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Open AccessArticle

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

1
Department of Neurology, Otto-von-Guericke University, 39120 Magdeburg, Germany
2
Department of Neurology with Institute of Translational Neurology, University Hospital Münster, 41849 Münster, Germany
3
Department of Diagnostic and Interventional Neuroradiology, Hannover Medical School, 30625 Hannover, Germany
4
Department of Neurology, Hannover Medical School, 30625 Hannover, Germany
5
Department of Clinical Immunology and Rheumatology, Hannover Medical School, 30625 Hannover, Germany
*
Authors to whom correspondence should be addressed.
These authors contributed equally to this work.
J. Clin. Med. 2020, 9(5), 1482; https://doi.org/10.3390/jcm9051482
Received: 31 March 2020 / Revised: 10 May 2020 / Accepted: 12 May 2020 / Published: 14 May 2020
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 (621–50,000) vs. 585 pg/mL (357–729), p = 0.009). One patient showed sustained, highly increased NFL levels (50,000 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. View Full-Text
Keywords: Sjögren’s syndrome; Spinal cord; Neurofilament light chain; Antibodies; Cerebrospinal fluid; Myelitis Sjögren’s syndrome; Spinal cord; Neurofilament light chain; Antibodies; Cerebrospinal fluid; Myelitis
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Butryn, M.; Neumann, J.; Rolfes, L.; Bartels, C.; Wattjes, M.P.; Mahmoudi, N.; Seeliger, T.; Konen, F.F.; Thiele, T.; Witte, T.; Meuth, S.G.; Skripuletz, T.; Pawlitzki, M. Clinical, Radiological, and Laboratory Features of Spinal Cord Involvement in Primary Sjögren’s Syndrome. J. Clin. Med. 2020, 9, 1482.

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