Hereditary angioedema presenting in late middle age after angiotensin-converting enzyme inhibitor treatment.
BACKGROUND
Angioedema due to Cl esterase inhibitor (CI-INH) deficiency may be hereditary (HAE), commonly first occurring in childhood, or acquired (AAE), with onset usually in middle age. Type I HAE exhibits low levels of functionally normal C1-INH. Dysfunctional Cl-INH typifies type II HAE. Patients with type I AAE have low levels of Cl-INH, Clq complement, and C4 complement. In type II AAE, there is immune blockade of C1-INH.


OBJECTIVE
To describe a man who first presented at the age of 52 years with type I HAE triggered by the administration of an angiotensin-converting enzyme (ACE) inhibitor.


METHODS
The patient had C1-INH levels, a complement profile, and a lack of underlying co-pathology that led to the diagnosis of type I HAE triggered by the administration of an ACE inhibitor.


RESULTS
The patient presented with life-threatening angioedema. His C4 complement and Cl-INH serum levels were below the reference ranges, and his C3 complement, total hemolytic complement assay, and Clq complement levels remained within the reference ranges. During the 10 years between his initial episode of angioedema and the second, he had not developed any secondary medical conditions, and he had been taking the ACE inhibitor lisinopril for 7 years.


CONCLUSIONS
Physicians must remain aware of the possibility of unmasking HAE in the adult and geriatric population with the common use of ACE inhibitors for the treatment of hypertension, cardiovascular diseases, and metabolic diseases.
Ricketti
Anthony J
AJ
Department of Medicine and Section of Allergy and Immunology, St Francis Medical Center, Trenton, New Jersey 08629, USA.
Cleri
Dennis J
DJ
Ramos-Bonner
Luz S
LS
Vernaleo
John R
JR
D000328:Adult; D000368:Aged; D000799:Angioedema / Q000139:chemically induced* / Q000378:metabolism; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000008:administration & dosage / Q000009:adverse effects*; D050718:Complement C1 Inhibitor Protein / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D013997:Time Factors
D000806:Angiotensin-Converting Enzyme Inhibitors; D050718:Complement C1 Inhibitor Protein