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We describe a previously unreported condition of severe, recurrent lupus enteritis accompanied with severe hypocomplementemia as the initial and only presentation of systemic lupus erythematosus. Systemic lupus erythematosus should be suspected in any patient with computed tomography findings of enteral vasculitis or ischemic enteritis, even without lupus-related symptoms or signs; C3/C4 levels may be helpful in the differential diagnosis. If the symptoms do not improve after medical treatment, such as using steroid or cyclophosphamide pulse therapy, or necrosis and perforation of the intestines are highly suspected, surgical intervention should be considered.

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