The Use of Plasma-Derived Complement C1-Esterase Inhibitor Concentrate (Berinert®) in the Treatment of Angiotensin Converting Enzyme-Inhibitor Related Angioedema

Abstract

Angioedema of the upper airways is a severe and potentially life-threatening condition. The incidence has been increasing in the past two decades, primarily due to pharmaceuticals influencing the generation or degradation of the vasoactive molecule bradykinin. Plasma-derived C1-esterase inhibitor concentrate is a well-established treatment option of hereditary and acquired complement C1-esterase inhibitor deficiency, which are also mediated by an increased level of bradykinin resulting in recurrent angioedema. We here present a case of severe angiotensin converting enzyme-inhibitor related angioedema (ACEi-AE) of the hypopharynx that completely resolved rapidly after the infusion of plasma-derived C1-inhibitor concentrate adding to the sparse reports in the existing literature.

C1 Esterase Inhibitor (Berinert) for ACE Inhibitor-Induced Angioedema: Two Case Reports

“…11 A third published case report successfully used a dose of 18 U/kg for a total dose of 2000 U. 13 In both of our cases, administration of the C1INH improved symptoms quickly, but in the first case, tongue swelling returned 4 hours later and the patient was agitated. The agitation may have been due to the history of alcohol intake.…”

“…9 A literature search of English-language publications in the past 10 years yielded documentation that medications that are used for hereditary angioedema (HAE) have been utilized in ACEI-induced angioedema. 8,[10][11][12][13][14] Icatibant is a selective bradykinin B2 receptor antagonist and is FDA approved for the treatment of HAE attacks. This medication competes with bradykinin for receptors, thereby mitigating the vasodilatory effects of bradykinin that may lead to angioedema.…”

“…15 C1INHs are FDA approved for HAE prophylaxis 16 and treatment 17 and have also been used in case reports of ACEI-induced angioedema. [11][12][13][14] C1INHs inhibit the enzyme kallikrein, 17,18 which cleaves kininogen to form bradykinin. 4 According to the Naranjo algorithm to assess ADR probability, the angioedema described in case 1 was estimated to probably be an ADR related to lisinopril with a score of 7.…”