ArticleClinical rheumatology2023
Clinical features and high-risk indicators of central nervous system involvement in primary Sjögren's syndrome.
Article in Clinical rheumatology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 19 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
19 citing papers in PubMed, 1 synthesis or guideline pooled it, 15 citations in OpenAlex.
- Pulmonary amyloidosis, gastric MALT lymphoma, and multiple sclerosis in a patient with Sjögren's disease: a case report and review of the literature.Rheumatology international · 2026Pooled it
- The Clinical Utility of Labial Gland Biopsy in Patients With Suspected Neuro-Sjögren.Immunity, inflammation and disease · 2026Article
- When Dryness Extends to the Brain: Brain-Related Non-Sicca Manifestations of Sjögren's Disease.Journal of clinical medicine · 2026Review
- Acute Ischemic Stroke as the Initial Presentation of Primary Sjögren's Syndrome: A Case Report.Cureus · 2026Article
- Central nervous symptoms as the prominent manifestation of Sjögren's disease (SjD): a case report.BMC neurology · 2026Article
- NeuroPORTRESS: prevalence, clinical spectrum, and factors associated with neurological involvement in Sjögren's disease.Frontiers in immunology · 2026Article
- Endovascular Stent-Assisted Intracranial Lysis for Acute Middle Cerebral Artery Occlusion in a Young Woman with Primary Sjögren's Syndrome: Case Report.Journal of inflammation research · 2026Article
- TLR7 and TLR9 have overlapping but distinct roles in systemic lupus erythematosus and Sjögren's disease.Frontiers in immunology · 2026Review
- Elevated levels of nitrotyrosine-modified protein adducts and their association with the severity of primary sjogren's syndrome and secondary sjogren's syndrome with rheumatoid arthritis in Taiwanese Patients.Clinical rheumatology · 2026Article
- Initial assessment of blood-brain barrier permeability alterations and associated clinical characteristics in patients with primary Sjögren's syndrome: Insights from DCE-MRI.Clinical rheumatology · 2026Article
- Frequency-dependent and temporal variability of low-frequency fluctuations in patients with primary Sjögren's syndrome.Lupus science & medicine · 2025Article
- Primary sjögren's syndrome among patients with cerebral infarction.Scientific reports · 2025Article
- Regional brain function study in patients with primary Sjögren's syndrome.Arthritis research & therapy · 2025Article
- Impact of disease duration on systemic clinical profile in Sjogren's syndrome.Arthritis research & therapy · 2025Article
- Diversity of central nervous system manifestations in Sjogren's Disease: a case-based review.Rheumatology international · 2025Article
- Neurological and Psychiatric Clinical Manifestations of Sjögren Syndrome.Current neurology and neuroscience reports · 2024Review
- Spectrum of Central Nervous System Involvement in Childhood-Onset Sjögren's Syndrome: A Case-Based Review.Mediterranean journal of rheumatology · 2024Article
- Protein-A immunoadsorption combined with immunosuppressive treatment in refractory primary Sjögren's syndrome coexisting with NMOSD: a case report and literature review.Frontiers in immunology · 2024Review
- Hypertension, hyperlipidaemia and anti-Ro52 antibody need to be taken into account: comments on clinical features and high‑risk indicators of central nervous system involvement in primary Sjögren's syndrome.Clinical rheumatology · 2023Article
Corrections and comments
- Commented on by
- Erratum issued
Authors and funding
9 authors at 3 institutions in 2 countries.
Funding
Abstract
backgroundEvidence for central nervous system involvement in primary Sjögren's syndrome (pSS) patients is controversial and extremely limited. We aimed to describe the clinical profiles and high-risk indicators of primary Sjögren's syndrome (pSS) patients with central nervous system (CNS) involvement (pSS-CNS).
methodsA total of 412 participants with pSS from a hospital in China from January 2012 to December 2019 were enrolled in the retrospective study. 42 pSS-CNS patients were compared with 370 pSS patients without CNS involvement. The clinical features, laboratory examinations, imaging characteristics, and treatment of the pSS-CNS cases were systematically analyzed. Potential risk factors related to pSS-CNS patients were identified by multivariate logistic regression analysis.
resultsThe prevalence of central nervous system involvement in the studied pSS patients was 10.2% (42/412), with 31.3% (14/42) of pSS patients having neurological manifestations as the initial symptom. The manifestations of hemiparesis (35.7%, 15/42), paraparesis (28.6%, 12/42), dysphonia (31.0%, 13/42), blurred vision (21.4%, 9/42), and dysfunctional proprioception (23.8%, 10/42) were more common in the pSS-CNS patients. Cerebral infarction (57.1%, 24/42), demyelination (31.0%, 13/42), myelitis (23.8%, 11/42), and angiostenosis (21.4%, 9/42) were most often found on MRI or CT scan imaging in the pSS-CNS patients. Intrathecal IgG level and total protein of cerebrospinal fluid were increased in 50% (8/16) of the pSS-CNS group. In comparison with patients without CNS involvement, the pSS-CNS patients were found to also have kidney and lung involvement, hematologic abnormalities, positive ANA and anti-SSA antibody tests, and reduced complement 3 (C3) and complement 4 (C4) levels (all p < 0.05). The prevalence of lung involvement, immune thrombocytopenia, and high-titer ANA (1:1000) were significantly higher in pSS-CNS disease activity compared to those in the moderately active group. Multivariate analysis identified lung involvement, anti-SSA positivity, and low C3 levels as prognostic factors for pSS-CNS. After high-dose glucocorticoids and immunosuppressive therapy, 60.5% (26/38) of pSS-CNS patients improved, 36.8% (14/38) were unresponsive to treatment, and 2.6% (1/38) died.
conclusionClinical features are diverse in pSS-CNS patients, and the morbidity rate is low. CNS involvement was the initial presentation in state percentage here pSS patients. Pulmonary involvement, a positive anti-SSA antibody test, and reduced C3 levels are potential risk factors for CNS involvement in pSS. Treatment with high-dose glucocorticoids and immunosuppressive therapy appeared effective in 60% of pSS-CNS patients. Key Points • The CNS manifestations of pSS are diverse, and CNS imaging and CSF analysis are important for the diagnosis. • Pulmonary involvement, positive anti-SSA, and reduced C3 levels are potential risk factors of pSS-CNS. • About 60% of pSS-CNS patients were responsive to high-dose glucocorticoid administration and immunosuppressive therapy.
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