title: 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.
authors: Department of Nephrology and Rheumatology, Sixth People's Hospital, Shanghai Jiao Tong Unversity, 600 Yishan Road, 200233, Shanghai, China.
authors: Yu Qiang
authors: YQ
authors: Chen
authors: Qing
authors: Q
authors: Xue
authors: Nian Song
authors: NS
authors: Wang
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D001659:Biliary Tract / Q000473:pathology; D049448:Cholangiopancreatography, Magnetic Resonance; D004108:Dilatation, Pathologic; D004334:Drug Administration Schedule; D005260:Female; D005769:Gastrointestinal Motility; D006801:Humans; D006869:Hydronephrosis / Q000209:etiology; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007418:Intestinal Pseudo-Obstruction / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014515:Ureteral Diseases / Q000209:etiology; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents