Angioedema of the tongue due to acquired C1 esterase inhibitor deficiency.
We describe the management of an 83-year-old woman who presented with upper airway obstruction due to angioedema of the tongue. Following definitive airway management, investigation showed a diagnosis of acquired C1 esterase inhibitor deficiency (acquired angioedema) that was considered to be subsequent to haematological malignancy. Resolution of the macroglossia followed treatment with C1 esterase inhibitor concentrate, but the patient failed to wean from ventilatory support and died in the Intensive Care Unit. This case report highlights the potential for acquired angioedema to cause upper airway obstruction. The various treatment modalities for acquired C1 esterase inhibitor deficiency are summarized.
Dobson
G
G
The Ulster Hospital, Belfast, Northern Ireland.
Edgar
D
D
Trinder
J
J
D000368:Aged; D000369:Aged, 80 and over; D000402:Airway Obstruction / Q000209:etiology*; D000799:Angioedema / Q000150:complications* / Q000209:etiology; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000150:complications; D008260:Macroglossia / Q000150:complications* / Q000188:drug therapy / Q000201:enzymology
D003174:Complement C1 Inactivator Proteins