Immunotherapy for autoimmune autonomic ganglionopathy

Valeria Iodice, Kurt Kimpinski, Steven Vernino, Paola Sandroni, Phillip Anson Low

Research output: Contribution to journalArticle

32 Citations (Scopus)

Abstract

Purpose of review: To provide an update on recent advances in the treatment of autoimmune autonomic ganglionopathy (AAG). AAG is an immune-mediated disorder characterized by prominent and selective involvement of autonomic nerve fibers or ganglia. Treatment with intravenous immunoglobulin (IVIG) or plasma exchange (PE) has been reported to be effective in single case reports and recent case series. This review summarizes current treatment options including IVIG, PE, and immunosuppressants agents, alone or in combination. Results: Controlled clinical trials of immunotherapy are not available and experience with immunotherapy is confined to single case reports. Some patients with AAG seem to respond to IVIg or PE as initial therapy but often require subsequent immunosuppressive treatment to sustain or advance the improvement. Preliminary studies suggest that the most severely affected patients may need a protracted and combined treatment program to achieve clinical improvement. Summary: Several recent studies suggest that IVIg, PE, and immunosuppressant agents are effective treatment for AAG. Current available treatments used as monotherapy or in combination may be efficacious and the specific regimen may depend on the severity of autonomic failure. There is preliminary evidence that novel immunosuppressant agents such as Mycophenolate mofetil (MMF) and Rituximab may be effective in certain patients who are unresponsive to IVIG or PE.

Original languageEnglish (US)
Pages (from-to)22-25
Number of pages4
JournalAutonomic Neuroscience: Basic and Clinical
Volume146
Issue number1-2
DOIs
StatePublished - Mar 12 2009

Fingerprint

Immunotherapy
Plasma Exchange
Immunosuppressive Agents
Intravenous Immunoglobulins
Therapeutics
Autonomic Ganglia
Mycophenolic Acid
Autonomic Pathways
Immune System Diseases
Controlled Clinical Trials
Nerve Fibers

Keywords

  • Autoantibody
  • IVIG
  • Plasma exchange
  • Plasmapheresis

ASJC Scopus subject areas

  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Endocrine and Autonomic Systems

Cite this

Immunotherapy for autoimmune autonomic ganglionopathy. / Iodice, Valeria; Kimpinski, Kurt; Vernino, Steven; Sandroni, Paola; Low, Phillip Anson.

In: Autonomic Neuroscience: Basic and Clinical, Vol. 146, No. 1-2, 12.03.2009, p. 22-25.

Research output: Contribution to journalArticle

Iodice, Valeria ; Kimpinski, Kurt ; Vernino, Steven ; Sandroni, Paola ; Low, Phillip Anson. / Immunotherapy for autoimmune autonomic ganglionopathy. In: Autonomic Neuroscience: Basic and Clinical. 2009 ; Vol. 146, No. 1-2. pp. 22-25.
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