Autoimmune autonomic ganglionopathy: treatment by plasma exchanges and rituximab.
This case report describes successful treatment of autoimmune autonomic ganglionopathy (AAG) in a 74-year-old woman by total plasma exchanges (PLEX) and rituximab. Two series of PLEX temporarily, but dramatically improved orthostatic intolerance and hypotension and baroreflex function, in a manner inversely related to ganglionic neuronal nicotinic receptor antibody titer. After rituximab treatment, the antibody titer was decreased modestly but persistently, and the patient had symptomatic and clinical laboratory evidence of continued benefit for at least 10 months, supporting the autoimmune pathogenesis of AAG.
Imrich
Richard
R
Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, Bethesda, MD 20892-1620, USA. richard.imrich@savba.sk
Vernino
Steven
S
Eldadah
Basil A
BA
Holmes
Courtney
C
Goldstein
David S
DS
D000368:Aged; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000097:blood; D020274:Autoimmune Diseases of the Nervous System / Q000628:therapy*; D017704:Baroreflex / Q000502:physiology; D005260:Female; D005725:Ganglia, Autonomic / Q000276:immunology*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D010951:Plasma Exchange; D011978:Receptors, Nicotinic / Q000276:immunology; D000069283:Rituximab
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007155:Immunologic Factors; D011978:Receptors, Nicotinic; D000069283:Rituximab