Thread by @NeuroSjogrens

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 👋👀⬇️🚨
Paradigm shifting article in the Green Journal of Neurology courtesy of cooperation between Neuro and Rheum at hosp in Hanover Germany. A cohort study of 512 "primary" #Sjogrens presenting to Neuro and to Rheum were evaluated for "strictly selected neuro symptoms." 1/n

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 2/n NB many of the most common neuro accompaniments to Sjogrens WERE NOT tested for (or were not reported) in this cohort including autonomics, HA, hearing loss, trigeminal involvement etc.) Despite this, 46% (!!) of #Sjogrens pts are #NeuroSjogrens patients.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 3/n IMPORTANTLY, the authors take pains to note
1) Neuro involvement has been v underestimated
2) The disease course is more severe than previously anticipated BUT responds well to immunosuppression
3) ESSDAI issues--multiple neuro probs w same level activity only scored once so

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 4/n a NEW score is created to measure Neuro issues and is recommended to be added to ESSDAI in the 'diagnostic algorithm' to better identify and follow #NeuroSjogrens.

Its called NISSDAI- Neurological involvement of Sjogrens Syndrome disease activity score

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 5/n NISSDAI was added in part bc current systematic workup of patients w/ neuro involvement in #Sjogrens "is still insufficient for early identification and thus for early adequate treatment initiation."

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 6/n What are laboratory predictors of PSSN?
*47% were SSA negative
*Higher ESSDAI score (18.2 in PSSN vs 7.7 PSS)
*Lower IgG (incl low end normal range)
*Fewer RF + in PSSN (17% vs 30%
* trace incr eos, wbc, PMN's in PSSN (still in nl range)
*higher CK

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 7/n Other characteristics of PSSN vs PSS:
*more balanced F:M ratio (65% PSSN vs 85% PSS)
*Older age (PSSN IQR 55yrs vs 45 yrs)
*More apt to be hospitalized at presentation
* Fewer drug allergies and seasonal allergies

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 8/n Of note from entire cohort PSSN+PSS:
*5% had multiple slcerosis!
*1% had myasthenia gravis
*1% had NMOSD
*4% had a stroke
*2% had TIA

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 9/n "The diagnostic algorithm should b thoroughly pursued in pt pw neurologic features, especially in cases of neuropathy, myasthenia, MS, NMOSD and cerebrovascular accidents."

Of note in peripheral neuropathies:
*20% are axonal
*46% are demyelinating
*31% are mixed

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 10/10 Myositis was diagnosed based on muscle biopsy in 18 patients & suggestive enhancement on PET combined with elevated CK in 1 patient.

"Our data support the hyporthesis that neurological involvement in Sjogren's has been previously underestimated and underdiagnosed."

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 11/11 "Many problems arose with the ESSDAI assessment when evaluating neurological patients in a cohort of this size. Therefor, the NISSDAI was developed as an initial proposal for a more adaptive scoring system for neurological symptoms in Sjogren's syndrome." -needs validation

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 12/12 Here's a link to this great paper

read.qxmd.com/read/36802030

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 13/n Why is this such a big deal? The authors of this paper recognize there is a problem with the current method of using ESSDAI/ESSPRI in diagnostic algorithms and tracking of #NeuroSjogrens patients course of disease and response to treatment.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 14/n ESSDAI and ESSPRI do not adequately capture the severity of #NeuroSjogrens/PSSN. They do not include the seemingly omnipresent small fiber damage and symptoms, including the very, very common autonomic neuropathy.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 15/n The authors could have stopped there but they created the NISSDAI (2b validated) to better capture the Neuro features that have been so long overlooked by Neuro and Rheum publications. NISSDAI items in next tweet.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 16/n Neurologic involvement in Sjogrens Syndrome disease activity score (NISSDAI)

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 17/n Despite failure to capture MANY neurologic features of #NeuroSjogrens (authors acknowledged they looked only for certain syndromes) such that this data is a significant underestimation, ALMOST HALF OF SJOGRENS HAVE NEUROSJOGRENS.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 18/n Clearly if autonomics (cardiovascular, GI, GU-bladder/sexual, sweating, cerebral blood flow etc), better cognitive testing, audiometry/BAER's, trigeminal lrg & small fiber, polyradiculopathy/itis &SSEP's, HA & more, a large majority of Sjogren's are NeuroSjogrens patients.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 19/n In an ideal world, if Rheumatology diagnoses #Sjogrens and there is ANY question of Neuro symptoms whatsoever, ALL these patients would get baseline Sjogrens specific Neurologic evaluation.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 20/n CLEARLY Neurologists are not yet ready for this as, far and wide Sjogren's patients report Neurologists who still say "no neuropathy" with just EMG/routine NCS. They report Neuro's having difficultiy identifying autonomic neuropathies and their common symptoms.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 21/n In addition, there is still the oddity of Sjogrens Classification Criteria/CC fixating on 2 tiny sets of glands/sicca when majority have Neuro damage, high % have pulmonary, many have renal, loads have cytopenias and more systemic involvement that is 100% IGNORED in CC's.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 22/n This ABSURDITY of Sj Classification Criteria mean MANY with #NeuroSjogrens were not picked up in this study bc most studies report a significant %age of NeuroSjogrens do not develop sicca for many yrs after debilitating Neuro damage and terrible QOL.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren Science may need to progress b4 it figures out if these patients belong to #Sjogrens or not. At present they are included in Sjogrens because on longitudinal f/u many do develop sicca and SSA. At present most of these patients (in the West) find themselves in a medical desert.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 24/n While there are problems with this paper I cannot get too upset for now because this article has moved the needle in #NeuroSjogrens in a significant way and it feels amazing to see researchers grappling w recognition NeuroSj is common & ESSDAI/ESSPRI inadequate.

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren 25/25
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@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren @SandhyaRheumat @DrChadJohr @SarahSchaferMD @SjogrensOrg @SjogrensForum @SjogrensCa @SjogrensIrl @SjogrensUMCG Thread on groundbreaking new study of #NeuroSjogrens!
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@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren @SandhyaRheumat @DrChadJohr @SarahSchaferMD @SjogrensOrg @SjogrensForum @SjogrensCa @SjogrensIrl @SjogrensUMCG @AbrahamAubry sorry, they only included some skin biopsies and failed to do LEP's, CCM's & autonomic testing. But they added small fiber neuropathy to the NISSDAI. Its a work in progress, but this study is a big step and is published in the main Neuro journal in US!

@mariaj_morenom @AbrahamAubry @SEReumatologia @SMurcianaReuma @reumalorca5 @aesjogren @CatSjogren @dysclinic, I wanted to call your attention to this great Neuro study on #Sjogrens #NeuroSjogrens. See summary & link in above thread. We need help 2 get dysautonomia included in new NISSDAI scale. Dysautonomia International is on board. You would b a gr8 addition to letter/s!