MCID: ACQ012
MIFTS: 44

Acquired Angioedema

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

Aliases & Classifications for Acquired Angioedema

MalaCards integrated aliases for Acquired Angioedema:

Name: Acquired Angioedema 53 73
Acquired C1 Inhibitor Deficiency 53
Angioedema, Acquired 53

Classifications:



Summaries for Acquired Angioedema

NIH Rare Diseases : 53 Acquired angioedema (AAE) is a rare disorder that causes recurrent episodes of swelling (edema) of the face or body, lasting several days. People with AAE may have swelling of the face, lips, tongue, limbs, or genitals. People with AAE can have edema of the lining of the digestive tract, which can cause abdominal pain and nausea, as well as edema of the upper airway, which can be life-threatening. Swelling episodes may have various triggers, such as mild trauma (such as dental work), viral illness, cold exposure, pregnancy, certain foods, or emotional stress. The frequency of episodes is unpredictable and can vary widely. There are two forms of AAE. Type 1 is associated with various other diseases including lymphoproliferative disorders, and autoimmune diseases that may not become apparent until years after the angioedema begins. Type 2 is associated with an autoimmune abnormality in which a person has autoantibodies against a protein in the blood called C1-INH. In some cases, it is hard to distinguish between AAE types 1 and 2.   Treatment options depend on the severity of symptoms, the parts of the body affected, and the type of AAE a person has. Various medications may be used to relieve symptoms or prevent complications. When an underlying disease is present, episodes may stop if the underlying disease is treated, but some people continue to experience episodes despite treatment. During severe or life-threatening episodes, intensive support may be needed (such as IV fluids or intubation for a blocked airway).

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 SERPING1 (Serpin Family G Member 1), and among its related pathways/superpathways are Creation of C4 and C2 activators and Complement Pathway. The drugs Complement C1 Inactivator Proteins and Complement C1 Inhibitor Protein have been mentioned in the context of this disorder. Affiliated tissues include tongue, b cells and testes, and related phenotypes are facial edema and urticaria

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 64)
# Related Disease Score Top Affiliating Genes
1 angioedema 31.1 C1S SERPING1
2 c1 inhibitor deficiency 30.7 C1S SERPING1
3 hereditary angioedema 30.5 C1S SERPING1
4 systemic lupus erythematosus 28.3 C1S C3 CRP
5 anemia, autoimmune hemolytic 28.3 C1S C3 HP
6 acquired angioedema type 2 12.2
7 acquired angioedema type 1 12.1
8 angioedema induced by ace inhibitors 11.2
9 louse-borne relapsing fever 10.3 CRP SERPING1
10 hydrops, lactic acidosis, and sideroblastic anemia 10.3 C1S SERPING1
11 mediastinitis 10.2 CRP F5
12 peripheral vertigo 10.1 CRP F5
13 complement deficiency 10.1 C3 SERPING1
14 arteritic anterior ischemic optic neuropathy 10.1 CRP F5
15 acute maxillary sinusitis 10.1 C3 CRP
16 complement component 4, partial deficiency of 10.1
17 lymphoma 10.1
18 hypersensitivity vasculitis 10.0 C3 CRP
19 hypersensitivity reaction type iii disease 10.0 C3 CRP
20 lemierre's syndrome 10.0 C3 CRP
21 homocysteinemia 10.0 CRP F5
22 pulmonary embolism 9.9 CRP F5
23 leukemia, chronic lymphocytic 2 9.9
24 leukemia, chronic lymphocytic 9.9
25 type i 9.9
26 leukemia, b-cell, chronic 9.9
27 afibrinogenemia 9.9 C3 F5
28 lyme disease 9.9 C3 CRP
29 autoimmune lymphoproliferative syndrome, type v 9.8
30 leukemia 9.8
31 melancholia 9.8 CRP HP
32 cerebrovascular disease 9.8 CRP F5
33 priapism 9.8 F5 HP
34 dysentery 9.7 CRP HP
35 dirofilariasis 9.7 CRP HP
36 skeletal tuberculosis 9.7 CRP HP
37 blepharochalasis and double lip 9.7
38 thrombophilia due to thrombin defect 9.7
39 myelofibrosis 9.7
40 helicobacter pylori infection 9.7
41 psoriatic arthritis 9.7
42 antithrombin iii deficiency 9.7
43 anaplastic large cell lymphoma 9.7
44 arthritis 9.7
45 b-cell lymphomas 9.7
46 hemolytic anemia 9.7
47 hepatitis 9.7
48 marginal zone b-cell lymphoma 9.7
49 sinusitis 9.7
50 splenic marginal zone 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:

32 (show all 8)
# Description HPO Frequency HPO Source Accession
1 facial edema 32 occasional (7.5%) HP:0000282
2 urticaria 32 occasional (7.5%) HP:0001025
3 ascites 32 occasional (7.5%) HP:0001541
4 abdominal pain 32 hallmark (90%) HP:0002027
5 intestinal obstruction 32 occasional (7.5%) HP:0005214
6 intestinal edema 32 occasional (7.5%) HP:0005225
7 laryngeal edema 32 occasional (7.5%) HP:0012027
8 angioedema 32 hallmark (90%) HP:0100665

MGI Mouse Phenotypes related to Acquired Angioedema:

46
# 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,Phase 3
2 Complement C1 Inhibitor Protein Phase 2, Phase 3,Phase 3
3 Complement Inactivating Agents Phase 2, Phase 3,Phase 3
4 Complement System Proteins Phase 2, Phase 3,Phase 3
5 Immunosuppressive Agents Phase 2, Phase 3,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
4 Epidemiological Analysis for Hereditary Angioedema Disease (EHA Study) Not yet recruiting NCT03558009

Search NIH Clinical Center for Acquired Angioedema

Genetic Tests for Acquired Angioedema

Anatomical Context for Acquired Angioedema

MalaCards organs/tissues related to Acquired Angioedema:

41
Tongue, B Cells, Testes, Skin, Colon

Publications for Acquired Angioedema

Articles related to Acquired Angioedema:

(show top 50) (show all 80)
# Title Authors Year
1
Acquired angioedema in juvenile systemic lupus erythematosus: case-based review. ( 29951963 )
2018
2
Case report presenting the diagnostic challenges in a patient with recurrent acquired angioedema, antiphospholipid antibodies and undetectable C2 levels. ( 29881401 )
2018
3
Acquired Angioedema With Anti-C1-inhibitor Autoantibodies During Assisted Reproduction Techniques. ( 29461216 )
2018
4
Atypical presentation of acquired angioedema. ( 29554170 )
2018
5
Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes. ( 29393169 )
2018
6
Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency. ( 29545957 )
2018
7
Idiopathic Nonhistaminergic Acquired Angioedema Versus Hereditary Angioedema. ( 29715562 )
2018
8
Acquired Angioedema. ( 28377018 )
2017
9
Idiopathic non-histaminergic acquired angioedema: a case series and discussion of published clinical trials. ( 28861213 )
2017
10
Perioperative course in patients with hereditary or acquired angioedema. ( 27687418 )
2016
11
A case of acquired angioedema possibly associated with adenocarcinoma of the colon. ( 27026513 )
2016
12
Comparing acquired angioedema with hereditary angioedema (type I/II): Findings from the Icatibant Outcome Survey. ( 27936514 )
2016
13
Rituximab in a patient with splenic marginal zone lymphoma and acquired angioedema. ( 27017557 )
2016
14
Shortened Activated Partial Thromboplastin Time May Help in Diagnosing Hereditary and Acquired Angioedema. ( 27463190 )
2016
15
Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema? ( 26478549 )
2015
16
The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity. ( 26068904 )
2015
17
A UK national audit of hereditary and acquired angioedema. ( 23786259 )
2014
18
Acquired angioedema with C1 inhibitor deficiency secondary to splenic marginal zone B-cell lymphoma. ( 24970460 )
2014
19
Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema. ( 25506230 )
2014
20
New perspectives in acquired angioedema. ( 24979839 )
2014
21
Use of recombinant C1 inhibitor in patients with resistant or frequent attacks of hereditary or acquired angioedema. ( 24556385 )
2014
22
An evidence based therapeutic approach to hereditary and acquired angioedema. ( 24983294 )
2014
23
Acquired angioedema--occurrence, clinical features and associated disorders in a Danish nationwide patient cohort. ( 23921495 )
2013
24
Acquired angioedema with raised serum tryptase. ( 23244664 )
2013
25
A case of multicentric Castleman's disease in HIV infection with the rare complication of acquired angioedema. ( 24352125 )
2013
26
A patient with chronic lymphocytic leukemia and acquired angioedema: correlation of clinical and biochemical response to CLL therapy. ( 23441138 )
2013
27
International consensus on hereditary and acquired angioedema. ( 23176876 )
2012
28
Acquired angioedema and marginal zone lymphoma. ( 22508826 )
2012
29
Chapter 22: Hereditary and acquired angioedema. ( 22794695 )
2012
30
Successful treatment of an acute attack of acquired angioedema with the bradykinin-B2-receptor antagonist icatibant. ( 20477917 )
2011
31
"Surgical" abdomen in a patient with chronic lymphocytic leukemia: a case of acquired angioedema. ( 21997434 )
2011
32
Update on laboratory tests for the diagnosis and differentiation of hereditary angioedema and acquired angioedema. ( 22195757 )
2011
33
Acquired angioedema with C1 inhibitor deficiency associated with anticardiolipin antibodies. ( 22230421 )
2011
34
Acquired angioedema responding to rituximab. ( 21691703 )
2011
35
Treatment of acquired angioedema with icatibant: a case report. ( 20680518 )
2011
36
Rapid resolution of erythema marginatum after icatibant in acquired angioedema. ( 21279303 )
2011
37
Optimal medical management of acute attacks of acquired angioedema. ( 20042567 )
2010
38
Acquired angioedema: Autoantibody associations and C1q utility as a diagnostic tool. ( 20929611 )
2010
39
Successful use of etanercept in acquired angioedema in a patient with psoriatic arthritis. ( 20040647 )
2010
40
Acquired angioedema. ( 20667117 )
2010
41
Acquired angioedema associated with hereditary angioedema due to C1 inhibitor deficiency. ( 18447143 )
2008
42
Acquired angioedema associated with primary antiphospholipid syndrome in a patient with antithrombin III deficiency. ( 18204284 )
2008
43
Acquired angioedema in non-Hodgkin's lymphoma. ( 17317233 )
2007
44
Acute upper airway obstruction from acquired angioedema. ( 17305664 )
2007
45
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
46
Rituximab-induced elimination of acquired angioedema due to C1-inhibitor deficiency. ( 16887400 )
2006
47
Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. ( 16630950 )
2006
48
Skin venous thromboembolism by combined oral contraceptive in a woman with acquired angioedema and Factor V Leiden mutation. ( 16472576 )
2006
49
Acquired angioedema secondary to hormone replacement therapy. ( 16272680 )
2005
50
Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. ( 15356535 )
2004

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 9.65 C1S C3 CRP HP SERPING1
2 extracellular region GO:0005576 9.63 C1S C3 CRP F5 HP SERPING1
3 extracellular space GO:0005615 9.43 C1S C3 CRP F5 HP SERPING1
4 platelet alpha granule lumen GO:0031093 9.32 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:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 proteolysis GO:0006508 9.67 C1S C3 HP
2 immune system process GO:0002376 9.61 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 innate immune response GO:0045087 9.46 C1S C3 CRP SERPING1
6 complement activation GO:0006956 9.4 C1S C3
7 hemostasis GO:0007599 9.37 F5 SERPING1
8 blood circulation GO:0008015 9.32 F5 SERPING1
9 acute-phase response GO:0006953 9.26 CRP HP
10 regulation of complement activation GO:0030449 9.13 C1S C3 SERPING1
11 complement activation, classical pathway GO:0006958 8.92 C1S C3 CRP 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
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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