Spencer Hutto, MD Profile picture
APD @EmoryNeurology | Autoimmune & Hospital Neurology @EmoryUniversity | Former AGAIN Fellow @MGHNeurology | Interested in Neurosarcoidosis & Neurorheumatology

Apr 24, 2023, 11 tweets

#Neuro manifestations in #Sjögren’s are common, present in nearly 20% (❗️), and 4M are estimated to have Sjögren’s in the US.

Neuro-Sjögren’s is poorly understood and underrecognized, and you’ll miss it if you aren’t looking for it. 🫣

But how do you find it❓

You’ve got to know the neurologic phenotypes.

What are they? Let’s see how well you know them ⬇️

What’s the most common neurologic manifestation?

Distal symmetric polyneuropathy - usually axonal and sensory, but can be sensorimotor.

▫️EMG is critical for diagnosis (see 👇) and to differentiate from other forms of neuropathy.
▫️Consider skin biopsy if negative for small fiber.

DOI: 10.1111/ene.15555

What type is highly suggestive of Sjögren’s? Clue: and also anti-Hu

Sensory neuronopathy - it carries an interesting DDx (anti-Hu, B6 toxicity, platinum chemo, etc), but Sjögren’s deserves to be at the top of the list of considerations.

🔑 - just know that Neuro manifestations precede typical Sjögren’s symptoms at 2:1.

DOI: 10.1002/mus.24943

What antibody has to be checked in someone with Sjögren’s-related myelitis? 🧐

AQP4 - LETM is usually the radiographic phenotype of Sjögren’s-related myelitis, and a recent lit review showed 89% were AQP4+❗️

Also 🔑: if one autoimmune sz doesn’t explain the full clinical picture, consider another. Might change mgmt.

DOI: 10.1016/j.semarthrit.2010.05.005

Now if 20% of 4M have Sjögren’s = 800k people! 🤯

How many doctors are dedicated to studying and caring for people with these neurological complications? Good question, I can only name a few.

It starts with you. #advocacyinmedicine #AANAM @AANmember #rheumtwitter

I should add that neurological manifestations in #Sjögren’s are wide-ranging, and the discussion here only mentions a few of the neurological phenotypes to be aware of.

If you want a more in-depth review:
PMID: 24307005

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