RGD Reference Report - Clinical, Radiological, and Laboratory Features of Spinal Cord Involvement in Primary Sjögren's Syndrome. - Rat Genome Database

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Clinical, Radiological, and Laboratory Features of Spinal Cord Involvement in Primary Sjögren's Syndrome.

Authors: Butryn, Michaela  Neumann, Jens  Rolfes, Leoni  Bartels, Claudius  Wattjes, Mike P  Mahmoudi, Nima  Seeliger, Tabea  Konen, Franz F  Thiele, Thea  Witte, Torsten  Meuth, Sven G  Skripuletz, Thomas  Pawlitzki, Marc 
Citation: Butryn M, etal., J Clin Med. 2020 May 14;9(5). pii: jcm9051482. doi: 10.3390/jcm9051482.
RGD ID: 127284890
Pubmed: PMID:32423153   (View Abstract at PubMed)
PMCID: PMC7290729   (View Article at PubMed Central)
DOI: DOI:10.3390/jcm9051482   (Journal Full-text)


OBJECTIVE: To identify radiological and laboratory hallmarks in patients with primary Sjögren's syndrome (pSS) presenting with spinal cord involvement.
METHODS: Clinical and laboratory routine parameters were analyzed in a retrospective multicenter case series of four patients who developed myelitis associated with pSS. Serological and cerebrospinal fluid (CSF) measurements of pSS associated anti-SSA(Ro)-antibodies were initiated, and CSF neurofilament light chain (NFL) levels were assessed. NFL values were compared with results from 15 sex- and age-matched healthy controls. Radiological assessment was performed using multi-sequence spinal cord magnetic resonance imaging.
RESULTS: Three of the four patients initially developed neurological signs suggestive of myelitis and were subsequently diagnosed with pSS. All patients presented a longitudinal spinal T2-hyperintense lesion in the cervical spinal cord, whereas only two patients showed pleocytosis and oligoclonal bands in the CSF. Median (range) CSF-NFL levels were significantly elevated in patients compared to controls (6672 pg/mL (621-50000) vs. 585 pg/mL (357-729), p = 0.009). One patient showed sustained, highly increased NFL levels (50000 pg/mL) in the initial assessment when radiological signs of axonal injury were still absent. Anti-SSA(Ro)-antibodies were found in the serum of three patients, while two patients additionally presented intrathecal anti-SSA(Ro)-antibody production. Elevated CSF-NFL levels and intrathecal synthesis of anti-SSA(Ro)-antibodies were associated with a relapsing and treatment-resistant disease course.
CONCLUSION: Inflammatory spinal cord lesions associated with pSS are a rare but serious disease leading to severe disability. NFL and anti-SSA(Ro)-antibodies in CSF might serve as prognostic biomarkers and should be routinely assessed in patients with pSS.



RGD Manual Disease Annotations    Click to see Annotation Detail View

  
Object SymbolSpeciesTermQualifierEvidenceWithNotesSourceOriginal Reference(s)
NEFLHumanSjogren's syndrome  IEP protein:increased expression:CSF (human)RGD 
NeflRatSjogren's syndrome  ISORGD:1344238protein:increased expression:CSF (human)RGD 
NeflMouseSjogren's syndrome  ISORGD:1344238protein:increased expression:CSF (human)RGD 

Objects Annotated

Genes (Rattus norvegicus)
Nefl  (neurofilament light chain)

Genes (Mus musculus)
Nefl  (neurofilament, light polypeptide)

Genes (Homo sapiens)
NEFL  (neurofilament light chain)


Additional Information