MCID: ACQ012
MIFTS: 37

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 72
Acquired C1 Inhibitor Deficiency 53
Angioedema, Acquired 53

Classifications:



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UMLS 72 C2931758

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 Inhibitor Protein and Complement System Proteins have been mentioned in the context of this disorder. Affiliated tissues include tongue, testes and b 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 88)
# Related Disease Score Top Affiliating Genes
1 angioedema 31.7 SERPING1 C1S
2 c1 inhibitor deficiency 31.2 SERPING1 C1S
3 hereditary angioedema 31.1 SERPING1 C1S
4 hydrops, lactic acidosis, and sideroblastic anemia 30.7 SERPING1 C1S
5 urticaria 30.3 SERPING1 C1S
6 anemia, autoimmune hemolytic 29.3 HP C3 C1S
7 deficiency anemia 28.8 HP CRP
8 acquired angioedema type 1 12.5
9 acquired angioedema type 2 12.5
10 acquired angioedema with c1inh deficiency 12.3
11 angioedema induced by ace inhibitors 11.7
12 complement component 4, partial deficiency of 10.5
13 lymphoproliferative syndrome 10.3
14 complement deficiency 10.2 SERPING1 C3
15 lymphoma 10.2
16 lymphoma, hodgkin, classic 10.1
17 sjogren syndrome 10.1
18 lymphoma, non-hodgkin, familial 10.1
19 louse-borne relapsing fever 10.1 SERPING1 CRP
20 autoimmune disease 10.1
21 leukemia, chronic lymphocytic 10.1
22 marginal zone b-cell lymphoma 10.1
23 lymphocytic leukemia 10.1
24 leukemia, b-cell, chronic 10.1
25 acute maxillary sinusitis 10.0 CRP C3
26 hemoglobinuria 10.0 HP C3
27 hypersensitivity vasculitis 10.0 CRP C3
28 hypersensitivity reaction type iii disease 10.0 CRP C3
29 lemierre's syndrome 10.0 CRP C3
30 henoch-schoenlein purpura 10.0 CRP C3
31 thrombophilia due to thrombin defect 10.0
32 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
33 splenic marginal zone lymphoma 10.0
34 antiphospholipid syndrome 10.0
35 melkersson-rosenthal syndrome 10.0
36 mantle cell lymphoma 10.0
37 facial paralysis 10.0
38 echinococcosis 10.0
39 monoclonal paraproteinemia 10.0
40 cryoglobulinemia 10.0
41 lymphosarcoma 10.0
42 dysentery 10.0 HP CRP
43 hemolytic-uremic syndrome 10.0 HP C3
44 reactive arthritis 10.0 HP CRP
45 autoimmune disease of musculoskeletal system 10.0 CRP C3
46 lyme disease 9.9 CRP C3
47 cholangitis 9.9 HP CRP
48 angioedema, hereditary, type i 9.8
49 blepharochalasis and double lip 9.8
50 systemic lupus erythematosus 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 abdominal pain 32 hallmark (90%) HP:0002027
2 angioedema 32 hallmark (90%) HP:0100665
3 ascites 32 occasional (7.5%) HP:0001541
4 urticaria 32 occasional (7.5%) HP:0001025
5 intestinal obstruction 32 occasional (7.5%) HP:0005214
6 facial edema 32 occasional (7.5%) HP:0000282
7 laryngeal edema 32 occasional (7.5%) HP:0012027
8 intestinal edema 32 occasional (7.5%) HP:0005225

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

41
Tongue, Testes, B Cells, Colon, Skin, Heart, Thyroid

Publications for Acquired Angioedema

Articles related to Acquired Angioedema:

(show top 50) (show all 226)
# Title Authors PMID Year
1
Serum complexes between C1INH and C1INH-autoantibodies for the diagnosis of Acquired Angioedema. 38
31397881 2019
2
Anaesthetic management of patients with pre-existing allergic conditions: a narrative review. 38
30916009 2019
3
Long-term effects upon rituximab treatment of acquired angioedema due to C1-inhibitor deficiency. 38
30488963 2019
4
Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate. 38
30866985 2019
5
Deletions in SERPING1 Lead to Lower C1 Inhibitor Function: Lower C1 Inhibitor Function Can Predict Disease Severity. 38
30278448 2019
6
Melkersson-Rosenthal syndrome: a case report of a rare disease with overlapping features. 38
30622569 2019
7
Identification of the recently described plasminogen gene mutation p.Lys330Glu in a family from Northern Germany with hereditary angioedema. 38
30809376 2019
8
Diagnosis of solitary extramedullary plasmacytoma located in the nasopharynx in a patient with acquired angioedema. 38
30618535 2018
9
Quantification of human complement C2 protein using an automated turbidimetric immunoassay. 38
29729138 2018
10
Splenic marginal zone lymphomas in acquired C1-inhibitor deficiency: clinical and molecular characterization. 38
30073422 2018
11
Acquired angioedema in juvenile systemic lupus erythematosus: case-based review. 38
29951963 2018
12
Idiopathic Nonhistaminergic Acquired Angioedema Versus Hereditary Angioedema. 38
29715562 2018
13
Epidemiology of Bradykinin-mediated angioedema: a systematic investigation of epidemiological studies. 38
29728119 2018
14
Atypical presentation of acquired angioedema. 38
29554170 2018
15
Acquired Angioedema With Anti-C1-inhibitor Autoantibodies During Assisted Reproduction Techniques. 38
29461216 2018
16
Omalizumab for Idiopathic Nonhistaminergic Angioedema: Evidence for Efficacy in 2 Patients. 38
30140474 2018
17
Case report presenting the diagnostic challenges in a patient with recurrent acquired angioedema, antiphospholipid antibodies and undetectable C2 levels. 38
29881401 2018
18
Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency. 38
29545957 2018
19
Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes. 38
29393169 2018
20
Management of pediatric 'cannot intubate, cannot oxygenate'. 38
29123910 2017
21
Diagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency. 38
28284781 2017
22
Acquired C1 Inhibitor Deficiency. 38
28687105 2017
23
Angioedema. 38
28818177 2017
24
Comparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey. 38
27936514 2017
25
Angioedema. 38
28291095 2017
26
Acquired Angioedema. 38
28377018 2017
27
Idiopathic Non-histaminergic Angioedema: Successful Treatment with Omalizumab in Five Patients. 38
27826875 2017
28
Idiopathic non-histaminergic acquired angioedema: a case series and discussion of published clinical trials. 38
28861213 2017
29
Recurrent angioedema associated with pharmacological inhibition of dipeptidyl peptidase IV. 38
28073873 2017
30
Angioedema: Perioperative management. 38
28634542 2017
31
Monoclonal gammopathy of cutaneous significance: review of a relevant concept. 38
27501129 2017
32
Perioperative course in patients with hereditary or acquired angioedema. 38
27687418 2016
33
Angioedema Phenotypes: Disease Expression and Classification. 38
27113957 2016
34
"Nuts and Bolts" of Laboratory Evaluation of Angioedema. 38
27142368 2016
35
A nationwide study of acquired C1-inhibitor deficiency in France: Characteristics and treatment responses in 92 patients. 38
27537564 2016
36
Idiopathic histaminergic angioedema without wheals: a case series of 31 patients. 38
26969870 2016
37
Clinical presentation, pathophysiology, diagnosis, and treatment of acquired and hereditary angioedema: Exploring state-of-the-art therapies in RI. 38
27247973 2016
38
Rituximab in a patient with splenic marginal zone lymphoma and acquired angioedema. 38
27017557 2016
39
Nanofiltrated C1-esterase-inhibitor in the prophylactic treatment of bradykinin-mediated angioedema. 38
26756974 2016
40
A case of acquired angioedema possibly associated with adenocarcinoma of the colon. 38
27026513 2016
41
High prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibitor deficiency. 38
26728240 2016
42
The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity. 38
26068904 2016
43
[Acquired angioedema – clinical characteristic of the patients diagnosed in 2012-2016 with acquired C1 inhibitor deficiency]. 38
29693974 2016
44
Novelties in the Diagnosis and Treatment of Angioedema. 38
27470642 2016
45
Hereditary and acquired C1-inhibitor-dependent angioedema: from pathophysiology to treatment. 38
27018196 2016
46
Overview of Laboratory Testing and Clinical Presentations of Complement Deficiencies and Dysregulation. 38
27717414 2016
47
Shortened Activated Partial Thromboplastin Time May Help in Diagnosing Hereditary and Acquired Angioedema. 38
27463190 2016
48
'Epinephrine-resistant' angioedema. 38
26823364 2016
49
Bradykinin-mediated angioedema. 38
26842379 2016
50
ELISA to measure neutralizing capacity of anti-C1-inhibitor antibodies in plasma of angioedema patients. 38
26318839 2015

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 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 proteolysis GO:0006508 9.46 HP C1S
2 innate immune response GO:0045087 9.46 SERPING1 CRP C3 C1S
3 platelet degranulation GO:0002576 9.43 SERPING1 F5
4 complement activation GO:0006956 9.4 C3 C1S
5 hemostasis GO:0007599 9.37 SERPING1 F5
6 blood circulation GO:0008015 9.32 SERPING1 F5
7 acute-phase response GO:0006953 9.26 HP CRP
8 regulation of complement activation GO:0030449 9.13 SERPING1 C3 C1S
9 complement activation, classical pathway GO:0006958 8.92 SERPING1 CRP C3 C1S

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 8.62 HP 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 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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