Document Detail


Combined immunomodulatory therapy in autoimmune autonomic ganglionopathy.
MedLine Citation:
PMID:  18268189     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Autoimmune autonomic ganglionopathy is a disorder defined by antibodies to the nicotinic acetylcholine receptor of the autonomic ganglia. Patients present with symptoms of autonomic failure, including syncope, orthostatic hypotension, bowel and bladder hypomotility, pupillary dysfunction, and dry mouth and eyes. Symptomatic and immunomodulatory therapy has provided limited clinical benefit in small uncontrolled studies.
OBJECTIVE: To investigate the effects of combined immunosuppressive therapy and plasmapheresis in autoimmune autonomic ganglionopathy.
DESIGN: Prospective case series.
SETTING: Academic medical center.
PATIENTS: Three patients with autoimmune autonomic ganglionopathy who had a limited response to symptomatic therapy, such as midodrine, fludrocortisone, vasopressin, and erythropoietin. Additional treatment with plasmapheresis alone and intravenous immunoglobulin alone provided no additional clinical benefit. Patients underwent 6 months of treatment with prednisone and mycophenolate mofetil followed by 5 cycles of plasma exchange.
RESULTS: Immunosuppressive therapy (prednisone and mycophenolate mofetil) combined with plasmapheresis resulted in substantial improvements in bowel control, pupillary function, dry mouth, and dry eyes. Mean (SD) blood pressure during immunosuppressive therapy was 162/83 (16/12) mm Hg supine and 76/45 (22/11) mm Hg standing (3 minutes). After 5 cycles of plasmapheresis, mean blood pressure was 132/82 (7/4) mm Hg supine and 127/81 (5/1) mm Hg standing (3 minutes; P < .01). Mean antibody level was 7.92 nmol/L on combined immunosuppressive therapy alone and dropped to 0.5 nmol/L after plasmapheresis.
CONCLUSIONS: In patients with autoimmune autonomic ganglionopathy, combining immunosuppressive medications prednisone and mycophenolate mofetil with plasmapheresis provides substantial and sustained clinical improvement that was not seen using either treatment alone. Multi-agent immunomodulatory therapies may be necessary to satisfactorily treat this immune-mediated disorder.
Authors:
Christopher H Gibbons; Steven A Vernino; Roy Freeman
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Archives of neurology     Volume:  65     ISSN:  0003-9942     ISO Abbreviation:  Arch. Neurol.     Publication Date:  2008 Feb 
Date Detail:
Created Date:  2008-02-12     Completed Date:  2008-03-19     Revised Date:  2014-09-08    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  213-7     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Adult
Autoantibodies / blood
Autoimmune Diseases of the Nervous System / drug therapy*,  physiopathology
Autonomic Nervous System Diseases / drug therapy*,  physiopathology
Blood Pressure
Female
Ganglia, Autonomic / drug effects,  physiopathology*
Heart Rate
Humans
Immunosuppressive Agents / administration & dosage,  therapeutic use*
Middle Aged
Mycophenolic Acid / administration & dosage,  analogs & derivatives*,  therapeutic use
Plasmapheresis*
Prednisone / administration & dosage,  therapeutic use*
Prospective Studies
Tilt-Table Test
Time Factors
Treatment Outcome
Grant Support
ID/Acronym/Agency:
K23 NS050209/NS/NINDS NIH HHS; K23 NS050209-01A2/NS/NINDS NIH HHS
Chemical
Reg. No./Substance:
0/Autoantibodies; 0/Immunosuppressive Agents; 9242ECW6R0/mycophenolate mofetil; HU9DX48N0T/Mycophenolic Acid; VB0R961HZT/Prednisone

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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