Isolated Visceral Angioedema: An Uncommon Complication of ACEI Therapy

Abstract

Angiotensin Converting Enzyme Inhibitors (ACEIs) are commonly prescribed medicines with well-known side effects. We report a lesser known side effect with a stereotypical presentation, “Isolated Visceral Angioedema” (IVAE). This can occur within hours to years after initiation of ACEIs with a strong female predominance clinically presenting with variable levels of abdominal pain, emesis, and diarrhea. Abdominal CT shows diffuse small bowel thickening and ascites. Recognition of this presentation will prevent unnecessary maneuvers including endoscopic procedures and surgical intervention. A 45-year-old white female with hypertension and hypothyroidism was taking Lisinopril (for 18 months), Levothyroxine and oral contraceptives. She experienced her first episode of abdominal pain with nausea and vomiting six months prior to presentation which spontaneously resolved. She described six additional episodes over the next six months leading to her presentation with severe abdominal pain which brought her into the hospital. At presentation she was in moderate to severe abdominal pain, vital signs were stable and abdominal exam demonstrated diffuse abdominal tenderness without rebound. CBC showed only a WBC of 17.7, chemistries were normal. CT abdomen and pelvis revealed thickening of her distal jejunum and proximal ileum spanning 30 cm in length and ascites. Negative stool culture, ova and parasites, and C. Difficile. C1 esterase inhibitor, C3 and C4 complement levels were normal. Treatment consisted of discontinuing her Lisinopril. Repeat abdominal and pelvic CT two weeks later, showed complete resolution of small bowel thickening and ascites. To date, 36 months later, the patient has had no recurrence of symptoms. Diagnosis of IVAE is based on the triad of typical symptoms (abdominal pain, nausea, vomiting and diarrhea); typical CT findings of thickened small bowel and ascites; and history of ACEI use. This occurs in the absence of peripheral manifestations of angioedema (lip or tongue swelling, urticaria, and respiratory symptoms). The differential of small bowel thickening and ascites includes: infections, ischemia, inflammation (Crohn's) and neoplastic causes. IVAE commonly resolves after recognition via history and imaging studies with immediate cessation of ACEIs followed by NPO and IVF hydration, and possible fresh frozen plasma used as second line agent in complicated refractory cases.Figure: Abdominal oral and IV contrast CT showing peri-hepatic ascites and thickening of the stomach walls.Figure: Abdominal oral and IV contrast CT showing peri-hepatic ascites with thickened stomach walls.Figure: Abdominal oral and IV contrast CT showing visible thickening of the intestinal wall.