Visceral muscle dysmotility syndrome in systemic lupus erythematosus: case report and review of the literature

Abstract

Intestinal pseudo-obstruction (IPO) is not uncommon in systemic lupus erythematosus (SLE), and IPO in SLE has an apparent association with ureterohydronephrosis. However, hepatobiliary dilatation without mechanical obstruction presenting together with IPO and ureterohydronephrosis is much more scarce in SLE. Here, we named this rare triad of IPO, ureterohydronephrosis, and biliary tract dilatation as visceral muscle dysmotility syndrome (VMDS). It always imitates an acute abdomen and is even life-threatening if treated incorrectly. To diagnose a VMDS, infections and mechanical obstructions should be ruled out carefully. Here, we report a 24-year-old Chinese woman with SLE who presented of VMDS that associated with corticoids tapering induced SLE flare. In this case, early vigorous immunosuppressive treatment conquered the triad timely and thus yielded a good outcome.