Lupus Enteritis in a Young Female: A Rare Disease Requiring a Rapid Diagnosis

Abstract

Intro: Though systemic lupus erythematosus (SLE) frequently involves the gastrointestinal tract, lupus enteritis (LE) is a rare manifestation of the autoimmune disease and can have catastrophic effects if missed. We present a case of LE to raise physician awareness of an uncommon disease. Case: A 21-year-old female with newly diagnosed SLE and class V lupus nephritis presented to the ER with a 4 day history of nausea, non-bilious, non-bloody vomiting, abdominal pain and decreased appetite. Symptoms began within 1 week of initiating treatment with mycophenolate mofetil and prednisone 50mg. Physical exam was notable for mild abdominal distention with diffuse tenderness on palpation, and a faint malar rash. Labs were notable for normocytic anemia, paraprotein gap of 5mg/dL, nephrotic range proteinuria, and elevated levels of CRP 1.92mg/dL, ESR 29mm/Hr, double-stranded DNA antibody 39 IU/mL, and anticardiolipin IgM 12.8 MPL. CT abdomen with contrast demonstrated ileitis with marked wall thickening and stranding involving multiple ileal loops from the mid jejunum to terminal ileum, engorgement of mesenteric vessels, and a 0.9 x 1.2 x 1.6 inferior vena cava thrombus between the liver and right atrium. She was to follow up as an outpatient for colonoscopy. For lupus nephritis and enteritis, she was treated with 3 days of pulse dosed steroids with immediate improvement of symptoms. She was discharged on day 5, with complete symptom resolution on a steroid taper. Discussion: The British Isles Lupus Assessment Group define LE as a vasculitis or inflammation of the small bowel with supportive image or biopsy findings. As seen in our patient, LE most often presents with abdominal pain and vomiting. Abdominal CT is the gold standard for diagnosis and images show bowel wall thickening and dilatation, engorgement of mesenteric vessels, and increased attenuation of mesenteric fat. The disease is reversible and steroid responsive. First line treatment in a severe flare involves IV corticosteroids. Cyclophosphamide and mycophenolate can be added in cases of steroid resistance, enteritis recurrence, or as in our case, if additional organs are involved. If unrecognized, LE may result in perforation and bowel necrosis. Knowledge of this rare complication of SLE is paramount to the diagnosis, treatment, and prevention of potentially fatal complications. As depicted in our case, rapid detection and treatment results decreased hospital length of stay, morbidity, and mortality.Figure: CT (axial view). Marked wall thickening and inflammatory stranding involving terminal ileum.Figure: CT (coronal view). Marked thickening and surrounding inflammatory stranding in the small bowel with engorgement of mesenteric vessels.