Rare Classroom: Autoimmune Autonomic Ganglionopathy

Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.

Eyes front and ears open. Class is now in session.

The disease that we will be learning about today is:

Autoimmune Autonomic Ganglionopathy

Sometimes called autoimmune autonomic neuropathy.

What is Autoimmune Autonomic Ganglionopathy?

  • This condition is a form of autonomic failure triggered by the immune system
  • It’s associated with the presence of antibodies targeted against the nicotinic acetylcholine receptor, which is found on the autonomic ganglion
  •  The disease can vary in severity and may have a sudden onset, but in other instances, symptoms gradually worsen over time

How Do You Get It?

  • In some cases, autoimmune autonomic ganglionopathy is idiopathic, meaning that a distinctive cause cannot be identified
  • It can also be the result of paraneoplastic syndrome, which indicates the presence of a tumor
    • In these cases, the patient has cancer and the nicotinic acetylcholine receptor antibodies are produced by the immune system as a response to it

What Are the Symptoms?

  • The symptoms that present can vary by case, with some experiencing severe symptoms
  • Symptoms can include:
    • Orthostatic hypotension
    • Gastrointestinal dysmotility (vomiting, abdominal pain)
    • Anhidrosis
    • Fainting
    • Dizziness
    • Dry eyes and mouth
    • Urinary retention
    • Constipation
    • Impotence
    • Sensitivity to light
    • Intestinal pseudo-obstruction, which can be potentially deadly

How Is It Treated?

  • If a cancer or tumor is recognized to be causing the symptoms, then prompt treatment of it is critical in order to halt the symptoms progression
  • The exact route forward will vary depending on the cancer type, location, and staging, but may include chemotherapy, radiation, or surgery, among others
  • When there is no clear cause, immunosuppression is typically the approach employed
    • Options may include corticosteroids, plasmapheresis, rituximab, intravenous immunoglobulin (IVIG), azathioprine, or mycophenolate mofetil

Where Can I Learn More???

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