Cerebral Venous Thrombosis As a Rare Complication of Sjögren's Syndrome: Case Series and Literature Review

Overview

Journal Clin Rheumatol

Publisher Springer

Specialty Rheumatology

Date 2025 Jan 1

PMID 39743644

Authors

Qiuju Liao

Yi Zhao

Xia Li

Xuemei Li

Xu Huang

Li Su

Affiliations

Soon will be listed here.

Abstract

Objective: As few cerebral venous thrombosis (CVT) patients with primary Sjögren's syndrome (pSS) have been reported, little is known about the characteristics of this rare complication. This study is aimed at describing the clinical features, treatment, and outcome of CVT combined with pSS.

Materials And Methods: We reported five patients of CVT and pSS admitted to our hospital and searched the relevant case reports in PubMed for literature review.

Results: We reviewed a total of twelve patients with pSS and CVT. Among them, five patients were from our report in the present paper, and seven other patients were from the case reports searched in PubMed. In total twelve patients, eleven patients were female. The twelve patients had an average age of 43.7 ± 8.3 years (age range, 26-57 years). The symptoms of pSS included multiple caries (50%), dry mouth (41.7%), dry eyes (33.3%), arthritis symptoms (16.7%), and parotid gland swelling (8.3%). Headache was the most common neurological symptom in all patients. Four patients (33.3%) had no clinical symptoms associated with pSS. Anti-SSA antibodies were positive in all patients. Antiphospholipid antibodies (aPLs) were positive in 33.3% of patients. Unilateral transverse sinus (75.0%) was the most commonly involved venous sinus. All patients received anticoagulant therapy. Hydroxychloroquine was also administered to the patients. Seven patients were treated with glucocorticoids. All patients recovered completely with no clinical or radiological recurrence.

Conclusion: pSS combined with CVT is a rare condition. Middle-aged women with pSS should be alert to the presence of CVT. It is of great importance to screen for autoimmune diseases during the clinical course of CVT, especially in patients with unilateral transverse sinus thrombosis. Effective treatment strategies require further study. Key Points • Patients combined with CVT are very rare. • Screening for autoimmune diseases in CVT patients is great important. • Patients may have good prognosis when effective treatment is administrated.

References

Shen C, Yang A, Kuo B, Tsai S . Risk of Psychiatric Disorders Following Primary Sjögren Syndrome: A Nationwide Population-based Retrospective Cohort Study. J Rheumatol. 2015; 42(7):1203-8. DOI: 10.3899/jrheum.141361. View

Appenzeller S, de Oliveira Andrade S, Bombini M, Sepresse S, Reis F, Franca Jr M . Neuropsychiatric manifestations in primary Sjogren syndrome. Expert Rev Clin Immunol. 2022; 18(10):1071-1081. DOI: 10.1080/1744666X.2022.2117159. View

Delalande S, De Seze J, Fauchais A, Hachulla E, Stojkovic T, Ferriby D . Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004; 83(5):280-291. DOI: 10.1097/01.md.0000141099.53742.16. View

Jaskolska M, Chylinska M, Masiak A, Sieminski M, Zietkiewicz M, Czuszynska Z . Neuro-Sjögren: Uncommon or underestimated problem?. Brain Behav. 2020; 10(8):e01665. PMC: 7428478. DOI: 10.1002/brb3.1665. View

Zhang B, Lang Y, Zhang W, Cui L, Deng F . Characteristics and Management of Autoimmune Disease-Associated Cerebral Venous Sinus Thrombosis. Front Immunol. 2021; 12:671101. PMC: 8339549. DOI: 10.3389/fimmu.2021.671101. View

Alunno A, Carubbi F, Bartoloni E, Cipriani P, Giacomelli R, Gerli R . The kaleidoscope of neurological manifestations in primary Sjögren's syndrome. Clin Exp Rheumatol. 2019; 37 Suppl 118(3):192-198. View

Coutinho J, Zuurbier S, Aramideh M, Stam J . The incidence of cerebral venous thrombosis: a cross-sectional study. Stroke. 2012; 43(12):3375-7. DOI: 10.1161/STROKEAHA.112.671453. View

Devasagayam S, Wyatt B, Leyden J, Kleinig T . Cerebral Venous Sinus Thrombosis Incidence Is Higher Than Previously Thought: A Retrospective Population-Based Study. Stroke. 2016; 47(9):2180-2. DOI: 10.1161/STROKEAHA.116.013617. View

Wei H, Jiang H, Zhou Y, Liu L, Zhou C, Ji X . Intracranial hypertension after cerebral venous thrombosis-Risk factors and outcomes. CNS Neurosci Ther. 2023; 29(9):2540-2547. PMC: 10401171. DOI: 10.1111/cns.14194. View

10.

Urban E, Jabbari B, Robles H . Concurrent cerebral venous sinus thrombosis and myeloradiculopathy in Sjögren's syndrome. Neurology. 1994; 44(3 Pt 1):554-6. DOI: 10.1212/wnl.44.3_part_1.554. View

11.

Liu S, Bai M, Zhao Y . Cerebral venous thrombosis in patients with Sjögren's syndrome: A case report and literature review. Heliyon. 2023; 9(4):e15246. PMC: 10119761. DOI: 10.1016/j.heliyon.2023.e15246. View

12.

Lang Y, Zhang W, Wu X, Deng F, Cui L . Sjögren's Syndrome with Cerebral Venous Sinus Thrombosis: A Case Report and Literature Review. Ann Indian Acad Neurol. 2020; 23(1):110-112. PMC: 7001427. DOI: 10.4103/aian.AIAN_224_18. View

13.

Chandra M, G A M, M R . Primary Sjögren's Syndrome Presenting as Cerebral Venous Thrombosis: A Rare Case. Cureus. 2022; 14(9):e28772. PMC: 9531848. DOI: 10.7759/cureus.28772. View

14.

Mercurio A, Altieri M, Saraceni V, Paolucci T, Lenzi G . Cerebral venous thrombosis revealing primary sjögren syndrome: report of 2 cases. Case Rep Med. 2013; 2013:747431. PMC: 3570939. DOI: 10.1155/2013/747431. View

15.

Baldini C, Talarico R, Tzioufas A, Bombardieri S . Classification criteria for Sjogren's syndrome: a critical review. J Autoimmun. 2012; 39(1-2):9-14. DOI: 10.1016/j.jaut.2011.12.006. View

16.

Luo Y, Tian X, Wang X . Diagnosis and Treatment of Cerebral Venous Thrombosis: A Review. Front Aging Neurosci. 2018; 10:2. PMC: 5797620. DOI: 10.3389/fnagi.2018.00002. View

17.

Petri M . Antiphospholipid syndrome. Transl Res. 2020; 225:70-81. PMC: 7487027. DOI: 10.1016/j.trsl.2020.04.006. View

18.

Schreiber K, Sciascia S, de Groot P, Devreese K, Jacobsen S, Ruiz-Irastorza G . Antiphospholipid syndrome. Nat Rev Dis Primers. 2018; 4:17103. DOI: 10.1038/nrdp.2017.103. View

19.

Cervera R, Khamashta M, Shoenfeld Y, Camps M, Jacobsen S, Kiss E . Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2008; 68(9):1428-32. DOI: 10.1136/ard.2008.093179. View

20.

Ramos-Casals M, Nardi N, Brito-Zeron P, Aguilo S, Gil V, Delgado G . Atypical autoantibodies in patients with primary Sjögren syndrome: clinical characteristics and follow-up of 82 cases. Semin Arthritis Rheum. 2006; 35(5):312-21. DOI: 10.1016/j.semarthrit.2005.12.004. View