Although approximately half of the patients with AIAI had prior presentations involving facial and/or oropharyngeal swelling, no patient had concomitant airway compromise. However, a cautionary approach must be taken, and respiratory status must be closely monitored in these patients. Patients should continue to receive nothing by mouth, and be given supportive care and adequate fluid resuscitation, and the ACE inhibitor should be discontinued. The possible development of hypovolemia and small bowel obstruction must be closely monitored in the acute setting. However, in all nine cases described, symptoms resolved within 24 to 48 h without the need for specific medical therapy. In particular, there is no role for antihistamine or corticosteroids during the acute symptomatic period. The reversible nature of the disorder is clearly revealed by the prompt resolution of symptoms once the diagnosis is made and the offending drug discontinued.
Follow-up care is crucial to establish a continued resolution of symptoms and to confirm the diagnosis. The long term management issues involve a life-long abstinence from ACE inhibitors and the use of an alternative agent(s). Angiotensin II receptor antagonists (ARA) are not suitable substitutes for ACE inhibitors in patients diagnosed with AIAI. Angioedema has also been reported with ARA, albeit with a lower incidence and/or severity, irrespective of a prior history of ACE inhibitor-induced angioedema. For example, 32% of patients with reported ARA-induced angioedema had experienced a prior episode of angioedema attributed to ACE inhibitor therapy. ARA, via an indirect pathway, may lead to inhibition of endogenous ACE activity. Unless the patient has documented C1-INH deficiency, the use of synthetic androgens (danazol or stanozolol) is not indicated. We recommend that a safety wrist bracelet be worn so that health professionals can be forewarned of the underlying medical condition.