Usefulness of C1 esterase inhibitor protein concentrate in the management of hereditary angioedema of oropharyngeal tissue

Abstract

Hereditary angioedema is an autosomal‑dominant disorder caused by mutation of the gene encoding the C1 esterase inhibitor (C1‑INH). It manifests as painless, nonpruritic, nonpitting episodic swelling of the subcutaneous tissues, gastrointestinal, and upper respiratory tracts in the absence of urticaria. An attack typically takes 24 h to peak and resolves over 48–72 h. The most serious manifestation is a laryngeal attack associated with upper airway swelling. The aim of this case report is to describe the lifesaving use of a novel C1‑INH protein concentrate in a patient with  mild‑to‑moderate dyspnea caused by swelling of the upper airway (larynx) and tongue.

Keywords: C1 esterase inhibitor protein, hereditary angioedema, laryngeal edema, oropharyngeal swelling