368: ANGIOEDEMA PREVENTING EXTUBATION IN A CASE OF ACQUIRED C-1 ESTERASE DEFICIENCY

Abstract

Introduction: Acquired C1 esterase deficiency is a rare, life-threatening phenomenon that has been reported in association with lymphoproliferative and autoimmune disorders. Diagnosis is made based on the clinical presentation of angioedema and low C1 and C1q esterase inhibitor activity in a patient without a previous hereditary diagnosis. Treatment is aimed at addressing the underlying condition and plasma-derived C1 esterase inhibitor (C1-INH) concentrate, but clinical response can vary. Description: 75 year-old female with a past medical history of chronic lymphocytic leukemia (CLL) presented with spontaneous mouth and neck swelling unrelieved by antihistamines. Epinephrine and Solumedrol were administered, however the neck swelling worsened and the patient was intubated for airway protection. CT imaging of the neck revealed extensive subcutaneous neck edema. Attempts at extubation were limited by the patient’s persistent angioedema which was visualized by bronchoscopy. Glucocorticoids were ineffective as the patient had a reported allergy to Prednisone, therefore were further avoided. C1 esterase inhibitor (C1-INH) function was initially low at 7mg/dL, and repeat remained low at 10mg/dL with a C4 of .7mg/dL. C1q later returned low at < 1.2Eq/mL. The patient did not improve after treatment with plasma-derived C1-INH (Berinert). She eventually required tracheostomy placement, but had difficulty with subsequent downsizing due to persistent laryngeal edema with limited therapeutic options. Eventually, after 4 weeks, the patient was successfully decannulated. Discussion: There are no definitive guidelines for treatment of acquired C1 esterase deficiency, however therapies often follow the same course for hereditary forms including plasma-derived C1-INH. Often, these patients may not respond or require increased doses. If Berinert is ineffective, some immunologists and allergists have seen favorable outcomes and recommend treatment with the kallikrein inhibitor, ecallantide and the bradykinin B2 antagonist icatibant. To date, therapies targeted at prevention of angioedema in acquired cases have not been as successful as in hereditary forms, therefore, treatment is directed at the underlying condition, such as immunotherapy or chemotherapy for CLL.