Abstract
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.
Highlights
Primary Sjögren’s syndrome is a chronic autoimmune disease characterized by lymphocytic infiltrations in secretory glands and extraglandular neurological manifestations [1,2]
The patients were classified according to the 2017 ACR-EULAR score (American College of Rheumatology/European League against Rheumatic Disease) for primary Sjögren’s syndrome and the EULAR Sjögren’s syndrome disease activity index (ESSDAI) at baseline [14,15,16]
Due to the severe clinical decline in our two patients with intrathecal synthesis of anti-SSA(Ro)-antibodies, we propose that cerebrospinal fluid (CSF) antibody measurement should be a standard assessment in patients with a suspected neurological manifestation of Primary Sjögren’s syndrome (pSS)
Summary
Primary Sjögren’s syndrome (pSS) is a chronic autoimmune disease characterized by lymphocytic infiltrations in secretory glands and extraglandular neurological manifestations [1,2]. The affection of the peripheral nervous system is more common than central nervous system (CNS) involvement. Anti-SSA(Ro)-antibodies can be found in approximately 50% of patients suffering from pSS. These antibodies have a high diagnostic value and seem to be associated with extraglandular disease manifestations, seronegative patients with CNS involvement have been described [6,7,8,9]. A direct pathogenic effect has been proposed, as anti-SSA(Ro)-antibodies can be detected serologically in patients with recurrent myelitis and without previous diagnosis of pSS [10]
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