MCID: ACQ012
MIFTS: 45

Acquired Angioedema

Categories: Bone diseases, Gastrointestinal diseases, Genetic diseases, Immune diseases, Oral diseases, Rare 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 System Proteins and Complement C1 Inactivator Proteins have been mentioned in the context of this disorder. Affiliated tissues include tongue, skin and bone, and related phenotypes are ascites and abdominal pain

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 75)
# Related Disease Score Top Affiliating Genes
1 angioedema 31.3 C1S SERPING1
2 c1 inhibitor deficiency 30.6 C1S SERPING1
3 hereditary angioedema 30.4 C1S SERPING1
4 anemia, autoimmune hemolytic 29.4 C1S C3 HP
5 acquired angioedema type 2 12.4
6 acquired angioedema type 1 12.2
7 angioedema induced by ace inhibitors 11.4
8 complement component 4, partial deficiency of 10.3
9 lymphoma 10.2
10 hydrops, lactic acidosis, and sideroblastic anemia 10.1 C1S SERPING1
11 louse-borne relapsing fever 10.1 CRP SERPING1
12 urticaria 10.1 C1S SERPING1
13 melancholia 10.1 CRP HP
14 leukemia, chronic lymphocytic 2 10.0
15 leukemia, chronic lymphocytic 10.0
16 lymphocytic leukemia 10.0
17 leukemia, b-cell, chronic 10.0
18 dysentery 10.0 CRP HP
19 reactive arthritis 10.0 CRP HP
20 mediastinitis 10.0 CRP F5
21 priapism 10.0 F5 HP
22 peripheral vertigo 10.0 CRP F5
23 cholangitis 10.0 CRP HP
24 arteritic anterior ischemic optic neuropathy 10.0 CRP F5
25 homocysteinemia 10.0 CRP F5
26 complement deficiency 10.0 C3 SERPING1
27 pulmonary embolism 10.0 CRP F5
28 rheumatic disease 10.0 C1S CRP
29 melkersson-rosenthal syndrome 10.0
30 sjogren syndrome 10.0
31 lymphosarcoma 10.0
32 acute maxillary sinusitis 9.9 C3 CRP
33 intestinal disease 9.9 CRP F5
34 hypersensitivity vasculitis 9.9 C3 CRP
35 lemierre's syndrome 9.9 C3 CRP
36 hypersensitivity reaction type iii disease 9.9 C3 CRP
37 crohn's disease 9.9 CRP HP
38 henoch-schoenlein purpura 9.9 C3 CRP
39 hellp syndrome 9.9 F5 HP
40 autoimmune disease of musculoskeletal system 9.9 C3 CRP
41 lyme disease 9.9 C3 CRP
42 hemoglobinuria 9.9 C3 HP
43 hemolytic-uremic syndrome 9.9 C3 HP
44 afibrinogenemia 9.9 C3 F5
45 immune system disease 9.9 C3 CRP
46 cerebrovascular disease 9.9 CRP F5
47 deficiency anemia 9.9 CRP HP
48 coronary artery anomaly 9.8 CRP F5 HP
49 vascular disease 9.8 CRP F5 HP
50 blepharochalasis and double lip 9.8

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 ascites 32 occasional (7.5%) HP:0001541
2 abdominal pain 32 hallmark (90%) HP:0002027
3 intestinal obstruction 32 occasional (7.5%) HP:0005214
4 urticaria 32 occasional (7.5%) HP:0001025
5 angioedema 32 hallmark (90%) HP:0100665
6 facial edema 32 occasional (7.5%) HP:0000282
7 intestinal edema 32 occasional (7.5%) HP:0005225
8 laryngeal edema 32 occasional (7.5%) HP:0012027

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 System Proteins Phase 2, Phase 3,Phase 3
2 Complement C1 Inactivator Proteins Phase 2, Phase 3,Phase 3
3 Immunologic Factors Phase 2, Phase 3,Phase 3
4 Complement Inactivating Agents Phase 2, Phase 3,Phase 3
5 Complement C1 Inhibitor Protein Phase 2, Phase 3,Phase 3
6 Immunosuppressive Agents Phase 2, Phase 3,Phase 3

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)

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, Skin, Bone, B Cells, Colon, Testes, Heart

Publications for Acquired Angioedema

Articles related to Acquired Angioedema:

(show top 50) (show all 82)
# 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
Nine year follow-up of a rare case of angioedema due to acquired C1-inhibitor deficiency with late onset and good response to attenuated androgen. ( 30386386 )
2018
9
Splenic marginal zone lymphomas in acquired C1-inhibitor deficiency: clinical and molecular characterization. ( 30073422 )
2018
10
Acquired Angioedema. ( 28377018 )
2017
11
Idiopathic non-histaminergic acquired angioedema: a case series and discussion of published clinical trials. ( 28861213 )
2017
12
Perioperative course in patients with hereditary or acquired angioedema. ( 27687418 )
2016
13
A case of acquired angioedema possibly associated with adenocarcinoma of the colon. ( 27026513 )
2016
14
Comparing acquired angioedema with hereditary angioedema (type I/II): Findings from the Icatibant Outcome Survey. ( 27936514 )
2016
15
Rituximab in a patient with splenic marginal zone lymphoma and acquired angioedema. ( 27017557 )
2016
16
Shortened Activated Partial Thromboplastin Time May Help in Diagnosing Hereditary and Acquired Angioedema. ( 27463190 )
2016
17
Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema? ( 26478549 )
2015
18
The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity. ( 26068904 )
2015
19
A UK national audit of hereditary and acquired angioedema. ( 23786259 )
2014
20
Acquired angioedema with C1 inhibitor deficiency secondary to splenic marginal zone B-cell lymphoma. ( 24970460 )
2014
21
Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema. ( 25506230 )
2014
22
New perspectives in acquired angioedema. ( 24979839 )
2014
23
Use of recombinant C1 inhibitor in patients with resistant or frequent attacks of hereditary or acquired angioedema. ( 24556385 )
2014
24
An evidence based therapeutic approach to hereditary and acquired angioedema. ( 24983294 )
2014
25
Acquired angioedema--occurrence, clinical features and associated disorders in a Danish nationwide patient cohort. ( 23921495 )
2013
26
Acquired angioedema with raised serum tryptase. ( 23244664 )
2013
27
A case of multicentric Castleman's disease in HIV infection with the rare complication of acquired angioedema. ( 24352125 )
2013
28
A patient with chronic lymphocytic leukemia and acquired angioedema: correlation of clinical and biochemical response to CLL therapy. ( 23441138 )
2013
29
International consensus on hereditary and acquired angioedema. ( 23176876 )
2012
30
Acquired angioedema and marginal zone lymphoma. ( 22508826 )
2012
31
Chapter 22: Hereditary and acquired angioedema. ( 22794695 )
2012
32
Successful treatment of an acute attack of acquired angioedema with the bradykinin-B2-receptor antagonist icatibant. ( 20477917 )
2011
33
"Surgical" abdomen in a patient with chronic lymphocytic leukemia: a case of acquired angioedema. ( 21997434 )
2011
34
Update on laboratory tests for the diagnosis and differentiation of hereditary angioedema and acquired angioedema. ( 22195757 )
2011
35
Acquired angioedema with C1 inhibitor deficiency associated with anticardiolipin antibodies. ( 22230421 )
2011
36
Acquired angioedema responding to rituximab. ( 21691703 )
2011
37
Treatment of acquired angioedema with icatibant: a case report. ( 20680518 )
2011
38
Rapid resolution of erythema marginatum after icatibant in acquired angioedema. ( 21279303 )
2011
39
Optimal medical management of acute attacks of acquired angioedema. ( 20042567 )
2010
40
Acquired angioedema: Autoantibody associations and C1q utility as a diagnostic tool. ( 20929611 )
2010
41
Successful use of etanercept in acquired angioedema in a patient with psoriatic arthritis. ( 20040647 )
2010
42
Acquired angioedema. ( 20667117 )
2010
43
Acquired angioedema associated with hereditary angioedema due to C1 inhibitor deficiency. ( 18447143 )
2008
44
Acquired angioedema associated with primary antiphospholipid syndrome in a patient with antithrombin III deficiency. ( 18204284 )
2008
45
Acquired angioedema in non-Hodgkin's lymphoma. ( 17317233 )
2007
46
Acute upper airway obstruction from acquired angioedema. ( 17305664 )
2007
47
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
48
Rituximab-induced elimination of acquired angioedema due to C1-inhibitor deficiency. ( 16887400 )
2006
49
Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. ( 16630950 )
2006
50
Skin venous thromboembolism by combined oral contraceptive in a woman with acquired angioedema and Factor V Leiden mutation. ( 16472576 )
2006

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 region GO:0005576 9.63 C1S C3 CRP F5 HP SERPING1
2 extracellular space GO:0005615 9.43 C1S C3 CRP F5 HP SERPING1
3 platelet alpha granule lumen GO:0031093 9.26 F5 SERPING1
4 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 proteolysis GO:0006508 9.63 C1S C3 HP
2 innate immune response GO:0045087 9.46 C1S C3 CRP SERPING1
3 platelet degranulation GO:0002576 9.43 F5 SERPING1
4 complement activation GO:0006956 9.4 C1S C3
5 hemostasis GO:0007599 9.37 F5 SERPING1
6 blood circulation GO:0008015 9.32 F5 SERPING1
7 acute-phase response GO:0006953 9.26 CRP HP
8 regulation of complement activation GO:0030449 9.13 C1S C3 SERPING1
9 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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