Long-term treatment with rituximab of autoimmune autonomic ganglionopathy in a patient with lymphoma.
OBJECTIVE
To report on the response to therapy in a patient with autoimmune autonomic ganglionopathy with a high titer of an autoantibody directed against the α-3 subunit of the nicotinic acetylcholine receptor (nAChR) of the autonomic ganglia.


METHODS
Case report.


METHODS
University-based referral center for autonomic dysfunction.


METHODS
Patient with prior indolent B-cell lymphoma who presented with symptomatic orthostatic hypotension and autonomic failure and was found to have a high titer of nAChR antibody.


METHODS
Plasma exchange and rituximab therapy (both initial 4-week therapy and maintenance therapy).


METHODS
Autonomic ganglionic antibody titer; the autonomic assessments were the presence of orthostatic hypotension, the concentration of plasma norepinephrine, and quantitative sweat testing.


RESULTS
Treatment with rituximab followed by plasma exchange significantly decreased the nAChR antibody titers for a short time, and then the titers increased. The titers suppressed to almost undetectable levels once regular maintenance therapy with rituximab was initiated. Reduction in nAChR antibody titer resulted in a decrease in orthostatic hypotension, an increased concentration of upright plasma norepinephrine, improvement in some sweat function, and improvement in symptoms.


CONCLUSIONS
Long-term rituximab therapy suppressed autoantibody production to undetectable levels over the course of 2 years and resulted in sustained clinical improvement in this patient with debilitating autoimmune autonomic ganglionopathy. More data are needed before rituximab therapy can be recommended as routine therapy for this disorder.
MSCI, Autonomic Dysfunction Center, Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University School of Medicine, AA3228 Medical Center North, 1161 21st Ave S, Nashville, TN 37232-2195, USA.
Ryan
R
Hollenbeck
Bonnie K
BK
Black
Amanda C
AC
Peltier
Italo
I
Biaggioni
David
D
Robertson
Elliott F
EF
Winton
Satish R
SR
Raj
D000368:Aged; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001323:Autoantibodies / Q000032:analysis / Q000276:immunology; D020274:Autoimmune Diseases of the Nervous System / Q000150:complications / Q000188:drug therapy* / Q000628:therapy; D001342:Autonomic Nervous System Diseases / Q000150:complications / Q000188:drug therapy* / Q000628:therapy; D001794:Blood Pressure / Q000502:physiology; D005260:Female; D005725:Ganglia, Autonomic; D006801:Humans; D007024:Hypotension, Orthostatic / Q000209:etiology; D016393:Lymphoma, B-Cell / Q000150:complications*; D009638:Norepinephrine / Q000097:blood; D010951:Plasma Exchange; D011978:Receptors, Nicotinic / Q000276:immunology; D000069283:Rituximab; D013546:Sweating / Q000502:physiology
D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D001323:Autoantibodies; D011978:Receptors, Nicotinic; D000069283:Rituximab; D009638:Norepinephrine