Autoimmune autonomic ganglionopathy: treatment by plasma exchanges and rituximab.

Abstract

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.

• Authors: Richard Imrich, Steven Vernino, Basil A Eldadah, Courtney Holmes, David S Goldstein
• Journal: Clinical autonomic research : official journal of the Clinical Autonomic Research Society (Clin Auton Res) Vol. 19 Issue 4 Pg. 259-62 (Aug 2009) ISSN: 1619-1560 [Electronic] Germany
• PMID: 19399547 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Intramural)

Chemical References

• Antibodies, Monoclonal
• Antibodies, Monoclonal, Murine-Derived
• Autoantibodies
• Immunologic Factors
• Receptors, Nicotinic
• Rituximab

Topics

• Aged
• Antibodies, Monoclonal (therapeutic use)
• Antibodies, Monoclonal, Murine-Derived
• Autoantibodies (blood)
• Autoimmune Diseases of the Nervous System (therapy)
• Baroreflex (physiology)
• Female
• Ganglia, Autonomic (immunology)
• Humans
• Immunologic Factors (therapeutic use)
• Plasma Exchange
• Receptors, Nicotinic (immunology)
• Rituximab