Lupus

A 28-year-old woman presented, and was admitted, to our hospital with a 2-day history of periumbilical postprandial abdominal pain with nausea and vomiting; she was receiving a maintenance regimen of low-dose prednisolone. Three years before admission at our hospital, she had been diagnosed with systemic lupus erythematosus (SLE) after presenting with malar rash, proteinuria, high-titer antinuclear antibodies, and elevated anti–double-stranded DNA antibodies. On admission to our hospital, she was afebrile and normotensive, and the physical examination revealed only diffuse abdominal distention and mild rebound pain without rigidity; occult testing of stool for the presence of blood was negative. A laboratory evaluation was unremarkable except for a platelet count of 90,000 per cubic millimeter, hypocomplementemia, and elevated levels of anti–double-stranded DNA antibodies. Antiphospholipid antibodies were not detected.
Computed tomography of the abdomen revealed diffuse circumferential wall thickening with submucosal edema of the entire small bowel, showing the "double halo," or "target," sign (Panels A and B, asterisks); there was no evidence of thrombosis, perforation, or infarction. The target sign is nonspecific but, in patients with active lupus, suggests the presence of inflammation or edema caused by small-vessel intestinal vasculitis. High-dose methylprednisolone (2 mg per kilogram of body weight per day) was administered, with the dose gradually tapered over a 2-week period; the patient had an excellent clinical response. There has been no recurrence, and her lupus is currently controlled with the use of prednisolone and azathioprine.

De-Feng Huang, M.D. Wei-Sheng Chen, M.D.
Taipei Veterans General Hospital Taipei 112, Taiwan
NEJM Volume 361:e3 July 16,2009 Number 3