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Recurrent Abdominal Pain in Systemic Lupus Erythematosus: Concurrent Lupus Enteritis and Lupus Cystitis

Contrast-enhanced abdominal computed tomography scan. (A) Diffuse bowel wall thickening of 13 mm showing the “target sign” (arrows). (B) Engorgement of mesenteric vessels or ‘‘comb sign’’ (arrow head) and bladder wall thickening (asterisk).

A 44-year-old woman was admitted to the hospital in March 2017 with a 5-day history of abdominal pain, vomiting, diarrhea, dysuria, and urinary frequency. She was afebrile, normotensive, and in sinus tachycardia. Her abdomen was distended and diffusely tender. Bowel sounds were hyperactive. No abdominal guarding or rebound pain in response to deep palpation was elicited.

She was diagnosed with systemic lupus erythematosus in 2005 due to the presence of malar rash, polyarthritis, lymphopenia, and positive antinuclear, anti-double-stranded DNA and anti-Sm antibodies. From May 2010 to August 2016, she experienced 4 episodes of lupus enteritis. These episodes were characterized by abdominal pain, vomiting, and diarrhea; abdominal computed tomography scans demonstrated diffuse mucosal edema of the bowel wall involving the jejunum and ileum. The first 2 episodes responded to high-dose oral prednisolone followed by azathioprine as maintenance therapy. The third episode was treated with high-dose glucocorticoids and a 6-month course of intravenous cyclophosphamide pulses followed by azathioprine. The fourth episode was treated with high-dose glucocorticoids and azathioprine. All episodes recurred after decreasing the dose of azathioprine.

On admission, laboratory findings demonstrated lymphopenia, hypocomplementemia, normal kidney function tests, an inactive urinary sediment, and a negative urine culture. Contrast-enhanced abdominal computed tomography showed diffuse multifocal bowel wall thickening of 13 mm involving the small bowel and the large bowel, dilatation of intestinal segments, engorgement of mesenteric vessels, and bladder wall thickening (Figure). Based on these findings, recurrent lupus enteritis and lupus cystitis were diagnosed. She was treated with intravenous methylprednisolone pulses for 3 days, followed by oral prednisone (1 mg/kg/day for 4 weeks followed by tapering it to 10 mg/day) and a 6-month course of intravenous cyclophosphamide pulses (1 g each) with marked improvement. Cyclophosphamide was switched to azathioprine (2 mg/kg/day) for maintenance; after 18 months of follow-up her gastrointestinal and urinary symptoms remained under control.

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-Pablo Andrés Castaño-González, MD, Luis Alonso González, MD

This article originally appeared in the May 2019 issue of The American Journal of Medicine.

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