Visceral muscle dysmotility syndrome in systemic lupus erythematosus: which is the role of 18 fluorodeoxyglucose-positron emission tomography-computed tomography? A clinical case and literature review.

Abstract

Visceral muscle dysmotility syndrome (VMDS) is a rare syndrome, described in the systemic lupus erythematosus (SLE) clinical course. It is characterized by diffuse thickened intestinal wall and gastrointestinal-genitourinary-hepatobiliary hollow viscera dilatation and dysmotility. Due to the rarity and the heterogeneity of the clinical characteristics of this syndrome, it is not entirely clear which is the best diagnostic imaging technique for the diagnosis and/or follow-up, even if, in all the described cases, computed tomography (CT) was generally used to study visceral involvement. However, there are no cases describing the visceral metabolic activity by 18 fluorodeoxyglucose (18FDG)-positron emission tomography-CT (18FDG-PET-CT). Here, we reported the first clinical case of VMDS studied by 18FDG-PET-CT, characterizing the metabolic activity of this rare syndrome during SLE flare. We found a high intestinal metabolic burden, hyper-fixation in duodenum, and high hepatic metabolic activity. Moreover, we reviewed the literature on VMDS in SLE, focusing on imaging techniques in different anatomical sites (bowel, urinary tract, bile ducts), patients' symptoms, and treatment.