Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes Treatment & Management

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

Medical Care

The treatment of autoimmune autonomic neuropathy (AAN) is based on anecdotal evidence. No data from large, controlled trials are available owing to the rarity of the disorder. The treatment of chronic pure autonomic failure syndromes is symptomatic only. Postural orthostatic tachycardia syndrome can be treated by using low doses of beta-blockers as patients are normally sensitive to their adverse effects.

Nonpharmacologic measures are useful for all patients with autonomic dysfunction. [5]

  • Discontinue antihypertensive medications and other medications known to lower blood pressure, if feasible.

  • Increase fluid and salt intake.

  • Equipment aids may be helpful. These include tight support stockings, abdominal binders, or antigravity suits for symptomatic hypotension and bladder catheterization for urinary retention.

  • Dietary fiber and enemas may help improve bowel motility and decrease straining during defecation.

  • Patients with decreased sweating should limit their physical activity, particularly in hot weather. Sponging with water during activity may help prevent overheating.

  • Large meals may exacerbate hypotension and should be avoided.

  • Perform positional changes, such as standing up, slowly and gradually.

  • Elevate the head of the bed and avoid prolonged recumbency.

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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Activity

Symptoms limit activity. Precautions for falling should be taken in patients who have orthostatic hypotension. In those with decreased sweating, vigorous exercise should be limited, and patients should be advised to have spray bottles of water or wet sponges available during hot weather or during physical activity.

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