A 14 year old girl with a history of hypertension secondary to chronic nephritis presents with tongue swelling. Her physical exam is unremarkable aside from a swollen tongue and lips. Her phonation is abnormal due to the swollen tongue. She denies any history if allergic reactions to medications in the past. She has no vomiting, wheezing, cough, difficulty breathing or skin rashes. There are no fevers, joint swelling or oral lesions. She recently switched to a new blood pressure medicine that she cannot recall the name of, but knows that it is supposed to be good for her kidneys.
This is what her tongue looks like.
What is going on here?
Though she can’t recall the name of her new medicine she was likely placed on an angiotensin converting enzyme inhibitor (ACE inhibitor). They are known to case angioedema, which is is the rapid swelling of the dermis, subcutaneous tissue, mucosa and submucosal tissues. ACE inhibitors block the eponymous enzyme preventing it from degrading bradykinin, which is a potent vasodilator. The paucity of connective tissue in facial structures allows significant swelling to develop via fluid leak and the early development of edema.
What do we do now?
The rapid development of swelling of the airway is a medical emergency. Unless immediate interventions decrease the swelling drastically control of the airway is warranted. As this is predicted to be a difficult airway advanced measures are warranted such as nasal tracheal intubation and involvement of the most experienced incubator available. You can administer antihistamines, steroids and epinephrine but they may or may not help. Recent work has suggested that C1 esterase inhibitors and fresh frozen plasma may be effective. Patients with this condition should be monitored very closely, so admission to a facility and unit capable of advanced airway interventions is warranted.
