ACQUIRED ANGIOEDEMA – AN UNUSUAL PRESENTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS

Abstract

Introduction Acquired angioedema requires evaluation for underlying etiologies including lymphoproliferative processes and autoimmune conditions. We present a patient with recurrent angioedema that was found to have systemic lupus erythematosus (SLE). Angioedema was her only presenting symptom. She denied other common signs and symptoms of SLE. Case Description The patient is a 30-year old Hispanic female with obesity and 3-4 episodes of facial angioedema in the past 6 months. She was hospitalized for severe acute abdominal pain and emesis with no other associated symptoms. The patient was not on ACEI and has no family history of angioedema. Physical examination revealed diffuse abdominal tenderness. Imaging showed small bowel edema. Laboratory evaluation revealed leukocytosis and mild elevation of transaminases. Due to history of recurrent facial angioedema, further work-up was done that showed low C1 esterase inhibitor levels and decreased function, low C1q, low C3 and C4, positive antinuclear antibodies (ANA) and Anti- SSA/Anti-SSB antibodies, suggesting underlying SLE. She denied any previous diagnosis of SLE and no signs/symptoms consistent with autoimmune conditions. She was started on C1 esterase inhibitor replacement prophylactically and has icatibant for acute attacks. She was also started on hydroxychloroquine for the underlying SLE. Discussion Acquired C1 esterase deficiency should prompt a broad evaluation for an underlying disorder. Our case suggests the consideration of an autoimmune disorder in the differential diagnosis, especially in young women who have no other supporting features.