A Case of Pneumatosis Intestinalis Caused by Systemic Lupus Erythematous

Abstract

Introduction: Pneumatosis intestinalis (PI) is a radiographic finding with wide spectrum of presentation, ranging from asymptomatic to perforation and death. There are more than 60 known causes of PI, but systemic lupus erythematous is a rare cause, and infrequently described. Case 42 year old woman was admitted with non-bloody diarrhea and left upper quadrant abdominal cramps for one week. She has a history of GERD (status post fundoplication x3), chronic constipation due to pelvic floor dysfunction, SLE with autonomic instability, resulting in hypotension, syncope, and bradycardia requiring pacemaker placement. Her symptoms occurred in the setting of discontinuing her lupus medications (belimumab and leflunomide), tapering her prednisone, and holding IVIG to repeat testing for autonomic dysfunction, as she had worsening episodes of hypotension and bradycardia in Fall 2016, requiring pacemaker placement. She had a similar presentation in 2015, when she was found to have PI in the splenic flexure. Infectious work up was negative at that time, and symptoms did not improve with a week of IV antibiotics. It then resolved with prednisone taper for presumed SLE exacerbation, resulting in PI. CT abdomen this time showed increased prominence of PI at the splenic flexure, a watershed area. She was placed on bowel rest, given high flow oxygen, and started on metronidazole, but remained symptomatic with nausea, bloating and diarrhea. Colonoscopy showed melanosis coli without evidence of colitis or bowel perforation. Patient then improved with a higher dose of prednisone. Discussion SLE is a rare cause of PI, but could contribute to it through several different mechanisms. A mechanism that was proposed by previous case report was SLE causing enteric vasculitis. Antiphospholipid antibodies and/or lupus anticoagulant may affect the gut mucosa resulting in ischemia, although this patient did not have these antibodies. The patient's autonomic dysfunction may have resulted in hypotensive episodes that could damage the watershed area.