Autoimmune SFN Associated With Ehlers–Danlos Syndrome Treated With IVIG

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Autoimmune Small Fiber Neuropathy Associated With Ehlers–Danlos Syndrome Treated With Intravenous Immunoglobulins

Cook, Melissa K. MD; Jordan, Morgan DO

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Journal of Clinical Neuromuscular Disease: March 2021 - Volume 22 - Issue 3 - p 160-163
doi: 10.1097/CND.0000000000000341


Abstract


Patients with Ehlers–Danlos syndrome (EDS) have many associated symptoms of unclear cause, most recently suggested to be due to small fiber neuropathy (SFN).

Small fiber neuropathies are sorely underestimated and with minimal treatment options.

We report 2 cases of patients with EDS with presumed immune-mediated SFN, successfully treated with IV immunoglobulins.

There is a stark need for further investigational studies into immunosuppressant treatments for immune-mediated SFN as well as the link between EDS and immune-mediated SFN.