Angioedema as a presenting feature in a patient with SLE : a case report

Abstract

Systemic lupus erythematosus (SLE) is a multi-organ autoimmune disease that can be easily missed due to its variable presentation. Acquired angioedema (AAE) is a rare first presentation of SLE. We report a case of a 23-year-old woman who presented to the emergency department with rapidly progressive swelling of the tongue and neck, followed by respiratory discomfort and a generalized non-itchy rash. A tracheostomy was performed to relieve her symptoms. Her history, examination, and relevant investigations all indicated SLE. She was treated with high-dose steroids, and pulse therapy with methylprednisolone was given for three days. Hydroxychloroquine was added, but she developed sepsis and an acute flare of SLE secondary to tracheostomy site infection. Subsequently, she was treated with broad-spectrum antibiotics, followed by a tapering dose of steroids and a maintenance dose of azathioprine. She responded to treatment, tracheostomy reversal was performed, and the patient was discharged.