Department of Internal Medicine, Infectious Diseases, and Clinical Immunology, Reims Teaching Hospitals, Reims, France; University of Reims Champagne-Ardenne, EA-4684 / SFR CAP-SANTE, Reims F-51095, France. Electronic address: [Email]
Autoimmune autonomic ganglionopathy (AAG) is a rare disease with no well-established treatment. Until recently, AAG could be seropositive (50 to 60% of patients) or seronegative for ganglionic (α3-type) nicotinic acetylcholine receptor (Gα3NAChR) antibodies. In early 2018, the two forms of the disease were distinguished, separating seropositive from seronegative ones, designating this latter form "seronegative autoimmune autonomic neuropathy" (SAAN). Most described treatments are plasma exchange (PE) and intravenous immunoglobulin (IVIG). However in some cases with no or small benefit, other immunomodulatory therapies, such as rituximab have been reported. We report the case of a 24-year-old female patient successfully treated for SAAN with rituximab and steroids after IVIG and PE failure. We also provide a review of case-reports reporting rituximab treatment for both SAAN and AAG.