loading...| Preferred Name |
Acquired C1 esterase inhibitor deficiency |
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| Synonyms |
Acquired angioneurotic oedema with C1 inhibitor deficiency Acquired angio-oedema Acquired angioneurotic edema with C1Inh (C1 esterase inhibitor) deficiency Acquired angioneurotic edema with C1 inhibitor deficiency Acquired C1 esterase inhibitor deficiency (disorder) Acquired angioedema with C1Inh (C1 esterase inhibitor) deficiency Acquired angioedema Acquired angioneurotic oedema with C1Inh (C1 esterase inhibitor) deficiency |
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| Definitions |
A rare non-histaminic angioedema characterised by potentially life-threatening episodes of oedema of subcutaneous and/or mucosal tissues without urticaria, caused by excessive consumption of C1 esterase inhibitor (C1-INH) in the context of lymphoproliferative or autoimmune diseases. Patients typically present in the fourth decade of life or later and without a family history of angioedema. Clinical manifestation includes nonpitting oedema of the skin predominantly involving the face, but also the limbs or genitals, as well as abdominal pain due to involvement of the gastrointestinal mucosa and severe oedema of the upper airway and oral mucosa. Laboratory examination shows low C1-INH activity and low C3, C4, and C1q levels. Autoantibodies to C1-INH are frequently detectable. Acquired form of angioedema due to deficiency of C1 inhibitor A rare non-histaminic angioedema characterized by potentially life-threatening episodes of edema of subcutaneous and/or mucosal tissues without urticaria, caused by excessive consumption of C1 esterase inhibitor (C1-INH) in the context of lymphoproliferative or autoimmune diseases. Patients typically present in the fourth decade of life or later and without a family history of angioedema. Clinical manifestation includes nonpitting edema of the skin predominantly involving the face, but also the limbs or genitals, as well as abdominal pain due to involvement of the gastrointestinal mucosa and severe edema of the upper airway and oral mucosa. Laboratory examination shows low C1-INH activity and low C3, C4, and C1q levels. Autoantibodies to C1-INH are frequently detectable. |
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| ID |
http://purl.bioontology.org/ontology/SNOMEDCT/241955009 |
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| Active |
1 |
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| altLabel |
Acquired angioneurotic oedema with C1 inhibitor deficiency Acquired angio-oedema Acquired angioneurotic edema with C1Inh (C1 esterase inhibitor) deficiency Acquired angioneurotic edema with C1 inhibitor deficiency Acquired C1 esterase inhibitor deficiency (disorder) Acquired angioedema with C1Inh (C1 esterase inhibitor) deficiency Acquired angioedema Acquired angioneurotic oedema with C1Inh (C1 esterase inhibitor) deficiency |
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| CASE SIGNIFICANCE ID |
900000000000020002 900000000000448009 |
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| CTV3ID |
X70wB |
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| cui |
C2931758 |
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| definition |
A rare non-histaminic angioedema characterised by potentially life-threatening episodes of oedema of subcutaneous and/or mucosal tissues without urticaria, caused by excessive consumption of C1 esterase inhibitor (C1-INH) in the context of lymphoproliferative or autoimmune diseases. Patients typically present in the fourth decade of life or later and without a family history of angioedema. Clinical manifestation includes nonpitting oedema of the skin predominantly involving the face, but also the limbs or genitals, as well as abdominal pain due to involvement of the gastrointestinal mucosa and severe oedema of the upper airway and oral mucosa. Laboratory examination shows low C1-INH activity and low C3, C4, and C1q levels. Autoantibodies to C1-INH are frequently detectable. Acquired form of angioedema due to deficiency of C1 inhibitor A rare non-histaminic angioedema characterized by potentially life-threatening episodes of edema of subcutaneous and/or mucosal tissues without urticaria, caused by excessive consumption of C1 esterase inhibitor (C1-INH) in the context of lymphoproliferative or autoimmune diseases. Patients typically present in the fourth decade of life or later and without a family history of angioedema. Clinical manifestation includes nonpitting edema of the skin predominantly involving the face, but also the limbs or genitals, as well as abdominal pain due to involvement of the gastrointestinal mucosa and severe edema of the upper airway and oral mucosa. Laboratory examination shows low C1-INH activity and low C3, C4, and C1q levels. Autoantibodies to C1-INH are frequently detectable. |
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| DEFINITION STATUS ID |
900000000000074008 |
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| Effective time |
20020131 |
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| Has associated morphology | ||
| Has causative agent | ||
| notation |
241955009 |
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| Occurs in | ||
| prefLabel |
Acquired C1 esterase inhibitor deficiency |
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| Subset member |
447562003~MAPRULE~TRUE 6011000124106~MAPTARGET~D47.9 6011000124106~MAPADVICE~MAP SOURCE CONCEPT CANNOT BE CLASSIFIED WITH AVAILABLE DATA 6011000124106~MAPTARGET~ 6011000124106~MAPCATEGORYID~447638001 6011000124106~MAPGROUP~1 6011000124106~MAPGROUP~2 447562003~MAPADVICE~ALWAYS D84.1 6011000124106~MAPADVICE~IF ACQUIRED ANGIOEDEMA DUE TO C1 INHIBITOR AUTOANTIBODY CHOOSE T78.3XX? | EPISODE OF CARE INFORMATION NEEDED | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT | POSSIBLE REQUIREMENT FOR AN EXTERNAL CAUSE CODE 6011000124106~MAPADVICE~IF ACQUIRED ANGIOEDEMA DUE TO LYMPHOPROLIFERATIVE DISORDER CHOOSE T78.3XX? | EPISODE OF CARE INFORMATION NEEDED | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT | POSSIBLE REQUIREMENT FOR AN EXTERNAL CAUSE CODE 900000000000508004~ACCEPTABILITYID~900000000000549004 447562003~MAPGROUP~1 6011000124106~MAPRULE~IFA 703802001 | Acquired angioedema due to C1 inhibitor autoantibody (disorder) | 6011000124106~MAPTARGET~D84.1 447562003~CORRELATIONID~447561005 6011000124106~MAPRULE~IFA 703801008 | Acquired angioedema due to lymphoproliferative disorder (disorder) | 6011000124106~MAPCATEGORYID~447637006 900000000000509007~ACCEPTABILITYID~900000000000548007 900000000000508004~ACCEPTABILITYID~900000000000548007 447562003~MAPPRIORITY~1 6011000124106~MAPADVICE~ALWAYS D84.1 447562003~MAPCATEGORYID~447637006 6011000124106~MAPRULE~OTHERWISE TRUE 6011000124106~MAPADVICE~IF ACQUIRED ANGIOEDEMA DUE TO C1 INHIBITOR AUTOANTIBODY CHOOSE D84.1 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT 6011000124106~MAPCATEGORYID~447639009 447562003~MAPTARGET~D84.1 6011000124106~MAPPRIORITY~1 6011000124106~CORRELATIONID~447561005 6011000124106~MAPADVICE~IF ACQUIRED ANGIOEDEMA DUE TO LYMPHOPROLIFERATIVE DISORDER CHOOSE D47.9 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT 6011000124106~MAPRULE~IFA 703801008 | Acquired angioedema due to lymphoproliferative disorder | 6011000124106~MAPTARGET~T78.3XX? 6011000124106~MAPPRIORITY~3 900000000000509007~ACCEPTABILITYID~900000000000549004 900000000000497000~MAPTARGET~X70wB 6011000124106~MAPRULE~IFA 703802001 | Acquired angioedema due to C1 inhibitor autoantibody | 6011000124106~MAPPRIORITY~2 |
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| tui |
T047 |
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| Type ID |
900000000000003001 900000000000013009 |
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| subClassOf |