Genetic risk and Guillain-Barré syndrome

Our immune system is amazingly powerful, protecting us against known and unknown foreign invaders. Occasionally, things go awry, and autoimmune diseases, such as Guillain-Barré syndrome, can occur. This article dives into the research on Guillain-Barré syndrome, explaining the course of the disease, and covering the genetic variants that increase the relative risk of this serious autoimmune disease.

What is Guillain-Barré syndrome?

Guillain-Barré syndrome is an autoimmune disease that causes weakness in limbs and muscles. Referred to as 'acute flaccid paralysis', people with Guillain-Barré syndrome (GBS) have neuromuscular weakness usually starts in the feet and legs that can spread, causing paralysis throughout the body. Guillain-Barré syndrome is caused by an autoimmune reaction that attacks peripheral nerves, leading to demyelination or axonal damage.[ref] This is an autoimmune disease that usually slowly resolves and doesn't return. The peak paralysis is usually reached within 4 to 6 weeks.[ref] Many GBS patients require extended hospitalization. Mechanical ventilation may be required if the paralysis impacts breathing. This occurs in about 30% of Guillain-Barré syndrome patients. Often a feeding tube is needed due to problems with swallowing. Studies show that 85% of GBS patients regain the ability to walk again. The mortality rate is about 5%, and 20% of patients have continued significant disability from Guillain-Barré.[ref] The autoimmune reaction in GBS involves antiganglioside antibodies, which attack gangliosides that are necessary for neuronal plasticity. These molecules are similar to sialic acids found in viruses, and thus may be attacked after the immune system is activated by a virus.[ref] The autoimmune attack on the peripheral nerves can lead to nerve death or the inability of the nerve to transmit a signal.

How common is Guillain-Barré syndrome?

Guillain-Barré syndrome is considered an 'uncommon disease'. Around the world, prevalence ranges from <1 to 2 in 100,000 people per year.[ref] Known triggers of Guillain-Barré syndrome (GBS) include:[ref]
  • Campylobacter jejuni infection[ref]
  • adenovirus infection[ref][ref]
  • the flu vaccine (especially the early vaccines in the 90s)[ref]
  • Johnson & Johnson COVID-19 vaccine[ref]
  • other adenovirus vector vaccines[ref][ref]
  • Zika virus
  • chikungunya virus
  • Epstein-Barr virus
  • influenza[ref]
Viral or bacterial illnesses are the most common trigger of this autoimmune disease. About 70% of patients have a viral or bacterial illness within 1 to 6 weeks before the onset of GBS.[ref] One study in India found that two-thirds of the Guillain-Barré syndrome patients had an identifiable recent infection such as chikungunya virus (16%) or Campylobacter jejuni  (5%).[ref] Obviously, not everyone who gets sick from a virus or bacteria will get Guillain-Barré.
  • About 1 in 1,000 people infected with Campylobacter jejuni, a bacteria in poultry that causes food poisoning, will end up with GBS.[ref]
  • About 2 in 1,000 people infected with the Zika virus get GBS.[ref]

Clinical outcomes of Guillain-Barré syndrome:

A study of 156 GBS patients showed that common symptoms include weakness, paralysis, sensory symptoms, facial paralysis, problems swallowing, and eye problems. About a  quarter of the patients were on mechanical ventilation. In this study group, about half of the patients regained the ability to walk after 9 months.[ref] Another study found that all Guillain-Barré patients included in the study had limb weakness in both arms and legs, and decreased reflexes in the arms and legs that persisted even in follow-up.[ref] A study of 45 Guillain-Barré syndrome patients in a Swiss hospital from 2010-2018 showed that IL-8, an inflammatory cytokine, was elevated in patients. This may be a way to differentiate GBS from other demyelinating neuropathies.[ref]

Guillain-Barré syndrome and vaccines:

I know this is a touchy topic and want to tread lightly here. Please don't take my word, or anyone else's, on vaccine reactions. Instead, you can search the VAERS database for the information. The VAERS database contains reports from doctors and people in the United States who have reacted to any vaccine. The database interface is not intuitive, so be sure to read the directions. Additionally, you will need to limit your search to a specific vaccine manufacturer and specific diagnoses because you will get an error message if the results are for more than 10,000 events. As of early July, more than 400,000 serious adverse events have been recorded for the COVID-19 vaccines, so you will have to drill down into the data to be able to see results related to paralysis, demyelination, autoimmune reactions, or Guillain-Barre hospitalizations. Furthermore, you can search for other vaccine side effects to gain an overview of how frequently adverse events are reported. Keep in mind that correlation doesn't automatically mean causation.
  • J&J COVID-19 vaccine: The FDA now includes a warning with the Johnson and Johnson COVID-19 vaccine regarding the increased risk of GBS. It is a rare side-effect, but the frequency is enough to prompt an FDA warning.[ref][article]  The Johnson and Johnson COVID-19 vaccine is an adenovirus vector that delivers the spike protein as a vaccine.
  • An adenovirus vaccine given to military personnel starting in 2011 reported Guillain Barre as an adverse event.[ref]
  • Historically, flu vaccines have been linked to GBS. More recent flu vaccines, though, might increase the cases of GBS by 1 case in 1,000,000 vaccines administered.[ref][ref]
  • No increase in Guillain-Barré syndrome has been seen after vaccination against "hepatitis B, influenza, hepatitis A, varicella, rabies, polio(live), diphtheria, pertussis(acellular), tetanus, measles, mumps, rubella, Japanese Encephalitis, and meningitis".[ref]
  • A few studies have reported slight increases in the GBS rate in young women after the HPV vaccine.[ref]
  • The FDA also includes a warning of an increased risk of GBS after the shingles vaccine (Shingrix). The warning is based on a statistical signal in the increase in GBS cases according to Medicare claims.[ref] The VAERS database contains reports from doctors and people in the United States who have reacted to any vaccine.

Genetics and Guillain-Barre:

Important note about relative risk: When a disease is rare, a big increase in the relative risk may not mean much. For example, if the normal annual rate of GBS is 1 in 1000,000 people, quadrupling that risk would mean a risk of 4 in 1000,000 (0.004%). Practically speaking: Even if a vaccine is causing a 10-fold increase in GBS cases and you have a genetic risk variant, your odds of getting Guillain-Barre syndrome are still really slim. Please take this information as being educational and interesting, rather than an indication of whether you should take any particular action. Members: Log in to see your data below Not a member? Join now to see your data.

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