What is the role of immunomodulatory therapy for autonomic failure syndromes?

Updated: Oct 21, 2018
  • Author: Mohini Gurme, MD; Chief Editor: Selim R Benbadis, MD  more...
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Answer

Immunomodulatory therapy has been used successfully to shorten the duration of symptoms and improve overall prognosis in acute and chronic pandysautonomia. [11]

  • Cases in which clinical improvement began within a few days of intravenous immunoglobulin (IVIg) administration (2 g/kg body weight over 2-5 d), along with normalization of autonomic test parameters, have been reported. [12, 13]

  • Presumably, IVIg has an immunomodulatory action, but the exact mechanism of its effect in this disorder is unclear.

  • In 1 series, 2 patients with acute pandysautonomia were treated with prednisone 60 mg/d for several months and reported subjective improvement. No quantitative follow-up data were obtained.

  • In a series of 3 patients with autoimmune autonomic ganglionopathy, patients treated with prednisone, mycophenolate mofetil, and plasmapheresis reported improvement when plasmapheresis or IVIg alone was not effective. [14]

  • Other pharmacologic treatment options are directed toward symptomatic relief only (See Medication). [5]


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