Lupus enteritis is a rare manifestation of systemic lupus erythematous and is considered as a form of visceral vasculitis involving predominantly the jejunum and ileum, with subsequent submucosal edema. Clinical presentation is nonspecific, and main symptoms include focal or diffuse abdominal pain, vomiting, diarrhea, and fever. Even if there is no pathognomonic imaging feature of lupus enteritis, computed tomography may be suggestive. Typical computed tomography features that have been reported are bowel wall thickening over 3 mm (“target sign”) with abnormal bowel-wall contrast enhancement, dilatation of intestinal segments, and engorgement of mesenteric vessels (“comb sign”).
We report a case of lupus enteritis that was the presentation of a systemic lupus erythematous relapse, conferring a challenging diagnosis in which ultrasound examination was helpful for the detection of bowel edema.
A 31-year-old woman was admitted to Service de Néphrologie-Dialyse-Transplantation, CHU Angers with an acute history of diffuse abdominal pain, vomiting, and asthenia. Her medical history included cutaneous discoid lupus for 10 years that was treated with 5 mg/d of oral prednisone, without other organ involvement or antiphospholipid syndrome. Emergency abdominal ultrasound revealed an uncommon aspect of diffuse edema of the jejunum and moderate ascites. Abdominal ultrasound was completed by abdominal computed tomography that confirmed a diffuse jejunal wall thickening with intense bowel wall contrast enhancement (“target sign”). An upper endoscopy showed normal esophageal and gastric mucosa, but duodenal mucosa was punctuated with multiple unspecific deposits, without erosion or ulcer. The histologic and microbiological samples were normal.
At the same time, the patient developed rapidly progressive renal failure, from a serum creatinine at 50 μmol/L at baseline up to more than 300 μmol/L to maximum, with class IIIA proliferative lupus glomerulonephritis on renal biopsy. Autoimmunity screening was positive for anti-dsDNA antibodies and complement consumption. Diagnosis of lupus flare with kidney involvement and lupus enteritis was retained.
We initiated treatment with bowel rest and intravenous methylprednisolone pulses (500 mg/d) for 3 days followed by 1 mg/kg/d of prednisone and 3 g/d of mycophenolate mofetil. Under this regimen, digestive symptoms resolved quickly within 7 days and renal failure improved progressively with a serum creatinine of 65 μmol/L at day 14.
In lupus enteritis, endoscopic biopsies most often are of poor contribution to diagnosis and show normal histology because only superficial tissue is analyzed. It is worth noting that abdominal ultrasound allowed early detection of bowel edema in our case. Therefore, in patients with known systemic lupus erythematous, we suggest that abdominal ultrasound may be a simple and rapid tool to diagnose bowel edema suggestive of lupus enteritis, especially in the context of renal failure permitting to avoid contrast use. However, in some cases, complications such as bowel perforation or mesenteric ischemia may occur. If suspected, a screening with computed tomography remains necessary.
Even if lupus enteritis has been rarely described as the first presentation of systemic lupus erythematous, it is reported as a challenging diagnosis in such cases. The treatment of lupus enteritis relies on complete bowel rest and steroid treatment, usually with complete remission.
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-Julien Demiselle, MD, Johnny Sayegh, MD, Maud Cousin, MD, Anne Olivier, MD, Jean-François Augusto, MD, PhD
This article originally appeared in the May 2016 issue of The American Journal of Medicine.
