The burden and profile of neurological symptoms in patients with Sjögren disease: results from an international survey

OBJECTIVES: Neurological involvement in Sjögren's disease (neuro-SjD), reported by up to 20% of patients, represents a highly relevant extra-glandular manifestation. Owing to the clinical complexity, frequent diagnostic delay and the poor response to treatment, neuro-SjD remains a major unmet need. This study aimed to better understand the burden of neuro-SjD from the patient perspective, focusing on the overall care journey. METHODS: An anonymous multilingual survey was co-developed by patient research partners and rheumatologists and distributed via patient associations. RESULTS: A total of 4050 valid responses from SjD patients across 60 countries were collected and analysed. All respondents reported at least one of the symptoms listed in the survey, with each symptom having a medium-to-severe impact in at least half of the patients. A formal diagnosis of neuro-SjD most often made by a neurologist was reported by 21.6% of symptomatic patients. Among those diagnosed, only 21% received pharmacological therapies and just 23% of them reported significant or complete symptom improvement. Overall, satisfaction with neuro-SjD care was poor, with half of the respondents feeling that healthcare providers underestimated their neurological symptoms. CONCLUSION: Our findings suggest that the prevalence of neurological symptoms in SjD is likely underestimated and highlight neuro-SjD as a major unmet need. We advocate for raising awareness and prompting research in this area, facilitating the diagnosis and correct attribution of these debilitating manifestations to support timely diagnosis, accurate symptom attribution and ultimately identifying effective treatment strategies to improve the quality of life of people living with SjD.