{"Name":"Acquired angioedema","DiseaseID__c":"GARD:0008605","id":8605,"encodedName":"acquired-angioedema","IsDeleted":false,"Disease_Name_Full__c":"Acquired angioedema","Xref_IDs__c":"C2931758; C538173; DOID:0080941; MEDGEN:419486; MONDO:0019624; ORPHA:91385","USA_Estimate__c":"5,000","No_of_Specialist_Tagsa__c":3,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":"8,000 to 80,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":4,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0019624","Disease_Description__c":"A rare disease characterized by the occurrence of transitory and recurrent subcutaneous and/or submucosal edemas resulting in swelling and/or abdominal pain due to an acquired C1 inhibitor (C1-INH) deficiency.","GARD_Name__c":"Acquired angioedema","GARD_Synonym__c":"aae; acquired angioneurotic edema; acquired angioneurotic oedema; acquired bradykinine-induced angioedema; acquired c1 inhibitor deficiency; acquired non histamine-induced angioedema","Curated_Disease_Description_Source__c":"GARD:0008605","Curated_Disease_Description__c":"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 serious. 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. During severe or life-threatening episodes, intensive support may be needed (such as IV fluids or intubation for a blocked airway).","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"5,000","Age_at_Onset_Snippet_Text__c":"as an Adult","SourceID__c":"ORPHA:91385","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0019624","ORPHANET_ID__c":"ORPHA:91385","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Angioedema adquirido","Spanish_Description_Source__c":"ORPHA:91385","Spanish_Description__c":"Es una enfermedad poco frecuente caracterizada por la aparición recurrente y transitoria de edemas subcutáneos y/o submucosos que provocan inflamación y/o dolor abdominal debido a la deficiencia adquirida de inhibidor C1 (C1-INH).","Spanish_Disease_Name__c":"angioedema adquirido","Spanish_GARD_Synonym__c":"angioedema adquirido inducido por bradiquinina; angioedema adquirido no inducido por histamina; deficiencia adquirida de inhibidor c1; edema angioneurótico adquirido","Category_Linearization__c":"ORPHA:98023","icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"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 serious. 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. During severe or life-threatening episodes, intensive support may be needed (such as IV fluids or intubation for a blocked airway).","Curated_Disease_Description_Source__c":"GARD:0008605","GARD_Synonym__c":"aae; acquired angioneurotic edema; acquired angioneurotic oedema; acquired bradykinine-induced angioedema; acquired c1 inhibitor deficiency; acquired non histamine-induced angioedema","Name":"Acquired angioedema","Curated_USA_Estimate__c":"5,000","estimateUsa":"5,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Allergy & Asthma Network Mothers of Asthmatics","Website__c":"https://allergyasthmanetwork.org/"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Rheumatology","Tag_Category__c":"Specialist"},{"Tag_Name__c":"Immunology","Tag_Category__c":"Specialist"},{"Tag_Name__c":"Vascular Medicine","Tag_Category__c":"Specialist","curated_tag_name":"Vascular diseases"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adult","Provided_By__c":"ORPHA:91385"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C538173","Source__c":"MONDO:0019624","Xref__c":"C538173"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C2931758","Source__c":"C2931758","Xref__c":"C2931758"},{"URL__c":"https://www.orpha.net/en/disease/detail/91385","Source__c":"C2931758; MONDO:0019624; ORPHA:91385","Xref__c":"ORPHA:91385"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=419486","Source__c":"C2931758","Xref__c":"MEDGEN:419486"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A0080941","Source__c":"MONDO:0019624","Xref__c":"DOID:0080941"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0019624","Source__c":"GARD:0008605","Xref__c":"MONDO:0019624"}],"tags":{"Specialist":["Rheumatology","Immunology","Vascular Medicine"]},"synonyms":["aae"," acquired angioneurotic edema"," acquired angioneurotic oedema"," acquired bradykinine-induced angioedema"," acquired c1 inhibitor deficiency"," acquired non histamine-induced angioedema"]}