A case of seropositive autoimmune autonomic ganglionopathy treated with rituximab: Case report and review of the literature.

Autoimmune autonomic ganglionopathy (AAG) is an acquired disease characterized by the dysfunction of the autonomic system, involving the sympathetic, parasympathetic and enteric divisions. We describe the case of a 49-years-old woman who presented with an acute abdomen due to diverticulitis and a severe orthostatic hypotension. During hospitalization she developed fixed mydriasis, xerophthalmia, xerostomia, anhidrosis, and urinary retention. The patient was diagnosed as seropositive AAG. She was treated with intravenous immunoglobulin (IVIg) plus high-dose oral prednisone (OP), followed by plasma exchange (PLEX). The patient showed a good response to the combined therapy and was discharged after nearly one month of hospitalization. Ten days later she experienced a symptomatic relapse, for which a treatment with rituximab (RTX) 1 g once a week was initiated and maintained for 4 weeks. During the second week of infusion, she was readmitted to the hospital due to intestinal perforation. After recovery, RTX was resumed with clinical benefit. In addition to the case report, we present a review of AAG case reports available in the literature.