Laryngeal angioedema caused by a rare disease—A case report

Abstract

Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare disease, which often appears with hematological underlying diseases. It can be diagnosed by complement testing and its treatment consists of the management of the underlying disease and angioedema attacks.The patient (59 year-old male) who was taking angiotensin-converting-enzyme inhibitor, was referred to the Hungarian Angioedema Center of Reference and Excellence due to an upper airway edema which did not respond to conventional treatment and required tracheotomy. At our Center, C1-INH-AAE was diagnosed, and myeloma multiplex was found as an underlying disease. He have been followed up for the past 15 years.After the first treatment series (chemotherapy and stem cell transplantation), his complement parameters returned to normal, but 5 years later they started to deteriorate, and the angioedema attacks recurred. To avoid the recurrence of myeloma multiplex, and due to the frequent and serious angioedema attacks, the patient got another series of rituximab treatment. It resulted in the normalization of complement values, and he had no further angioedema attacks.Angiotensin-convertase-enzyme inhibitors can trigger C1-INH-AAE.The recurrence of the underlying disease or the angioedema attacks can be predicted by the regular follow up of the complement parameters. Thus, the treatment of the underlying disease can be started in time, before the onset of severe symptoms.

Keywords

• Acquired angioedema due to C1-inhibitor deficiency
• Long term follow up
• C1-inhibitor antibodies
• Classical complement pathway
• Myeloma multiplex
• Case report