Case Presentation: A 22 year-old female student with no medical history presented to Ohio State Medical Center complaining of throbbing epigastric pain, increased abdominal girth, and nausea after ingesting two bottles of wine. Patient denied obstructive symptoms, BRBPR, melena, fevers, recent travel, sick contacts, medication use, new or unusual foods. No family history. Clinical exam was notable only for pain in the epigastrium with guarding. Labs showed a WBC of 12.8, Sodium of 128, Cr of 1.11, and normal lipase. Patient had a CT of the abdomen performed which showed dilation and thickening of the jejunal and ileal loops with associated ascites. Diagnostic paracentesis was performed which ruled out SBP. PPD was checked and returned negative. While hospitalized, patient’s pain progressed to “pain worse than death.” Therefore, a CTA of the abdomen was performed, which again showed small bowel wall thickening and enlargement of mesenteric vessels without evidence of vasculitis. ESR and CRP were checked and noted to be normal. GI was consulted and performed a push enteroscopy with biopsies of the jejunum and ileum. Biopsies returned with increased intraepithelial lymphocytes and focal active inflammation without signs of lymphoma or infection. Rheumatological panel was sent and returned positive for ANA, c-ANCA, Low C3 and C4, Smith Ab, RNP Ab, SS-A, SS-B, DS DNA Ab.
Rheumatology was consulted. Patient was re-evaluated for any clinical signs of lupus, without any notable irregularities. Based on lab abnormalities and imaging, patient was diagnosed with lupus enteritis as a presenting sign of SLE and started on high dose steroids with improvement in her symptoms within 48 hours.
Discussion: Uncommon to be the sole presenting symptom of SLE, lupus enteritis manifests as abdominal pain, ascites, nausea, vomiting, and diarrhea. Lupus enteritis typically affects the jejunum and ileum. Laboratory abnormalities most commonly noted are: positive ANA, ds-DNA, anti-RNP, anti-SSA, anti-Sm, low complements, anemia, leukocytopenia, and thrombocytopenia. ESR & CRP are commonly normal. Key imaging findings on CT include: Target Sign – bowel thickening of greater than 3 mm and dilatation, Comb Sign – engorgement of mesenteric vessels, and Increased attenuation of mesenteric fat. Unfortunately, key imaging findings can also be seen in patients with pancreatitis or IBD.
Diagnosis is based on laboratory and imaging findings. Thankfully once diagnosed, lupus enteritis is reversible. Corticosteroids are first line agents with Cyclophosphamide or Mycophenolate Mofetil being added in cases with corticosteroid resistance or with CNS or renal involvement.
Conclusions: Lupus enteritis although uncommon, can be a presenting feature of lupus. These cases commonly present with abdominal pain, ascites, and jejunal and/or ileal inflammation. Diagnosis can be based on lab abnormalities and imaging findings. Once diagnosed most cases are steroid responsive with great prognosis.
1.) Janssens P, et al. Lupus enteritis: from clinical findings to therapeutic management. Orphanet Journal of Rare Disease. 2013;8:67.
2.) Smith LW, Petri M. Lupus Enteritis: an uncommon manifestation of systemic lupus erythematosus. J Clinical Rheumatology. 2013;19(2):84-86.
3.) Lee CK, Ahn MS, Lee EY, et al. Acute abdominal pain in systemic lupus erythematosus: focus on lupus enteritis (gastrointestinal vasculitis). Ann Rheum Dis. 2002;61:547–550.