The importance of recognizing and managing a rare form of angioedema: hereditary angioedema due to C1-inhibitor deficiency

Postgrad Med. 2021 May 17. doi: 10.1080/00325481.2021.1905364. Online ahead of print.ABSTRACTThe majority of angioedema cases encountered in clinical practice are histamine-mediated (allergic); however, some cases are bradykinin-related (non-allergic) and do not respond to standard anti-allergy medications. Among bradykinin-related angioedema, hereditary angioedema (hae) is a rare, but chronic and debilitating condition. The majority of hae is caused by deficiency (type 1) or abnormal function (type 2) of the naturally occurring protein, c1-inhibitor (c1-inh)-a major inhibitor of proteases in the contact (kallikrein-bradykinin cascade), fibrinolytic pathway, and complement systems. Failure to recognize hae and initiate appropriate intervention can lead to years of pain, disability, impaired quality of life (qol) and, in cases of laryngeal involvement, it can be life-threatening. Hae must be considered in the differential diagnosis of non-urticarial angioedema, particularly for patients with a history of recurrent angioedema attacks, family history of hae, symptom onset in childhood/adolescence, prodromal signs/symptoms before swellings, recurrent/painful abdominal symptoms, and upper airway edema. Management strategies for hae include on-demand treatment for acute attacks, short-term prophylaxis prior to attack-triggering events/procedures, and long-term or routine prophylaxis for attack prevention. Patients should be evaluated at least annually to assess need for routine pro...
Source: Postgraduate Medicine - Category: Internal Medicine Authors: Source Type: research