A 53-year-old woman has recurrent severe Abdominal pain. Imaging shows bowel wall edema, but her exam is normal. What rare condition could be the cause?
A previously healthy 53-year-old woman presented with a recurrent episode of severe, diffuse abdominal pain. During the previous month, she had been having sudden, self-limited bouts of abdominal pain twice per week. She had no history of swelling of the face or limbs and had not been taking an angiotensin-converting–enzyme inhibitor. There was no family history of angioedema. An abdominal examination was normal. CT of the abdomen revealed segmental thickening of the walls of the colon and rectum and mesenteric edema. A colonoscopy revealed edema of the entire colon. No abnormalities in the colonic mucosa were detected on biopsy. Laboratory testing showed low serum levels of C4, C1 inhibitor antigen, and C1q on repeated measurements, as well as decreased C1 esterase function. A diagnosis of angioedema of the intestines was made. Which of the following is the most likely cause?
What is the most likely diagnosis?
Abdominal pain, Acquired C1 inhibitor deficiency, Angioedema, C1q, Complement System
