MCID: ACQ012
MIFTS: 39

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 52 71
Acquired C1 Inhibitor Deficiency 52
Angioedema, Acquired 52

Classifications:



External Ids:

UMLS 71 C2931758

Summaries for Acquired Angioedema

NIH Rare Diseases : 52 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 c1 inhibitor deficiency and hydrops, lactic acidosis, and sideroblastic anemia. 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 Immunosuppressive Agents and Complement System Proteins have been mentioned in the context of this disorder. Affiliated tissues include tongue, b cells and t cells, and related phenotypes are abdominal pain and angioedema

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 132)
# Related Disease Score Top Affiliating Genes
1 c1 inhibitor deficiency 31.1 SERPING1 C1S
2 hydrops, lactic acidosis, and sideroblastic anemia 30.6 SERPING1 C1S
3 hereditary angioedema 30.4 SERPING1 CRP C1S
4 angioedema 30.3 SERPING1 CRP C3 C1S
5 urticaria 29.8 SERPING1 CRP C1S
6 anemia, autoimmune hemolytic 29.4 HP C3 C1S
7 antiphospholipid syndrome 29.0 F5 CRP C3
8 thrombocytopenia 28.2 HP F5 CRP C3
9 acquired angioedema type 1 12.5
10 acquired angioedema type 2 12.5
11 acquired angioedema with c1inh deficiency 12.3
12 angioedema induced by ace inhibitors 11.7
13 complement component 4, partial deficiency of 10.5
14 lymphoproliferative syndrome 10.3
15 lymphoma 10.2
16 marginal zone b-cell lymphoma 10.1
17 sjogren syndrome 10.1
18 complement component 3 deficiency 10.1 C3 C1S
19 autoimmune disease 10.1
20 leukemia, chronic lymphocytic 10.1
21 splenic marginal zone lymphoma 10.1
22 lymphocytic leukemia 10.1
23 dermatographia 10.1 SERPING1 CRP
24 epiglottitis 10.1 SERPING1 CRP
25 physical urticaria 10.1 SERPING1 CRP
26 gingival recession 10.0 CRP C1S
27 thrombotic thrombocytopenic purpura 10.0 HP C3
28 thrombophilia due to thrombin defect 10.0
29 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
30 hemoglobinuria 10.0 HP C3
31 louse-borne relapsing fever 10.0 CRP C3
32 melkersson-rosenthal syndrome 10.0
33 mantle cell lymphoma 10.0
34 facial paralysis 10.0
35 echinococcosis 10.0
36 monoclonal paraproteinemia 10.0
37 cryoglobulinemia 10.0
38 lymphosarcoma 10.0
39 relapsing fever 10.0 CRP C3
40 paroxysmal nocturnal hemoglobinuria 10.0 HP C3
41 splenic infarction 10.0 HP CRP
42 pyuria 10.0 CRP C3
43 vaginal discharge 10.0 HP CRP
44 melancholia 10.0 HP CRP
45 acute proliferative glomerulonephritis 10.0 CRP C3
46 hypersensitivity vasculitis 10.0 CRP C3
47 anuria 10.0 HP CRP
48 rapidly progressive glomerulonephritis 10.0 CRP C3
49 bronchopneumonia 10.0 HP CRP
50 dysentery 9.9 HP CRP

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 abdominal pain 31 hallmark (90%) HP:0002027
2 angioedema 31 hallmark (90%) HP:0100665
3 ascites 31 occasional (7.5%) HP:0001541
4 urticaria 31 occasional (7.5%) HP:0001025
5 intestinal obstruction 31 occasional (7.5%) HP:0005214
6 facial edema 31 occasional (7.5%) HP:0000282
7 laryngeal edema 31 occasional (7.5%) HP:0012027
8 intestinal edema 31 occasional (7.5%) HP:0005225

MGI Mouse Phenotypes related to Acquired Angioedema:

45
# 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 Immunosuppressive Agents Phase 2, Phase 3
2 Complement System Proteins Phase 2, Phase 3
3 Complement C1 Inactivator Proteins Phase 2, Phase 3
4 Immunologic Factors Phase 2, Phase 3
5 Complement C1 Inhibitor Protein Phase 2, Phase 3
6 Complement Inactivating Agents Phase 2, Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Pharmacokinetics, Clinical Efficacy and Safety of C1 Inhibitor Concentrate (C1-Esteraseremmer-N) for the Treatment of Hereditary (and Acquired) Angioedema Completed NCT00125541 Phase 2, Phase 3 C1 inhibitor concentrate (C1-esteraseremmer-N)
2 Pharmacokinetics, Clinical Efficacy and Safety of C1 Inhibitor Concentrate (C1-Esteraseremmer-N) for the Treatment of Hereditary (and Acquired) Angioedema Completed NCT00125151 Phase 3 C1 inhibitor concentrate (C1-esteraseremmer-N)
3 Pharmacokinetics, Clinical Efficacy and Safety of C1 Inhibitor Concentrate (C1-Esteraseremmer-N) for the Treatment of Hereditary (and Acquired) Angioedema Completed NCT00119431 Phase 2 C1 inhibitor concentrate
4 Biomarker for Hereditary Angioedema Disease Type 1 - An International, Multicenter, Epidemiological Protocol 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:

40
Tongue, B Cells, T Cells, Colon, Skin, Testes, Heart

Publications for Acquired Angioedema

Articles related to Acquired Angioedema:

(show top 50) (show all 231)
# Title Authors PMID Year
1
Serum complexes between C1INH and C1INH autoantibodies for the diagnosis of acquired angioedema. 61
31397881 2019
2
Hereditary and acquired angioedema. 61
31690390 2019
3
Angioedema as a systemic disease. 61
31864442 2019
4
A Case of Acquired Angioedema with Low C1 Inhibitor (C1-INH) Associated with Splenic Marginal Zone Lymphoma. 61
31588119 2019
5
Acquired Angioedema: A Rare Manifestation of Angioimmunoblastic T Cell Lymphoma. 61
31741940 2019
6
Anaesthetic management of patients with pre-existing allergic conditions: a narrative review. 61
30916009 2019
7
Long-term effects upon rituximab treatment of acquired angioedema due to C1-inhibitor deficiency. 61
30488963 2019
8
Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate. 61
30866985 2019
9
Systematic Review of Safety and Efficacy of Rituximab in Treating Immune-Mediated Disorders. 61
31555262 2019
10
Deletions in SERPING1 Lead to Lower C1 Inhibitor Function: Lower C1 Inhibitor Function Can Predict Disease Severity. 61
30278448 2019
11
Identification of the recently described plasminogen gene mutation p.Lys330Glu in a family from Northern Germany with hereditary angioedema. 61
30809376 2019
12
Melkersson-Rosenthal syndrome: a case report of a rare disease with overlapping features. 61
30622569 2019
13
Diagnosis of solitary extramedullary plasmacytoma located in the nasopharynx in a patient with acquired angioedema. 61
30618535 2018
14
Quantification of human complement C2 protein using an automated turbidimetric immunoassay. 61
29729138 2018
15
Splenic marginal zone lymphomas in acquired C1-inhibitor deficiency: clinical and molecular characterization. 61
30073422 2018
16
Acquired angioedema in juvenile systemic lupus erythematosus: case-based review. 61
29951963 2018
17
Idiopathic Nonhistaminergic Acquired Angioedema Versus Hereditary Angioedema. 61
29715562 2018
18
Epidemiology of Bradykinin-mediated angioedema: a systematic investigation of epidemiological studies. 61
29728119 2018
19
Atypical presentation of acquired angioedema. 61
29554170 2018
20
Omalizumab for Idiopathic Nonhistaminergic Angioedema: Evidence for Efficacy in 2 Patients. 61
30140474 2018
21
Acquired Angioedema With Anti-C1-inhibitor Autoantibodies During Assisted Reproduction Techniques. 61
29461216 2018
22
Case report presenting the diagnostic challenges in a patient with recurrent acquired angioedema, antiphospholipid antibodies and undetectable C2 levels. 61
29881401 2018
23
Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency. 61
29545957 2018
24
Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes. 61
29393169 2018
25
Management of pediatric 'cannot intubate, cannot oxygenate'. 61
29123910 2017
26
Diagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency. 61
28284781 2017
27
Acquired C1 Inhibitor Deficiency. 61
28687105 2017
28
Angioedema. 61
28818177 2017
29
Comparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey. 61
27936514 2017
30
Angioedema. 61
28291095 2017
31
Acquired Angioedema. 61
28377018 2017
32
Idiopathic Non-histaminergic Angioedema: Successful Treatment with Omalizumab in Five Patients. 61
27826875 2017
33
Idiopathic non-histaminergic acquired angioedema: a case series and discussion of published clinical trials. 61
28861213 2017
34
Recurrent angioedema associated with pharmacological inhibition of dipeptidyl peptidase IV. 61
28073873 2017
35
Angioedema: Perioperative management. 61
28634542 2017
36
Monoclonal gammopathy of cutaneous significance: review of a relevant concept. 61
27501129 2017
37
Perioperative course in patients with hereditary or acquired angioedema. 61
27687418 2016
38
Angioedema Phenotypes: Disease Expression and Classification. 61
27113957 2016
39
"Nuts and Bolts" of Laboratory Evaluation of Angioedema. 61
27142368 2016
40
A nationwide study of acquired C1-inhibitor deficiency in France: Characteristics and treatment responses in 92 patients. 61
27537564 2016
41
Idiopathic histaminergic angioedema without wheals: a case series of 31 patients. 61
26969870 2016
42
Clinical presentation, pathophysiology, diagnosis, and treatment of acquired and hereditary angioedema: Exploring state-of-the-art therapies in RI. 61
27247973 2016
43
Rituximab in a patient with splenic marginal zone lymphoma and acquired angioedema. 61
27017557 2016
44
Nanofiltrated C1-esterase-inhibitor in the prophylactic treatment of bradykinin-mediated angioedema. 61
26756974 2016
45
A case of acquired angioedema possibly associated with adenocarcinoma of the colon. 61
27026513 2016
46
High prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibitor deficiency. 61
26728240 2016
47
The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity. 61
26068904 2016
48
[Acquired angioedema – clinical characteristic of the patients diagnosed in 2012-2016 with acquired C1 inhibitor deficiency]. 61
29693974 2016
49
Novelties in the Diagnosis and Treatment of Angioedema. 61
27470642 2016
50
Hereditary and acquired C1-inhibitor-dependent angioedema: from pathophysiology to treatment. 61
27018196 2016

Variations for Acquired Angioedema

Expression for Acquired Angioedema

Search GEO for disease gene expression data for Acquired Angioedema.

Pathways for Acquired Angioedema

Pathways related to Acquired Angioedema according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.66 CRP C3 C1S
2
Show member pathways
11.46 SERPING1 C3 C1S
3 11.42 SERPING1 F5 C3 C1S
4 11.35 C3 C1S
5 11.21 SERPING1 C3 C1S
6
Show member pathways
11.05 SERPING1 CRP C3 C1S

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 SERPING1 HP F5 CRP C3 C1S
2 extracellular space GO:0005615 9.43 SERPING1 HP F5 CRP C3 C1S
3 platelet alpha granule lumen GO:0031093 9.26 SERPING1 F5
4 blood microparticle GO:0072562 8.92 SERPING1 HP C3 C1S

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 9.46 SERPING1 CRP C3 C1S
2 complement activation GO:0006956 9.4 C3 C1S
3 hemostasis GO:0007599 9.37 SERPING1 F5
4 acute-phase response GO:0006953 9.32 HP CRP
5 blood circulation GO:0008015 9.26 SERPING1 F5
6 regulation of complement activation GO:0030449 9.13 SERPING1 C3 C1S
7 complement activation, classical pathway GO:0006958 8.92 SERPING1 CRP C3 C1S

Sources for Acquired Angioedema

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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