MCID: ACQ012
MIFTS: 39

Acquired Angioedema malady

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

Aliases & Classifications for Acquired Angioedema

Aliases & Descriptions for Acquired Angioedema:

Name: Acquired Angioedema 50 69
Acquired C1 Inhibitor Deficiency 50
Angioedema, Acquired 50

Classifications:



Summaries for Acquired Angioedema

NIH Rare Diseases : 50 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 acquired angioedema type 2, and has symptoms including abdominal pain, ascites and intestinal obstruction. 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 Pathway. The drugs Histamine and Loratadine 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 Iii Angioedema, Hereditary, Types I and Ii
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 54)
id Related Disease Score Top Affiliating Genes
1 angioedema 30.9 C1S SERPING1
2 acquired angioedema type 2 12.1
3 acquired angioedema type 1 12.0
4 angioedema induced by ace inhibitors 11.1
5 leukocyte adhesion deficiency, type iii 10.2 C1S SERPING1
6 artemis deficiency 10.2 C1S SERPING1
7 charcot-marie-tooth disease 2a2b 10.1 C1S SERPING1
8 lymphangioma 10.1 C1S SERPING1
9 cicatricial lagophthalmos 10.1 CRP SERPING1
10 mechanical ectropion 10.1 C1S SERPING1
11 parapharyngeal meningioma 10.1 CRP F5
12 meesmann corneal dystrophy 10.1 C1S C3
13 houlston ironton temple syndrome 10.1 CRP F5
14 intestinal disaccharidase deficiency 10.1 CRP F5
15 villous adenoma 10.1 CRP F5
16 chronic mucocutaneous candidiasis 10.1 C3 CRP
17 cyclotropia 10.1 C3 CRP
18 dermatomycosis 10.0 C3 CRP
19 arcus senilis 10.0 C3 CRP
20 leukocyte disease 10.0 CRP F5
21 aids dementia complex 10.0 C3 F5
22 hemoglobin c disease 10.0 CRP HP
23 c1 inhibitor deficiency 10.0
24 lymphoma 10.0
25 lung combined large cell neuroendocrine carcinoma 10.0 HP SERPING1
26 cervix small cell carcinoma 10.0 CRP F5
27 spastic entropion 9.9 CRP HP
28 malignant acth producing neoplasm of pituitary gland 9.9 C1S C3 SERPING1
29 female breast upper-inner quadrant cancer 9.9 CRP HP
30 uterine ligament clear cell adenocarcinoma 9.9 CRP F5
31 hereditary angioedema 9.9
32 camptodactyly-arthropathy-coxa vara-pericarditis syndrome 9.9 C3 CRP SERPING1
33 endometrioid ovary carcinoma 9.9 C3 HP
34 pemphigus vulgaris 9.9 C3 HP
35 chronic lymphocytic leukemia 9.7
36 leukemia 9.7
37 cousin syndrome 9.7 C1S C3 CRP
38 acute proliferative glomerulonephritis 9.7 F5 HP
39 thrombosis 9.6
40 psoriatic arthritis 9.6
41 anaplastic large cell lymphoma 9.6
42 hepatitis c 9.6
43 arthritis 9.6
44 antiphospholipid syndrome 9.6
45 b-cell lymphomas 9.6
46 adenocarcinoma 9.6
47 autoimmune hemolytic anemia 9.6
48 hemolytic anemia 9.6
49 hepatitis 9.6
50 marginal zone b-cell lymphoma 9.6

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:

32 (show all 8)
id Description HPO Frequency HPO Source Accession
1 abdominal pain 32 HP:0002027
2 ascites 32 HP:0001541
3 intestinal obstruction 32 HP:0005214
4 urticaria 32 HP:0001025
5 angioedema 32 HP:0100665
6 facial edema 32 HP:0000282
7 intestinal edema 32 HP:0005225
8 laryngeal edema 32 HP:0012027

MGI Mouse Phenotypes related to Acquired Angioedema:

44
id 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 22)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Histamine Approved, Investigational Phase 4 75614-87-8, 51-45-6 774
2
Loratadine Approved Phase 4 79794-75-5 3957
3
Desloratadine Approved, Investigational Phase 4 100643-71-8 124087
4
Histamine Phosphate Phase 4 51-74-1 65513
5 Cholinergic Agents Phase 4
6 Cholinergic Antagonists Phase 4
7 Neurotransmitter Agents Phase 4
8 Histamine Antagonists Phase 4
9 Histamine H1 Antagonists Phase 4
10 Histamine H1 Antagonists, Non-Sedating Phase 4
11 Dermatologic Agents Phase 4
12 Anti-Allergic Agents Phase 4
13 Antipruritics Phase 4
14 Complement C1 Inactivator Proteins Phase 2, Phase 3
15 Complement C1 Inhibitor Protein Phase 2, Phase 3
16 Complement Inactivating Agents Phase 2, Phase 3
17 Complement System Proteins Phase 2, Phase 3
18 Immunosuppressive Agents Phase 2, Phase 3
19
Bradykinin 58-82-2 439201
20 Complement C1s
21 Vasodilator Agents
22 Kininogens

Interventional clinical trials:


id Name Status NCT ID Phase
1 Desloratadine 5, 10 and 20mg in Patients With Cold Urticaria Completed NCT01444196 Phase 4
2 C1-Esteraseremmer-N for the Treatment of Hereditary (and Acquired) Angioedema Completed NCT00125541 Phase 2, Phase 3
3 C1-Esteraseremmer-N for the Treatment of Hereditary (and Acquired) Angioedema Completed NCT00125151 Phase 3
4 Kinetics, Efficacy and Safety of C1-Esteraseremmer-N Completed NCT00119431 Phase 2
5 Biomarker for Hereditary Angioedema Disease Type 1 Recruiting NCT03029728
6 Determination of Specific Biomarkers of Angioneurotic Crisis Recruiting NCT02833675

Search NIH Clinical Center for Acquired Angioedema

Genetic Tests for Acquired Angioedema

Anatomical Context for Acquired Angioedema

MalaCards organs/tissues related to Acquired Angioedema:

39
B Cells, Tongue, Testes, Skin, Colon

Publications for Acquired Angioedema

Articles related to Acquired Angioedema:

(show top 50) (show all 71)
id Title Authors Year
1
Rituximab in a patient with splenic marginal zone lymphoma and acquired angioedema. ( 27017557 )
2016
2
A case of acquired angioedema possibly associated with adenocarcinoma of the colon. ( 27026513 )
2016
3
Shortened Activated Partial Thromboplastin Time May Help in Diagnosing Hereditary and Acquired Angioedema. ( 27463190 )
2016
4
Perioperative course in patients with hereditary or acquired angioedema. ( 27687418 )
2016
5
Comparing acquired angioedema with hereditary angioedema (type I/II): Findings from the Icatibant Outcome Survey. ( 27936514 )
2016
6
The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity. ( 26068904 )
2015
7
Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema? ( 26478549 )
2015
8
A UK national audit of hereditary and acquired angioedema. ( 23786259 )
2014
9
An evidence based therapeutic approach to hereditary and acquired angioedema. ( 24983294 )
2014
10
Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema. ( 25506230 )
2014
11
Acquired angioedema with C1 inhibitor deficiency secondary to splenic marginal zone B-cell lymphoma. ( 24970460 )
2014
12
Use of recombinant C1 inhibitor in patients with resistant or frequent attacks of hereditary or acquired angioedema. ( 24556385 )
2014
13
New perspectives in acquired angioedema. ( 24979839 )
2014
14
Acquired angioedema--occurrence, clinical features and associated disorders in a Danish nationwide patient cohort. ( 23921495 )
2013
15
A case of multicentric Castleman's disease in HIV infection with the rare complication of acquired angioedema. ( 24352125 )
2013
16
A patient with chronic lymphocytic leukemia and acquired angioedema: correlation of clinical and biochemical response to CLL therapy. ( 23441138 )
2013
17
Acquired angioedema with raised serum tryptase. ( 23244664 )
2013
18
International consensus on hereditary and acquired angioedema. ( 23176876 )
2012
19
Acquired angioedema and marginal zone lymphoma. ( 22508826 )
2012
20
Chapter 22: Hereditary and acquired angioedema. ( 22794695 )
2012
21
Acquired angioedema with C1 inhibitor deficiency associated with anticardiolipin antibodies. ( 22230421 )
2011
22
Treatment of acquired angioedema with icatibant: a case report. ( 20680518 )
2011
23
Successful treatment of an acute attack of acquired angioedema with the bradykinin-B2-receptor antagonist icatibant. ( 20477917 )
2011
24
Acquired angioedema responding to rituximab. ( 21691703 )
2011
25
"Surgical" abdomen in a patient with chronic lymphocytic leukemia: a case of acquired angioedema. ( 21997434 )
2011
26
Update on laboratory tests for the diagnosis and differentiation of hereditary angioedema and acquired angioedema. ( 22195757 )
2011
27
Rapid resolution of erythema marginatum after icatibant in acquired angioedema. ( 21279303 )
2011
28
Successful use of etanercept in acquired angioedema in a patient with psoriatic arthritis. ( 20040647 )
2010
29
Acquired angioedema. ( 20667117 )
2010
30
Acquired angioedema: Autoantibody associations and C1q utility as a diagnostic tool. ( 20929611 )
2010
31
Optimal medical management of acute attacks of acquired angioedema. ( 20042567 )
2010
32
Acquired angioedema associated with primary antiphospholipid syndrome in a patient with antithrombin III deficiency. ( 18204284 )
2008
33
Acquired angioedema associated with hereditary angioedema due to C1 inhibitor deficiency. ( 18447143 )
2008
34
Acute upper airway obstruction from acquired angioedema. ( 17305664 )
2007
35
Acquired angioedema in non-Hodgkin's lymphoma. ( 17317233 )
2007
36
Skin venous thromboembolism by combined oral contraceptive in a woman with acquired angioedema and Factor V Leiden mutation. ( 16472576 )
2006
37
Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. ( 16630950 )
2006
38
Rituximab-induced elimination of acquired angioedema due to C1-inhibitor deficiency. ( 16887400 )
2006
39
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
40
Acquired angioedema secondary to hormone replacement therapy. ( 16272680 )
2005
41
Hereditary and acquired angioedema: experience with patients in Puerto Rico. ( 15125214 )
2004
42
Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. ( 15356535 )
2004
43
Acquired angioedema: a new target for rituximab? ( 15339702 )
2004
44
Hereditary and acquired angioedema. ( 15515380 )
2004
45
Acquired angioedema and Helicobacter pylori infection in a child. ( 12898238 )
2003
46
[Profhylaxis and treatment of hereditary and acquired angioedema at HUB; use of the C1-esterase inhibitor]. ( 14974880 )
2003
47
Clinical and biological distinctions between type I and type II acquired angioedema. ( 14553889 )
2003
48
Polyclonal autoantibodies against C1 inhibitor in a case of acquired angioedema. ( 12086372 )
2002
49
Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2. ( 12208377 )
2002
50
Anaplastic large cell lymphoma and acquired angioedema: a novel association? ( 12402660 )
2002

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:

id 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:

id Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.61 C1S C3 SERPING1
2 immune system process GO:0002376 9.58 C1S C3 SERPING1
3 platelet degranulation GO:0002576 9.48 F5 SERPING1
4 complement activation GO:0006956 9.46 C1S C3
5 proteolysis GO:0006508 9.46 C1S C3 F5 HP
6 hemostasis GO:0007599 9.37 F5 SERPING1
7 blood circulation GO:0008015 9.32 F5 SERPING1
8 acute-phase response GO:0006953 9.26 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:

id Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase activity GO:0004252 8.92 C1S C3 F5 HP

Sources for Acquired Angioedema

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
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