MCID: ACQ012
MIFTS: 42

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 54 74
Acquired C1 Inhibitor Deficiency 54
Angioedema, Acquired 54

Classifications:



Summaries for Acquired Angioedema

NIH Rare Diseases : 54 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 Immunologic Factors have been mentioned in the context of this disorder. Affiliated tissues include tongue, b cells and skin, 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 79)
# Related Disease Score Top Affiliating Genes
1 angioedema 31.5 C1S SERPING1
2 c1 inhibitor deficiency 30.7 C1S SERPING1
3 hereditary angioedema 30.5 C1S SERPING1
4 anemia, autoimmune hemolytic 29.1 C1S C3 HP
5 acquired angioedema type 2 12.4
6 acquired angioedema with c1inh deficiency 12.3
7 acquired angioedema type 1 12.3
8 angioedema induced by ace inhibitors 11.6
9 complement component 4, partial deficiency of 10.3
10 lymphoma 10.2
11 hydrops, lactic acidosis, and sideroblastic anemia 10.2 C1S SERPING1
12 louse-borne relapsing fever 10.1 CRP SERPING1
13 mediastinitis 10.1 CRP F5
14 urticaria 10.1 C1S SERPING1
15 peripheral vertigo 10.1 CRP F5
16 complement deficiency 10.1 C3 SERPING1
17 leukemia, chronic lymphocytic 2 10.1
18 leukemia, chronic lymphocytic 10.1
19 leukemia 10.1
20 marginal zone b-cell lymphoma 10.1
21 lymphocytic leukemia 10.1
22 leukemia, b-cell, chronic 10.1
23 arteritic anterior ischemic optic neuropathy 10.0 CRP F5
24 acute maxillary sinusitis 10.0 C3 CRP
25 hypersensitivity vasculitis 10.0 C3 CRP
26 lemierre's syndrome 10.0 C3 CRP
27 homocysteinemia 10.0 CRP F5
28 hypersensitivity reaction type iii disease 10.0 C3 CRP
29 pulmonary embolism 10.0 CRP F5
30 henoch-schoenlein purpura 10.0 C3 CRP
31 afibrinogenemia 10.0 C3 F5
32 melkersson-rosenthal syndrome 10.0
33 sjogren syndrome 10.0
34 monoclonal paraproteinemia 10.0
35 lymphosarcoma 10.0
36 autoimmune disease of musculoskeletal system 10.0 C3 CRP
37 splenic marginal zone lymphoma 10.0
38 lyme disease 10.0 C3 CRP
39 intestinal disease 10.0 CRP F5
40 rheumatic disease 10.0 C1S CRP
41 dysentery 9.9 CRP HP
42 reactive arthritis 9.9 CRP HP
43 cerebrovascular disease 9.8 CRP F5
44 cholangitis 9.8 CRP HP
45 blepharochalasis and double lip 9.8
46 systemic lupus erythematosus 9.8
47 thrombophilia due to thrombin defect 9.8
48 myelofibrosis 9.8
49 helicobacter pylori infection 9.8
50 psoriatic arthritis 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:

33 (show all 8)
# Description HPO Frequency HPO Source Accession
1 abdominal pain 33 hallmark (90%) HP:0002027
2 angioedema 33 hallmark (90%) HP:0100665
3 ascites 33 occasional (7.5%) HP:0001541
4 intestinal obstruction 33 occasional (7.5%) HP:0005214
5 urticaria 33 occasional (7.5%) HP:0001025
6 facial edema 33 occasional (7.5%) HP:0000282
7 intestinal edema 33 occasional (7.5%) HP:0005225
8 laryngeal edema 33 occasional (7.5%) HP:0012027

MGI Mouse Phenotypes related to Acquired Angioedema:

47
# 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 7)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Complement System Proteins Phase 2, Phase 3,Phase 3
2 Immunologic Factors Phase 2, Phase 3,Phase 3
3 Complement C1 Inhibitor Protein Phase 2, Phase 3,Phase 3
4 Immunosuppressive Agents Phase 2, Phase 3,Phase 3
5 Complement C1 Inactivator Proteins Phase 2, Phase 3,Phase 3
6 Complement Inactivating Agents Phase 2, Phase 3,Phase 3
7 Angiotensin-Converting Enzyme Inhibitors

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 CLOUD-R HAE REGISTRY Recruiting NCT03845946

Search NIH Clinical Center for Acquired Angioedema

Genetic Tests for Acquired Angioedema

Anatomical Context for Acquired Angioedema

MalaCards organs/tissues related to Acquired Angioedema:

42
Tongue, B Cells, Skin, Colon, Testes, Spleen, Bone

Publications for Acquired Angioedema

Articles related to Acquired Angioedema:

(show top 50) (show all 131)
# Title Authors Year
1
Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate. ( 30866985 )
2019
2
Hereditary and Acquired Angioedema: Heterogeneity of Pathogenesis and Clinical Phenotypes. ( 29393169 )
2018
3
Acquired Angioedema With Anti-C1-inhibitor Autoantibodies During Assisted Reproduction Techniques. ( 29461216 )
2018
4
Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency. ( 29545957 )
2018
5
Atypical presentation of acquired angioedema. ( 29554170 )
2018
6
Idiopathic Nonhistaminergic Acquired Angioedema Versus Hereditary Angioedema. ( 29715562 )
2018
7
Case report presenting the diagnostic challenges in a patient with recurrent acquired angioedema, antiphospholipid antibodies and undetectable C2 levels. ( 29881401 )
2018
8
Acquired angioedema in juvenile systemic lupus erythematosus: case-based review. ( 29951963 )
2018
9
Long-term effects upon rituximab treatment of acquired angioedema due to C1-inhibitor deficiency. ( 30488963 )
2018
10
Diagnosis of solitary extramedullary plasmacytoma located in the nasopharynx in a patient with acquired angioedema. ( 30618535 )
2018
11
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
12
Splenic marginal zone lymphomas in acquired C1-inhibitor deficiency: clinical and molecular characterization. ( 30073422 )
2018
13
Comparing acquired angioedema with hereditary angioedema (types I/II): findings from the Icatibant Outcome Survey. ( 27936514 )
2017
14
Acquired Angioedema. ( 28377018 )
2017
15
Idiopathic non-histaminergic acquired angioedema: a case series and discussion of published clinical trials. ( 28861213 )
2017
16
Diagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency. ( 28284781 )
2017
17
Acquired C1 Inhibitor Deficiency. ( 28687105 )
2017
18
Clinical and biological response to rituximab treatment in 3 patients with acquired C1-inhibitor deficiency. ( 28866304 )
2017
19
The Janus faces of acquired angioedema: C1-inhibitor deficiency, lymphoproliferation and autoimmunity. ( 26068904 )
2016
20
Rituximab in a patient with splenic marginal zone lymphoma and acquired angioedema. ( 27017557 )
2016
21
A case of acquired angioedema possibly associated with adenocarcinoma of the colon. ( 27026513 )
2016
22
Shortened Activated Partial Thromboplastin Time May Help in Diagnosing Hereditary and Acquired Angioedema. ( 27463190 )
2016
23
Perioperative course in patients with hereditary or acquired angioedema. ( 27687418 )
2016
24
High prevalence of splenic marginal zone lymphoma among patients with acquired C1 inhibitor deficiency. ( 26728240 )
2016
25
Cases of acquired C1 inhibitor deficiency treated with rituximab. ( 27130715 )
2016
26
A nationwide study of acquired C1-inhibitor deficiency in France: Characteristics and treatment responses in 92 patients. ( 27537564 )
2016
27
Non-myeloablative allogeneic stem cell transplantation: a new treatment option for acquired angioedema? ( 26478549 )
2015
28
Benefits of hydroxychloroquine in the treatment of a patient with angioedema due to acquired C1 inhibitor deficiency. ( 25457864 )
2015
29
A UK national audit of hereditary and acquired angioedema. ( 23786259 )
2014
30
A case of multicentric Castleman's disease in HIV infection with the rare complication of acquired angioedema. ( 24352125 )
2014
31
Use of recombinant C1 inhibitor in patients with resistant or frequent attacks of hereditary or acquired angioedema. ( 24556385 )
2014
32
Acquired angioedema with C1 inhibitor deficiency secondary to splenic marginal zone B-cell lymphoma. ( 24970460 )
2014
33
New perspectives in acquired angioedema. ( 24979839 )
2014
34
An evidence based therapeutic approach to hereditary and acquired angioedema. ( 24983294 )
2014
35
Rituximab therapy in a patient with low grade B-cell lymphoproliferative disease and concomitant acquired angioedema. ( 25506230 )
2014
36
Acquired C1 inhibitor deficiency: should we monitor for associated antibody deficiency? ( 24565597 )
2014
37
A case of transient acquired C1 inhibitor deficiency. ( 24950848 )
2014
38
Acquired angioedema with raised serum tryptase. ( 23244664 )
2013
39
A patient with chronic lymphocytic leukemia and acquired angioedema: correlation of clinical and biochemical response to CLL therapy. ( 23441138 )
2013
40
Acquired angioedema--occurrence, clinical features and associated disorders in a Danish nationwide patient cohort. ( 23921495 )
2013
41
A focused parameter update: hereditary angioedema, acquired C1 inhibitor deficiency, and angiotensin-converting enzyme inhibitor-associated angioedema. ( 23726531 )
2013
42
Small bowel angioedema due to acquired C1 inhibitor deficiency: a case report and overview. ( 23255023 )
2013
43
Acquired C1-inhibitor deficiency and lymphoproliferative disorders: a tight relationship. ( 23490322 )
2013
44
Acquired angioedema and marginal zone lymphoma. ( 22508826 )
2012
45
Chapter 22: Hereditary and acquired angioedema. ( 22794695 )
2012
46
International consensus on hereditary and acquired angioedema. ( 23176876 )
2012
47
Acquired C1-inhibitor deficiency: 7 patients treated with rituximab. ( 22526593 )
2012
48
Icatibant treatment for acquired C1-inhibitor deficiency: a real-world observational study. ( 22686628 )
2012
49
Acquired C1-inhibitor deficiency: a case report. ( 23092008 )
2012
50
Successful treatment of an acute attack of acquired angioedema with the bradykinin-B2-receptor antagonist icatibant. ( 20477917 )
2011

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 9.46 C1S 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 8.62 C1S HP

Sources for Acquired Angioedema

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
Content
Loading form....