angioedema--occurrence, clinical features and associated disorders in a Danish nationwide patient cohort. ( 23921495 )
2013
20
Acquired angioedema with raised serum tryptase. ( 23244664 )
2013
21
Chapter 22: Hereditary and acquired angioedema. ( 22794695 )
2012
22
International consensus on hereditary and acquired angioedema. ( 23176876 )
2012
23
Acquired angioedema and marginal zone lymphoma. ( 22508826 )
2012
24
Successful treatment of an acute attack of acquired angioedema with the bradykinin-B2-receptor antagonist icatibant. ( 20477917 )
2011
25
Update on laboratory tests for the diagnosis and differentiation of hereditary angioedema and acquired angioedema. ( 22195757 )
2011
26
"Surgical" abdomen in a patient with chronic lymphocytic leukemia: a case of acquired angioedema. ( 21997434 )
2011
27
Acquired angioedema responding to rituximab. ( 21691703 )
2011
28
Rapid resolution of erythema marginatum after icatibant in acquired angioedema. ( 21279303 )
2011
29
Treatment of acquired angioedema with icatibant: a case report. ( 20680518 )
2011
30
Acquired angioedema with C1 inhibitor deficiency associated with anticardiolipin antibodies. ( 22230421 )
2011
31
Acquired angioedema: Autoantibody associations and C1q utility as a diagnostic tool. ( 20929611 )
2010
32
Successful use of etanercept in acquired angioedema in a patient with psoriatic arthritis. ( 20040647 )
2010
33
Acquired angioedema. ( 20667117 )
2010
34
Optimal medical management of acute attacks of acquired angioedema. ( 20042567 )
2010
35
Acquired angioedema associated with hereditary angioedema due to C1 inhibitor deficiency. ( 18447143 )
2008
36
Acquired angioedema associated with primary antiphospholipid syndrome in a patient with antithrombin III deficiency. ( 18204284 )
2008
37
Acquired angioedema in non-Hodgkin's lymphoma. ( 17317233 )
2007
38
Acute upper airway obstruction from acquired angioedema. ( 17305664 )
2007
39
Rituximab-induced elimination of acquired angioedema due to C1-inhibitor deficiency. ( 16887400 )
2006
40
Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. ( 16630950 )
2006
41
Skin venous thromboembolism by combined oral contraceptive in a woman with acquired angioedema and Factor V Leiden mutation. ( 16472576 )
2006
42
Deep venous thrombosis associated with acquired angioedema type II in a patient heterozygous for the mutation of factor V Leiden: effective treatment and follow-up for four years. ( 16676085 )
2006
43
Acquired angioedema secondary to hormone replacement therapy. ( 16272680 )
2005
44
Hereditary and acquired angioedema. ( 15515380 )
2004
45
Acquired angioedema: a new target for rituximab? ( 15339702 )
2004
46
Hereditary and acquired angioedema: experience with patients in Puerto Rico. ( 15125214 )
2004
47
Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. ( 15356535 )
2004
48
[Profhylaxis and treatment of hereditary and acquired angioedema at HUB; use of the C1-esterase inhibitor]. ( 14974880 )
2003
49
Acquired angioedema and Helicobacter pylori infection in a child. ( 12898238 )
2003
50
Clinical and biological distinctions between type I and type II acquired angioedema. ( 14553889 )
2003

Variations for Acquired Angioedema

Expression for Acquired Angioedema

Search GEO for disease gene expression data for Acquired Angioedema.

Pathways for Acquired Angioedema

GO Terms for Acquired Angioedema

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

# Name GO ID Score Top Affiliating Genes
1 extracellular exosome GO:0070062 9.72 C1S C3 CRP HP SERPING1
2 extracellular space GO:0005615 9.55 C3 CRP F5 HP SERPING1
3 extracellular region GO:0005576 9.43 C1S C3 CRP F5 HP SERPING1
4 platelet alpha granule lumen GO:0031093 9.26 F5 SERPING1
5 blood microparticle GO:0072562 8.92 C1S C3 HP SERPING1

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

# Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.58 C1S C3 SERPING1
2 immune system process GO:0002376 9.54 C1S C3 SERPING1
3 negative regulation of endopeptidase activity GO:0010951 9.48 C3 SERPING1
4 platelet degranulation GO:0002576 9.46 F5 SERPING1
5 complement activation GO:0006956 9.43 C1S C3
6 hemostasis GO:0007599 9.32 F5 SERPING1
7 blood circulation GO:0008015 9.26 F5 SERPING1
8 acute-phase response GO:0006953 9.16 CRP HP
9 complement activation, classical pathway GO:0006958 9.13 C1S C3 SERPING1
10 regulation of complement activation GO:0030449 8.8 C1S C3 SERPING1

Molecular functions related to Acquired Angioedema according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase activity GO:0004252 8.8 C1S C3 HP

Sources for Acquired Angioedema

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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